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primary cutaneous tuberculosis results from the direct inoculation of mycobacterium tuberculosis into the skin of a person with no previous history of tuberculosis infection 1 . cutaneous tuberculosis is considered predominantly an occupational disease and is a challenging diagnosis to make , especially in low - income countries due to a wide array of differential diagnoses , for example , fungal infections , leishmaniasis 2,3 . a 27-year - old previously healthy african male medical intern sustained a needle - stick injury from a wide bore needle ( gauge 18 ) to his little finger while performing a lumbar puncture on a hiv - infected patient . he sustained a small lesion that bled a little and he immediately washed it with water and soap . he was immediately started on postexposure prophylaxis anti - retroviral drugs ( arvs ) : zidovudine , lamivudine and kaletra for 28 days as per the kenya national aids control program protocol . his initial rapid hiv test ( determine ) test was negative and so was a pcr done on completion of the arvs . the patient source , an african female , was who clinical stage 4 , not on arvs and was being investigated for meningitis died soon the lumbar puncture and her results were not followed up until several months later . two weeks after the injury , the intern had swelling of the little finger associated with a persistent dull ache for which he sought surgical intervention . pus was aspirated from the finger and incision and drainage were done under local anesthesia . culture of the pus grew staphylococcus aureus sensitive to flucloxacillin on which he was started . his little finger now had an open wound that persisted for several months despite debridement and different antibiotic regimens : levofloxacin , clindamycin , ceftriaxone , and vancomycin . for the next 6 months , there was persistent swelling of the little finger which seemed to be spreading to the hand ( fig.1 ) . this was accompanied with low - grade fever , night sweats , and subjective weight loss . he underwent a surgical debridement 6 months after the injury and was started on levofloxacin . serial blood counts done in the course of illness showed persistently elevated lymphocytes and a raised esr . ten months later and with no improvement of symptoms , he underwent yet another surgical debridement . histological examination of the tissue taken revealed a chronic inflammatory process ( fig.2 ) , granulomatous tubercles with epithelioid cells ( fig.3 ) , giant cells of langerhans ( fig.4 ) , and a mononuclear infiltrate but no acid - fast bacilli ( afb ) were demonstrated on ziehl nelson stain . he was started on rifampicin , isoniazid , pyrazinamide , and ethambutol for duration of 2 months to be followed by a 4-month course of rifampicin and isoniazid . a rapid hiv test done at the end of the anti - tb treatment was negative . tuberculosis continues to pose a significant public health problem and kills about 3 million people annually 4 . it is largely an airborne infection , but skin manifestations may be caused by hematogenous spread or contiguity from foci of infection which may be active or latent . primary inoculation , another mode of transmission 5 , results from direct inoculation of m. tuberculosis into the skin of a person who has no previous exposure and subsequently no immunity to the organism 6 . cutaneous tuberculosis is rare and accounts for 0.1% of dermatology cases and only 1.5% of extra pulmonary tuberculosis cases 7,8 . once the traumatized skin of a previously uninfected person is inoculated with m. tuberculosis , a tuberculous chancre develops at that site within 3 weeks . a painless regional lymphadenopathy becomes prominent 36 weeks after inoculation , and a previously negative , intradermal , intermediate - strength purified protein derivative ( ppd ) test converts to a positive test 1 . cutaneous tuberculosis is commonly seen amongst young adults because of their likelihood to sustain workrelated injuries and inoculation of tubercle bacilli 9 . the diagnosis of tuberculosis in this case was masked by an initial culture growth of s. aureus which led to a delay in diagnosis and several months of morbidity for the medical intern . on tuberculous arthritis of the knee with staphylococcus super infection in which a delay in the diagnosis led to adverse outcome 12 . diagnosis requires correlation of clinical and histopathologic findings but a mycobacterial culture is the most reliable method of detecting mycobacteria and monitoring treatment response . an absolute diagnosis can be made when afb is visualized on a ziehl nelson - stained slide of a smear prepared from material from lesions 13 . cutaneous tuberculosis that occurs by direct inoculation is a paucibacillary disease , sparse bacilli seen on histology and microorganisms are difficult to isolate 2 . smears , ziehl nelson staining , and mycobacterial cultures in lowenstein jensen and bactec media are frequently negative 14 . typical features of a tuberculous chancre of tuberculosis include granulomatous tubercles with epithelioid cells , langerhans giant cells , and a mononuclear infiltrate 15 . useful diagnostic tools in the diagnosis of cutaneous tuberculosis include histopathologic findings of tubercles , isolation of m. tuberculosis in cultures of biopsy material , or by polymerase chain reaction 16 . management of cutaneous tuberculosis is the treatment with four - agent regimen given for 2 months followed by a two - drug regimen for the next 4 months as per tuberculosis treatment guidelines for tuberculosis in other organs 13 . primary cutaneous tuberculosis is rare and should be suspected in all patients who present with skin lesions that do not respond to antibacterial treatment . . a high index of suspicion is required to make the diagnosis of cutaneous tuberculosis because diagnostic methods are not sufficient and may lead to a delay in starting appropriate methods . complete microbiological tests
key clinical messagethe authors report a case of cutaneous tuberculosis in a 27-year - old african male medical intern who contracted primary cutaneous from a needle - stick injury . cultures of pus aspirated from the finger initially grew staphylococcus aureus that led to a delay in the diagnosis .
one of the most frequently asked questions to those of us at june is what kinds of manuscripts are appropriate for inclusion in the journal . any manuscript with the aim of enabling others to enhance their teaching of neuroscience to undergraduates is appropriate ; those with empirically - tested protocols of innovative pedagogy are particularly welcomed and prioritized for publication in june . beyond manuscripts devoted to new classroom approaches and laboratory exercises , a variety of manuscripts in other aspects that are relevant to undergraduate neuroscience education are welcomed , such as : editorials . june welcomes book reviews ranging from popular press volumes relevant to neuroscience to textbooks.media reviews . june welcomes reviews of media such as films , television shows , websites , and software that may have particular value to neuroscience education.commentaries . june welcomes reviews of media such as films , television shows , websites , and software that may have particular value to neuroscience education . manuscripts must be properly formatted according to the instructions to authors available at the june website ( http://funjournal.org ) . manuscripts should be carefully proofread , and attention should be given to both the flow and appearance of information and positioning of tables , figures , and captions . to facilitate indexing , authors are welcome to provide a list of suggested reviewers , which may or may not be used in the review of their article . the first step in the process is an initial screen by the editors to ensure that the manuscript is appropriate for the journal . if not , a notification indicating why the manuscript does not fit the criteria for inclusion in june is sent to the author(s ) . sometimes , an editor may provide suggestions of how the manuscript may be modified to better fit the criteria . once the initial reviews are received , the editors make a decision to accept , request revisions and resubmissions , or reject . often times the difference between acceptance and rejection hinges on a few key issues . in the case of manuscripts detailing innovative approaches to teaching , contentions of effectiveness need to be supported with discussion of appropriate methods of assessment and supporting evidence provided by the results obtained . in some cases , an author may inadvertently fail to obtain permission to use and discuss copyrighted material . in all cases if an author has been asked to revise portions of their manuscript , a cover letter indicating how each of the reviewer s comments and concerns have been addressed should be included in the resubmission . one of the most frequently asked questions to those of us at june is what kinds of manuscripts are appropriate for inclusion in the journal . any manuscript with the aim of enabling others to enhance their teaching of neuroscience to undergraduates is appropriate ; those with empirically - tested protocols of innovative pedagogy are particularly welcomed and prioritized for publication in june . beyond manuscripts devoted to new classroom approaches and laboratory exercises , a variety of manuscripts in other aspects that are relevant to undergraduate neuroscience education are welcomed , such as : editorials . june welcomes book reviews ranging from popular press volumes relevant to neuroscience to textbooks.media reviews . june welcomes reviews of media such as films , television shows , websites , and software that may have particular value to neuroscience education.commentaries . june welcomes reviews of media such as films , television shows , websites , and software that may have particular value to neuroscience education . manuscripts must be properly formatted according to the instructions to authors available at the june website ( http://funjournal.org ) . manuscripts should be carefully proofread , and attention should be given to both the flow and appearance of information and positioning of tables , figures , and captions . to facilitate indexing , authors are welcome to provide a list of suggested reviewers , which may or may not be used in the review of their article . the first step in the process is an initial screen by the editors to ensure that the manuscript is appropriate for the journal . if not , a notification indicating why the manuscript does not fit the criteria for inclusion in june is sent to the author(s ) . sometimes , an editor may provide suggestions of how the manuscript may be modified to better fit the criteria . all manuscripts that are deemed appropriate for the journal are sent to reviewers . once the initial reviews are received , the editors make a decision to accept , request revisions and resubmissions , or reject . often times the difference between acceptance and rejection hinges on a few key issues . in the case of manuscripts detailing innovative approaches to teaching , contentions of effectiveness need to be supported with discussion of appropriate methods of assessment and supporting evidence provided by the results obtained . in some cases , an author may inadvertently fail to obtain permission to use and discuss copyrighted material . in all cases if an author has been asked to revise portions of their manuscript , a cover letter indicating how each of the reviewer s comments and concerns have been addressed should be included in the resubmission . there is a range of ways readers of june can contribute to the journal beyond the submission of manuscripts . one very important way is to use the journal as a resource in your own teaching and to encourage your colleagues to do this as well . in addition , you can promote june to other neuroscience educators , encouraging them to read the journal and to submit manuscripts discussing their own approaches and innovative techniques for teaching neuroscience . it s important to support june in another way as well by joining and holding membership in fun . while june is an open - access journal and free to all , june does cost money to publish . fun members , through a portion of their very reasonable annual dues , provide critical support for june . consider also being a reviewer for june . one is to become an ad hoc reviewer ; to do so , simply send the editor in - chief a short email message expressing your willingness to review articles , indicating your particular areas of expertise . while review board members are regularly called on to complete reviews , such service is typically limited to two or three reviews per year . review board members are also considered to fill vacancies that happen periodically on the editorial board . to be considered for service on the june review board , please contact the june editor - in - chief regarding a possible appointment . june has come a long way since being founded in 2002 . through the efforts of our contributing authors , reviewers , and the entire editorial board , june has contributed to the success of undergraduate programs and neuroscience education around the globe . an important milestone for the second decade of june will be the successful completion of our efforts to become indexed across major databases and services , including psych info , scopus , the national science digital library , the directory of open access journals , medline and pubmed ( grisham , 2012 ) . at this writing , only indexing in medline and pubmed remain to be accomplished , and the application process is partially completed . as technology changes and new avenues for electronic communication become available , expect june to change as well , adding new features or altering existing ones to reflect latest developments . with support from fun members , the undergraduate neuroscience educational community will continue to have a readily available venue for learning about the latest innovations in laboratory exercises and improved teaching approaches . be sure to visit june online at ( http://funjournal.org ) , in person at the fun and at the society for neuroscience annual meetings , or both . publish in , and review articles for june and join in the fun of promoting undergraduate neuroscience education and research .
in the fall of 2002 , the faculty for undergraduate neuroscience ( fun ) began publication of its flagship journal , the journal of undergraduate neuroscience education ( june ) . for the past ten years , june has been a major forum for the free exchange of information among undergraduate neuroscience educators . numerous articles on laboratory exercises , media , pedagogy , curriculum , and issues pertinent to neuroscience educators have been published in june during the past decade . given the vast expertise in pedagogy amongst the fun membership and within the undergraduate neuroscience education community at large , we strongly encourage all fun members and june readers to become actively involved in june by contributing manuscripts and/or by offering your services as a reviewer .
syndrome of inappropriate secretion of antidiuretic hormone ( siadh ) is a common cause of hyponatremia . although it has been associated with different pulmonary infections , there have been only few case reports describing the association of siadh with influenza . we report a case of siadh in a patient with influenza who was successfully treated with fluid restriction . it is essential for clinicians to be aware of the association between influenza and siadh . syndrome of inappropriate secretion of antidiuretic hormone ( siadh ) is one of the most common causes of hyponatremia in hospitalized patients , with a prevalence as high as 35% . it is characterized by the impairment of urinary dilution in the absence of any renal disease or any identifiable nonosmotic stimulus that induces antidiuretic hormone ( adh ) release . although siadh has been known to be associated with different pulmonary infections , only rarely has it been reported with influenza . we present a case of hyponatremia associated with influenza , which was subsequently diagnosed as siadh . a 65-year - oldmale presented to the emergency department with low - grade fever and productive cough for 2 days . he did not have any neurological symptoms , abdominal pain , nausea , vomiting , change in bowel movements , or loss of appetite or weight . he did not have any history of recent surgery , central nervous system disorders , malignancy , or known pulmonary disease . on examination , he had a temperature of 102.4f , heart rate 120/min , respiratory rate 24/min , blood pressure 110/60 mmhg without orthostatic vital signs , and oxygen saturation 96% on room air . laboratory examination revealed a serum sodium of 122 meq / l ( 135 - 145 ) , blood urea nitrogen ( bun ) 11 mg / dl ( 8 - 24 mg / dl ) , creatinine 1.35 mg / dl ( baseline 1.2 - 1.3 ) , plasma osmolality 276 mosm / kg ( 280 - 290 ) , urine specific gravity of 1.029(1.002 - 1.030 ) , urine osmolality 777 mosm / kg , urine sodium 63 meq / l , thyroid - stimulating hormone ( tsh ) 3.011 iu ( 0.5 - 5.0 mu / l ) , and random serum cortisol 20.2 g / dl ( 7 - 25 g / dl ) . the chest x - ray did not show any infiltrate , consolidation , or mass . the nasal swab polymerase chain reaction was positive for influenza a. urinary antigens for legionella , mycoplasma , and streptococcus were negative . a diagnosis of siadh was made and his hyponatremia was corrected gradually in 3 days with fluid restriction o less than 800 ml / day . at 2 weeks thus , a careful history to rule out other causes like malignancy , pulmonary conditions , central nervous system lesions , and medications is essential in the evaluation of any patient suspected with siadh . it has been reported in many pulmonary infections including asthma , atelectasis , acute respiratory failure , and pneumothorax . influenza has been implicated as a cause of siadh previously , but there is no published data regarding the actual incidence of siadh in these patients . our patient was diagnosed with siadh based on : hypoosmolar hyponatremia;urine osmolality > 100 mosm / kg;urine sodium concentration > 40 meq / l;euvolemic state ; andnormal renal , thyroid , and adrenal function . hypoosmolar hyponatremia ; urine osmolality > 100 mosm / kg ; urine sodium concentration > 40 meq / l ; normal renal , thyroid , and adrenal function . failure of improvement of serum sodium with intravenous normal saline further confirmed the diagnosis of siadh . although the exact pathogenesis for siadh in influenza is unknown , it is thought to be related to the secretion of proinflammatory cytokines . interleukin ( il)-2 , il-6 , il-1 , and tumor necrosis factor ( tnf)- have been reported to stimulate parvocellular and magnocellular neurons to secrete more adh ; thus causing siadh . fluid restriction is the main treatment modality in siadh , with a suggested goal intake of less than 800 ml / day . further treatment options depend on the severity of hyponatremia and the presence of other related symptoms . in the presence of severe or symptomatic hyponatremia , hypertonic saline goal should be to raise serum sodium less than 10 - 12 meq / lin 24 h to ovoid the potential complication of osmotic demyelination with rapid correction . oral salt tablets and loop diuretics may also be added if optimum response is not seen with fluid restriction alone . our patient 's siadh was thought to be secondary to influenza , which was treated with oseltamivir and led to steady improvement in the patient 's serum sodium levels . in conclusion , clinicians should be cognizant of the association between influenza and siadh to allow for accurate diagnosis and treatment of this condition . further studies are needed in future to find out the incidence and pathogenesis of siadh in patients with influenza . although treatment depends on the severity of hyponatremia and associated symptoms , fluid restriction remains the cornerstone of therapy .
context : syndrome of inappropriate secretion of antidiuretic hormone ( siadh ) is a common cause of hyponatremia . although it has been associated with different pulmonary infections , there have been only few case reports describing the association of siadh with influenza.case report : we report a case of siadh in a patient with influenza who was successfully treated with fluid restriction.conclusion:it is essential for clinicians to be aware of the association between influenza and siadh .
injury to intraperitoneal organs is unusual during percutaneous renal surgery . we report a splenic injury during upper pole percutaneous renal access for nephrostolithotomy that was managed conservatively . a 52-year - old male with left upper pole renal stones associated with a narrow upper pole infundibulum underwent upper pole renal access prior to percutaneous nephrostolithotomy ( pcnl ) . the access was performed in the 10th to 11th intercostal space , and the patient underwent pcnl with stone clearance . on postoperative day 5 , the patient was evaluated for persistent flank pain and bleeding from the nephrostomy tube . the patient was admitted to the hospital , and the general surgery service was consulted . a pullback nephrostogram revealed no perirenal leak , and no evidence was present of acute bleeding . follow - up computerized tomography on the same day revealed no evidence of acute bleeding . the patient was discharged without further complications and remains stone free at 1-year follow - up . a transsplenic renal access that was dilated and through which a successful left percutaneous nephrostolithotomy was performed is a highly unusual complication related to upper pole left renal access . percutaneous nephrostolithotomy is the treatment of choice for most large renal stones , and the success of the procedure is critically dependent on obtaining an access with optimal angles for lithotripsy and stone removal . while thoracic entry and pneumothorax is a known complication with intercostal renal access , injury to intraperitoneal organs is unusual . we report the successful conservative management of a splenic injury resulting from transsplenic access and tract dilation in a patient undergoing pcnl . a healthy 52-year - old male presented with left upper pole renal stones associated with a narrow infundibulum ( figure 1 ) . due to stone location and related renal anatomy , the upper pole approach was preferred , and upper pole renal access through the 10th to 11th intercostal space was obtained in interventional radiology prior to pcnl . the access was successful , and the patient underwent balloon dilation of the tract and pcnl with complete stone clearance . nephrostogram during initial puncture demonstrates upper pole calculi with a narrow , tortuous infundibulum . on postoperative day 3 , the patient was evaluated in the emergency room due to flank pain and bleeding through the nephrostomy tube . on postoperative day 5 , he was reevaluated for persistent bleeding from the nephrostomy tube and flank pain . computerized tomography of the abdomen and pelvis was obtained , revealing transsplenic percutaneous renal access ( figure 2 ) . in consultation with interventional radiology and general surgery , a decision was made to leave the nephrostomy in place for 2 weeks after surgery . nephrostomy tube found to traverse the spleen on computerized tomography without hematoma . at that time follow - up computerized tomography on the same day revealed no evidence of acute bleeding . splenic portion of the nephrostomy tract as seen by fluoroscopy ( a ) and computerized tomography ( b ) . most complications are related to percutaneous renal access , with bleeding and pneumothorax being most common . when supracostal puncture is performed , the risk of pneumothorax or pleural effusion requiring drainage is 4% to 12% . splenic injury may require surgical management . however , conservative management with splenic preservation is feasible as demonstrated here . the risk of splenic injury during pcnl has been estimated by hopper and yakes , who used ct to analyze the relationship of the kidney , spleen , and lower ribs . their analysis noted that splenic injury is highly unlikely if an 11th or 12th rib supracostal approach is made during expiration . the risk increases to 13% if this approach is taken on inspiration and may be as high as 33% if a 10th to 11th approach is used for access . splenic injury in our patient was most likely due to supra-11th puncture at the skin level . access was quite oblique with the needle directed caudally and , in retrospect , transperitoneally . the advantages of upper over lower pole access include direct access along the long axis of the kidney and to the ureteropelvic junction , usually allowing for less torque of the rigid nephroscope and less bleeding . we feel that the upper pole should have been accessed given the patient 's anatomy . however , this may have been achieved at the 11th to 12th intercostal space , thus lessening the risk of transsplenic puncture and splenic injury .
introduction : injury to intraperitoneal organs is unusual during percutaneous renal surgery . we report a splenic injury during upper pole percutaneous renal access for nephrostolithotomy that was managed conservatively.methods:a 52-year - old male with left upper pole renal stones associated with a narrow upper pole infundibulum underwent upper pole renal access prior to percutaneous nephrostolithotomy ( pcnl ) . the access was performed in the 10th to 11th intercostal space , and the patient underwent pcnl with stone clearance . plain film radiography after percutaneous access and pcnl revealed no pneumothorax or hydrothorax . the patient was discharged on postoperative day one with the nephrostomy tube in place.results:on postoperative day 5 , the patient was evaluated for persistent flank pain and bleeding from the nephrostomy tube . computerized tomography revealed a transsplenic percutaneous renal access . the patient was admitted to the hospital , and the general surgery service was consulted . the patient was placed on strict bedrest . his hematocrit was within normal limits and remained stable . the nephrostomy tube was kept in place for 2 weeks . a pullback nephrostogram revealed no perirenal leak , and no evidence was present of acute bleeding . follow - up computerized tomography on the same day revealed no evidence of acute bleeding . the patient was discharged without further complications and remains stone free at 1-year follow-up.conclusions:a transsplenic renal access that was dilated and through which a successful left percutaneous nephrostolithotomy was performed is a highly unusual complication related to upper pole left renal access . we were able to manage this complication with conservative measures .
pneumomediastinum is defined as air or free gas in the mediastinum , which invariably arises from the alveolar spaces or the conducting airways and has a multi - factorial aetiology . it is a benign and self - limiting condition that usually affects young males ( 1 ) . although uncommon , this condition is increasingly seen in emergency departments owing to the epidemic rise of substance abuse ; an effect which is thought to be related to the mechanism of intoxication rather than pharmacology of the offending compound ( 2 ) . we report here an interesting case of cocaine induced pneumomediastinum . a fit and healthy 27 year old gentleman presented to the accident and emergency department complaining of severe sharp central chest pain and vertigo . there were no other symptoms of note and his past medical history and family history were unremarkable for respiratory or cardiovascular disease . on examination his blood tests showed a crp 36 , with all other hematological and biochemical parameters within normal levels . an electrocardiogram and serum troponin were unremarkable . a chest x - ray ( fig . he was admitted and transferred to the care of the surgical team with a diagnosis of suspected bronchial or oesophageal perforation . 2 ) confirmed the presence of a pneumomediastinum extending from the lower neck at the level of c6 to the diaphragmatic hiatus . there were no visible mediastinal collections and no evidence of pulmonary /airway disease with intact pulmonary vessels . an oesophageal perforation was suspected but not shown on a water - soluble contrast swallow . reassessment of the patient 's personal history revealed that he had nasally inhaled powdered cocaine prior to the onset of his symptoms . he denied the use of other recreational substances although he admitted to a significant history of smoking and alcohol use . the patient was managed conservatively during the course of his admission with simple analgesia and iv maintenance fluids . an upper gi endoscopy revealed oesophagitis for which he was commenced on a proton pump inhibitor . therefore with the low probability of lung injury , a possible diagnosis of micro - perforation of the oesophagus was made . once again , the patient was managed conservatively and was discharged a few days later symptom free . its occurrence secondary to cocaine inhalation has been reported in the literature as a known but uncommon event . the presumed patho - physiology of acute cocaine medicated lung injury is believed to arise from a sequence of events that begin with inhalation induced bronschospasm , increased alveolar pressure followed by alveolar rupture leading to interstitial emphysema and pneumomediastinum ( 3 ) . patients are generally young and present hours after cocaine inhalation with symptoms of acute onset chest pain , neck pain and surgical emphysema . their vital signs will reveal a high respiratory rate and low oxygen saturation . in some institutions the term crack lung is more commonly used to identify this phenomenom ( 4 ) . feliciano et al have reported cocaine induced gastro - intestinal perforations and these events are said to be due to co - existing h.pylori infection leading to mucosal weakness , which is then exacerbated by valsalva action ( 5 ) . our case report demonstrates evidence of inflammation of the oesophagus , and low probability of a lung injury in the setting of pneumomediastinum secondary to cocaine use . indeed we postulate that oesophagitis may also contribute to mucosal weakness leading to microperforation of the oesophagus and thereafter pneumomediastinum . however with no signs of lung injury on examination or investigations , microperforation in the setting of oesophagitis seems to be the most likely cause . his continued use of cocaine despite counseling and community support is likely to place him at risk of recurrent episodes . repeat microperforations on the background of oesophagitis may predispose this patient to an oesophageal catastrophe with mediastinal contamination and sepsis . the use of a proton pump inhibitor to promote oesophageal healing may be important in preventing such an outcome .
we describe an interesting case of pneumomediastinum secondary to cocaine abuse . the patient presented with severe chest pain following nasal inhalation of a large quantity of cocaine . investigations revealed no chest injury ; however oesophagitis was proven leading to a possible aetiology of oesophageal microperforation . after conservative management there was spontaneous resolution of the pneumomediastinum .
temporal bone metastasis of hepatocellular carcinoma ( hcc ) has rarely been reported.1 only one case of facial nerve palsy due to temporal bone metastasis of hcc has been reported and it was detected by autopsy.2 we encountered a case of metastatic hcc to the temporal region of the skull that initially presented as facial nerve palsy and was incidentally found during the diagnostic work up . a 61-year - old man visited our hospital because of progressively worsening right facial pain and numbness over a 6-month period . he could not make abrupt facial expressions on the right side of his face for seven days before he came to our hospital . , he had paresthesia and hypesthesia over all territories of 3 branches of the trigeminal nerve , facial palsy in the right side of his face , and hearing loss without tinnitus in his right ear . in nasopharyngeal magnetic resonance image ( mri ) , a 5-cm , heterogeneously enhancing tumor was found in t1 weighted image ( fig . the tumor was located in the masticator space and right middle cranial fossa , abutting the right cavernous sinus and extending intracranially to the right ipsilateral infratemporal fossa . the tumor was hypermetabolic in positron emission tomography ( pet ) study using c-11-methionine , which suggested that the tumor might be a malignant lesion ( fig . bony destruction of the anterior wall , which was adjacent to the anterior genu portion of the facial nerve , was suspected to be the cause of facial nerve palsy . his laboratory findings were as follows : white blood cell count was 6,900 per mm , hemoglobin was 15.3 g / dl , platelet was 152,000 per mm , serum albumin was 3.4 g / dl , total bilirubin was 1.1 mg / dl , alkaline phosphatase was 134 iu / l , aspartate aminotransferase was 97 iu / l , alanine aminotransferase was 192 the serologic finding revealed positive for hepatitis b surface antigen ( hbsag ) , antibody to hepatitis b envelop antigen ( hbeag ) and antibody to hepatitis c virus ( anti - hcv ) . his serum hepatitis b virus dna level was 97,200 iu / ml . serum alpha - fetoprotein was 5,200 ng / ml and des - gamma - carboxy prothrombin was 17,268 nau / ml . endoscopic surgical biopsy with sphenoidotomy via the right nasal cavity was performed by an otolaryngologist . during surgical biopsy , immunohistochemical findings of the biopsy specimen revealed positive staining for alpha - fetoprotein , which was also consistent with metastatic hcc ( fig . 4 ) . other metastases were found in lumbar spines ( l1 , l2 ) and right femur in bone scan . radiation therapy was performed for the tumor in the masticator space and right middle cranial fossa and for the metastatic lesions in lumbar spines and right femur . after radiation therapy was finished , his facial pain subsided significantly and his facial nerve palsy was partially relieved . facial pain did not occur again and facial nerve palsy did not progress until 7 months later . to evaluate the primary hcc in liver , abdominal computed tomography ( ct ) was performed . a 5-cm - sized , fat - containing , ill - defined and subtly enhanced mass lesion was found in segment 8 in the cirrhotic liver ( fig . 5 ) . although repeated transarterial chemoembolization ( tace ) was tried 3 times over 7 months to treat this tumor , only marginal lipiodol uptake was observed with each tace . seven months after initial diagnosis of metastatic hcc , back pain occurred over 2 weeks . as no therapy remained for the multiple spinal metastases , the patient was transferred to a hospital near his home for supportive care . temporal bone metastasis of hcc has rarely been reported.2 though hcc commonly metastasizes to bone , metastasis of hcc to skull is uncommon.3 facial palsy due to hcc was reported once in an advanced cirrhotic patient after autopsy.2 therefore , our report might be the first case report of metastatic hcc to the temporal region of skull involving the facial and trigeminal nerve , as confirmed by surgical biopsy.2 metastatic hcc was diagnosed by surgical biopsy in this case . even if hcc had been found in liver prior to performing the biopsy of temporal metastatic lesion , tissue confirmation of the lesion in temporal area would have been done for proper management because exact diagnosis was needed for proper management and because the lesion could have been an isolated double primary tumor such as schwannoma . as far as temporal bone metastasis of malignant tumors , bilateral temporal bone involvements are more common than unilateral involvement , but in this patient and the previous autopsy - proven case of temporal bone metastasis of hcc , the lesions were only on the right side.2 hearing loss without vestibular manifestation was reported as the most common symptom of temporal bone metastases in a retrospective study of 47 autopsy cases , which was consistent with this case.4 in the case of facial palsy secondary to metastasis , involvement of other cranial nerves was more common than involvement of the facial nerve alone , which was also consistent with this case.4 radiological investigation is strongly recommended if the facial nerve palsy is clinically associated with other neurological signs.5,6 bone and brain metastases of hcc have been treated with palliative radiation therapy and pain relief has been observed in about 78% of patients , suggesting good responsiveness to radiation.7 therefore , we performed radiation therapy for the metastatic lesion in the temporal area and the pain completely subsided . our case may suggest that temporal bone metastasis of hcc is also a very good indication for radiation therapy . hepatic dysfunction is known to be the main prognostic factor in patients with hcc , even in patients with extrahepatic metastasis . the major survival factor for our patient might also be the progression of hepatic dysfunction.8,9 sorafenib treatment might have been considered in this patient because the intrahepatic hcc progressed despite repeated tace ; however , sorafenib was not available during our caring for him.10,11 in summary , we encountered a very rare case of histologically proven temporal bone metastasis of hcc that was found during the evaluation of a patient who presented with trigeminal , facial and auditory nerve dysfunction and who was positive for both hbsag and anti - hcv but without previous history of illness . our case suggests that hepatologists may consider metastatic hcc as a rare cause of new onset cranial nerve palsy in patients with chronic viral hepatitis , and radiation therapy may be considered for the treatment of temporal bone metastasis of hcc with cranial nerve involvement .
facial nerve palsy due to temporal bone metastasis of hepatocellular carcinoma ( hcc ) has rarely been reported . we experienced a rare case of temporal bone metastasis of hcc that initially presented as facial nerve palsy and was diagnosed by surgical biopsy . this patient also discovered for the first time that he had chronic hepatitis b and c infections due to this facial nerve palsy . radiation therapy greatly relieved the facial pain and facial nerve palsy . this report suggests that hepatologists should consider metastatic hcc as a rare but possible cause of new - onset cranial neuropathy in patients with chronic viral hepatitis .
a 53-year - old lady , of height 152 centimeters and weighing 45 kilograms diagnosed with rheumatoid arthritis was first seen by us in april 2003 for a pre - chloroquine workup . she was seen at six - monthly intervals and advised amsler grid self - evaluation between her scheduled eye examinations . in april 2006 , the vision in her left eye dropped to 20/30 n8 with no subjective change in vision . a clinically detectable rpe disturbance was noted , more prominent in her left eye [ figures 1 , 2 ] . amsler grid evaluation was normal , but hfa 10 - 2 revealed , repeatable superior paracentral defect in both eyes [ figure 3 ] . fundus fluorescein angiography ( ffa ) revealed rpe defects in the macular region in both eyes . a recommendation to the treating internist four months later , she reported distortion of vision in both eyes , which was reflected as a small blurred area in the center of the amsler grid and vision in both eyes of 20/30 n8 optical coherence tomography ( stratus 4 oct ; carl zeiss meditec , dublin , calif ) revealed a marked retinal thinning of the parafoveal region [ figure 4 ] . the internist was informed and hydroxychloroquine was replaced by methotrexate . since then , her vision has remained stable at 20/30 n8 . chloroquine , and more recently , the apparently less toxic hydroxychloroquine , is used for long periods of time for treatment of various autoimmune disorders . cambiaggi first described the classic rpe changes in 19571 and hobbs in 1959 established a definite link between long - term use of chloroquine and subsequent development of retinal pathology.6 early chloroquine retinopathy though still inadequately described , is defined as an acquired paracentral scotoma on threshold visual field testing , with no detectable retinal findings , while advanced retinopathy has associated parafoveal rpe atrophy.7 chloroquine and its metabolites have been found in the pigmented ocular structures at concentrations much greater than in any other tissue in the body , which may explain its toxic properties in the eye.3,8 animal studies have suggested that ganglion cell damage occurs early with choloroquine.4,8 human pathological studies are however limited to patients with advanced maculopathy.3 detection of resultant thinning of the ganglion cell and nerve fiber layer ( nfl ) would be useful in demonstrating the initial stages of toxicity , perhaps at a stage when further damage can be halted by stopping the drug at this point . the nfl measurements of the peripapillary region in patients on antimalarials by scanning polarimetry have indeed shown a significant thinning of the nfl which was dose and duration - dependent.4 however , the ganglion cell population is the densest in the macular region , and hence , toxicity of the drug would be greatest at this location . the fact that the first functional change is a paracentral scotoma , and the first observable rpe changes are seen in this area supports this assumption.7 thus retinal thickness and volume measurements in this area may give more accurate and earlier predictions of chloroquine toxicity , perhaps even before the scotoma develops . the oct , with its high resolution provides measurements of the retinal thickness and volume , both in the peripappillary area as well as at the macular region . it also gives information on the status of the retinal pigment epithelium , clearly revealing rpe defects . in addition , the limitations of the scanning laser polarimeter with respect to inaccuracies related to corneal and lens birefringence are not present . a recent study using a research prototype of a high - speed ultra - high - resolution oct reported discontinuity or loss of perifoveal photoreceptor inner segment / outer segment junctions and thinning of the outer nuclear layer in 15 patients receiving hydroxychloroquine.5 this instrument is however not available as yet for general clinical use . in our patient [ figure 2 ] , the fovea ( central circle ) is of normal thickness while the perifoveal ( inner circle ) and peripheral ( outer circle ) , are thinned mainly temporally and inferiorly . this is in agreement with the universally accepted early superior paracentral scotoma , also seen in this patient [ figure 1 ] . although reports in the older literature on chloroquine toxicity had suggested that the cumulative dose of chloroquine was the critical factor for toxicity , current evidence suggests that cumulative dosage and duration of therapy are relatively unimportant and that the crucial index is daily dosage normalized by lean body weight.7,9 in this patient , the dose of chloroquine she was on clearly exceeded the recommended daily dose of 4 mg / kg / day.10 this is probably why she developed maculopathy just 36 months after starting the chloroquine therapy for her rheumatoid arthritis . long - term prospective studies may determine when retinal thinning starts in patients on antimalarials , and whether irreversible paracentral field defects could be prevented if antimalarials are stopped on first detection of thinning .
we herein report the optical coherence tomography ( oct ) findings in a case of chloroquine - induced macular toxicity , which to our knowledge , has so far not been reported . a 53- year - old lady on chloroquine for treatment of rheumatoid arthritis developed decrease in vision 36 months after initiation of the treatment . clinical examination revealed evidence of retinal pigment epithelial ( rpe ) disturbances . humphrey field analyzer ( hfa ) , fundus fluorescein angiography ( ffa ) and oct for retinal thickness and volume measurements at the parafoveal region were done . the hfa revealed bilateral superior paracentral scotomas , ffa demonstrated rpe loss and oct revealed anatomical evidence of loss of ganglion cell layers , causing marked thinning of the macula and parafoveal region . parafoveal retinal thickness and volume measurements may be early evidence of chloroquine toxicity , and oct measurements as a part of chloroquine toxicity screening may be useful in early detection of chloroquine maculopathy .
from 1994 through 2005 , epidemiologic data on animal - related injuries and associated postexposure prophylaxis ( pep ) treatment were prospectively collected for marseille rabies treatment centre patients . only patients who had been injured in france were selected ; rabies pep for travelers who were injured abroad is detailed elsewhere ( 6 ) . of the 4,965 eligible patients , 4,367 were outpatients or inpatients ( 192488/year ) , and from 2001 through 2005 , a total of 598 were managed by teleconsultation only because their exposure risk was considered to be zero . the number of inpatients and outpatients decreased markedly from 1999 to 2001 ( figure 1 ) , which is consistent with the general decrease in the number of pep treatments in france after the elimination of terrestrial mammal rabies ( 7 ) . furthermore , prescreening of persons by telephone also contributed to this decrease . the increase observed during 20042005 is likely an effect of the international alert in relation to the cases of rabid dogs imported from morocco ; these cases were intensively reported by the french media . the proportion of animal - related injuries tended to increase in late spring / early summer ( figure 2 ) , probably as a result of increased outdoor activities in southern france , which makes contact with animals more likely . number of injured patients per year seeking care for rabies postexposure prophylaxis , marseille centre , marseille , france , 19942005 . average proportions of injured patients seeking care for rabies postexposure prophylaxis , by month , marseille centre , marseille , france , 19942005 . the overall annual incidence of injured patients seeking care for rabies pep was 16/100,000 , which is consistent with incidence recently reported in united states ( 8,9 ) ( where rabies is enzootic in bats and raccoons ) but far less than that reported in recently available studies from the canine rabies endemic countries of turkey ( 467/100,000 ) ( 10 ) and india ( 1,700/100,000 ) ( 11 ) . the overall mean annual incidence in our study was 20/100,000 before 2001 and 11/100,000 after 2001 . by contrast , a recent study on pet demographics in france indicated that dog and cat populations are nearly similar at 8.51 million and 9.94 million , respectively ( 12 ) . this finding suggests that dogs , more often than cats , are responsible for severe injuries that lead persons to seek care for rabies pep . the mean annual incidence of animal - related injuries was lower in rural than in urban communities ( technical appendix ) . because an estimation of the dog population in france indicated that 41% live in urban areas ( 12 ) , our results suggest that a high human population density increases the probability of human dog interactions and risk for injuries . among patients seeking care for rabies pep , most were male ( male : female ratio 1.49 ) and mean age was 31.5 ( median 29 , range 096 ) years . the likelihood for animal - related injuries among male patients was also dependant on the animal species involved ; dogs , bats , and monkeys accounted for most injuries ( technical appendix ) . in contrast , female patients were more likely to be injured by cats , a finding consistent with previous reports ( 13 ) . the mean time between injury and consultation was 2.6 days ( range 0365 days ) and did not statistically vary by sex or age group . time was longer in patients who were injured by bats ( p<10 , online technical appendix ) , probably because most bat bites are nonpainful and considered benign by patients who ignore the risk for rabies after bat contact . most injured persons experienced severe contact with animals ( 95.1% ) , categorized by the world health organization ( who ) as category iii ( 14 ) . rabies testing of animal is not available in southern france , and animals from this region should be sent to the rabies laboratory at the pasteur institute in paris , which was done for 89 cases , of which 20 cases were related to a confirmed rabid source from africa or the middle east ( table 1 ) . imported from morocco ( 187 treatments were given in france ; most in bordeaux centre ) . the proportion of patients who received treatment increased from 42% during 19942000 to 84.3% during 20012005 ( p<10 ) as a result of prescreening by telephone ( table 2 ) . since 2001 , when the animal was not available for surveillance by a veterinarian ( which includes numerous cases in which the animal was available for observation by its owner ) , complete treatment was given to most ( 89% ) patients . rabies immunoglobulin was provided to 3.2% of these patients , most of whom were injured by bats or severely injured by domestic animals when the owner was not identified or when surveillance of the responsible animal was not possible . * pep , postexposure prophylaxis ; rig , rabies immunoglobulin ( % as proportion of treatments including rabies pep ) . animal not available for observation by a veterinarian ( including cases where animal was available for observation by its owner ) . animal proven to be rabid by laboratory testing or considered rabid upon clinical criteria . animal proven to be not rabid by laboratory testing or after 2 weeks of observation by a veterinarian . our rabies pep data are consistent with data from the national french referral center ( 7 ) . the therapeutic approach in france is partly in accordance with who general recommendations that in rabies - free areas where adequate rabies surveillance is in effect , rabies pep may not be required , depending on the outcome of a risk assessment conducted by a medical expert ( 14 ) . systematic rabies pep is cost - effective and safe but should not be used if the biting animal is unlikely to be rabid . furthermore , treating a patient with only vaccine when the animal is under observation could reduce the benefit of further administration of rabies immunoglobulin if the time between vaccination and rabies immunoglobulin injection is > 7 days ( 15 ) . if the treatment can not be delayed , it should include both vaccination and rabies immunoglobulin in cases of category iii injury . from 2001 through 2005 , not vaccinating the patient when the animal was under observation by its owner or a veterinarian would have represented an overall savings of 177,600 euros . to minimize overprescription of vaccination for rabies pep when treatment may be unjustified , we recommend delaying the initiation of rabies treatment in injuries involving an apparently healthy indigenous dog or cat that can be kept under veterinary or animal - owner observation for 2 weeks , which is the maximum rabies incubation time in these animals . however , when animals are not available for observation , complete rabies pep treatment should be initiated . given the risk for importation of rabid animals from nearby rabies - endemic countries , immediate rabies pep treatment according to who guidelines should be given when the following are involved : indigenous bats ; animals illegally imported from rabies - endemic countries ; or animals found in railway stations , trains , or other ports of entry . if the animal is suspected of being rabid at the time of exposure , confirmatory testing should be conducted ( technical appendix ) . all travelers visiting countries where rabies is enzootic should be informed about the risks of bringing animals back to their home country and about the who recommendations regarding rabies vaccination of imported animals ( 14 ) .
the administration of human rabies postexposure prophylaxis near marseille ( southern france ) has changed since the eradication of terrestrial mammal rabies in 2001 . most injuries were associated with indigenous dogs ; rabies vaccine was overprescribed . we suggest that the world health organization guidelines be adapted for countries free of terrestrial mammal rabies .
unique to this case , the foley catheter beats the odds and traversed the fistulous tract . we outline the presentation , identification and accepted management for surgical treatment of vesicouterine fistulas . a 28-year - old g1p1 was referred to our fistula hospital with a 5-month history of leaking urine per vagina . the leakage was intermittent , and immediately followed caesarean delivery of a stillbirth after labouring 3 days . additionally , she had 5 months of amenorrhoea but cyclic haematuria accompanied by cramping pains . the genital examination was unremarkable , with no leakage of dye following retrograde filling of the bladder . given her complaints , she was scheduled for an exploratory laparotomy for suspected vesicouterine fistula . a foley catheter passed immediately before surgery was noted to have no urine drainage , but the examination confirmed proper transurethral placement . on surgical exploration , the bladder was identified , but the foley bulb was curiously absent . dissecting the bladder completely free from the uterus revealed the problem . surrounded by dense scar tissue was the missing foley catheter , traversing the fistulous tract ( fig . 1 ) . vesicouterine fistula clearly demonstrated the catheter found passing through the bladder ( reflected to the right by babcock clamps ) to the uterus . vesicouterine fistula clearly demonstrated the catheter found passing through the bladder ( reflected to the right by babcock clamps ) to the uterus . urogenital fistula continues to be a major problem in developing countries , primarily due to obstructed labour . in recent decades , however , there has been an increase in fistula cases resulting from caesarean section . while most obstetric fistulas are vesicovaginal , this case highlights the vesicouterine type , one recognized to occur most often secondary to caesarean delivery [ 13 ] . youssef described a common presentation to vesicouterine fistula of amenorrhoea , cyclic haematuria ( which he termed menouria ) and urinary continence . since then , case and series reports have demonstrated a variable presentation to this problem . some report urinary incontinence , which is commonly intermittent , while others present with no urinary leakage . it is believed that the passage of menses through the fistula is due to differences in the pressure gradients between the uterus and bladder , which also explains the lack of urinary leaking except when the pressure of the bladder is raised sufficiently , such as with micturition or valsalva . surgical management is the mainstay of treatment for vesicouterine fistula , though some consider hormonal attempts using ocps . transperitoneal access to the fistula was employed in this picture , with dissection of the uterus from the bladder , closure of the uterine rent , then opening the bladder and repairing its fistula using a modified o'connor technique . the follow - up at 3 and 10 months confirmed the resolution of this patient 's symptoms . similarly , published success rates for surgical management of this type of fistula are high . the authors attest to having obtained written consent from the patient whose case is reviewed in this report .
a 28-year - old g1p1 presented complaining of urine leakage per vaginum following caesarean delivery , accompanied by amenorrhoea , cyclic haematuria and cyclic pelvic pain . examination findings were suggestive of vesicouterine fistula and the patient was taken for exploratory laparotomy , during which the foley catheter could not be identified within the bladder . during separation of the bladder from the uterus , the catheter was found to be traversing the fistulous tract into the uterine cavity . vesicouterine fistula is a fairly uncommon type of urogenital fistula that is frequently associated with caesarean section . surgical treatment remains the mainstay and successfully cured this patient .
it is a benign locally aggressive tumor usually involving the distal end of the femur , proximal tibia and distal radius in young adults . the main variables to be considered for planning treatment include the site of involvement and campanacci stage of the tumor . different treatment options are available which include intralesional curettage , extended curettage , wide resection and reconstruction . functional and oncological outcome of these treatment options varies widely , the predominant detrimental factor being tumor recurrence rate . reconstruction of endoprosthesis after wide excision of the tumor offers good short - term and mid - term functional and oncological outcomes as established by previous studies . this study was conducted to evaluate the long - term outcome of 11 patients with gct who underwent wide excision and customized endoprosthetic replacement . this study included 11 patients ( eight men and three women ) aged 2448 ( mean 32 ) years with primary gct of proximal femur campanacci stage - iii who were available for mean follow - up duration of 10.6 ( range 10.214 ) years . the definite diagnosis was established on histopathological confirmation with incisional biopsy . computed tomography scan of chest and bone scan none of the patients had pulmonary metastasis , and all the 11 patients had a solitary lesion in the proximal femur . these patients underwent wide resection of the tumor using postero - lateral approach to the proximal femur . the proximal femur was reconstructed using a customized , titanium , cemented endoprosthesis [ figure 1 ] . hip abductors , short external rotators and iliopsoas tendon were secured onto the prosthesis and hip capsule repair was performed . postoperative rehabilitation protocol included nonweight bearing and abduction splinting of the limb for 6 weeks followed by nonweight bearing crutch walking for another 6 weeks . once the hip abductors and quadriceps strength was regained weight bearing was allowed . long - term functional outcome was evaluated at minimum 10 years duration using revised musculoskeletal tumor society rating scale . ( a ) radiogragh showing giant cell tumor of proximal femur in a 34-year - old man . ( b ) magnetic resonance imaging showing giant cell tumor of proximal femur with soft tissue extension . there were no instances of prosthesis related complications like aseptic loosening or dislocation . at the end of mean 10.6 years six patients had good hip function without any restrictions , four patients had intermediate functional restriction whereas one had the recreational restriction of function . nine patients enthusiastically accepted the outcome of the procedure whereas two patients had satisfactory emotional acceptance . eight patients were walking without any support with unlimited walking abilities whereas three patients were using a cane for support while walking . the mean is revised musculoskeletal tumor society score was 26.8 out of 30 [ table 1 ] . traditional treatment of gct has been a difficult problem in orthopaedic oncology owing to high recurrence rates following conventional treatment with curettage or extended curettage . ideally treatment currently with improvement in reconstructive surgical techniques and availability of high quality biomechanically designed megaprosthesis , wide resection of tumor with proximal femur endoprosthesis replacement is being considered as a treatment option for campanacci stage - iii lesions in proximal femur with extensive osteolysis and soft tissue extension . it offers good local control of s with least recurrence rate and favorable functional outcome . the previous studies have shown satisfactory short- and mid - term functional and oncological outcomes . this study shows good long - term functional , and oncological outcomes of the procedure and hence the authors recommended as an endoprosthetic replacement for advanced gct of the proximal femur . furthermore , randomized control trials are required to established this modality as a standard treatment .
introduction : giant cell tumor ( gct ) of bone is locally aggressive benign tumor involving the epiphysis of long bones in young adults . various treatment options include intralesional curettage , extended curettage , wide resection , resection and reconstruction and amputation . the main variables to be considered for planning treatment include the site of involvement and campanacci stage of the tumor . functional and oncological outcomes of these treatment options vary widely , the predominant detrimental factor being tumor recurrence rate.aim:a study was conducted to evaluate the long - term oncological and functional outcome of patients with gct of the proximal femur that underwent tumor resection and endoprosthetic replacement.materials and methods : eleven patients with campanacci stage - iii gct of proximal femur who underwent wide excision of tumor and endoprosthesis replacement with a mean follow - up the duration of 10.6 years were assessed using standard proforma . the treatment outcome was evaluated using the revised musculoskeletal tumor society rating scale for the lower extremity.results:at mean follow - up the duration of 10.6 years , none of the cases had tumor recurrence , infection , prosthesis loosening or dislocation . all the patients were community ambulators among whom eight patients were walking without support while three patients were using a cane for support . the mean total musculoskeletal tumor society score was 26.8 out of 30 indicating the good outcome.conclusions:the authors recommend that wide resection and endoprosthetic replacement should be considered as a preferred treatment option for proximal femur gct as the functional , and oncological outcome is satisfactory with this modality of treatment .
coronary arterial fistulas are rare cardiac anomalies that create new pathways of blood flow between coronary vessels and thoracic vasculature or chambers of the heart . although generally asymptomatic , patients can develop complications of thrombosis , congestive heart failure , rupture , endocarditis , and arrhythmias . here , we describe a patient with acute decompensated heart failure and transient left bundle branch block ( lbbb ) , found to have an underlying left anterior descending ( lad- ) to - pulmonary artery ( pa ) fistula . this case illustrates the potential of coronary fistulas to induce a clinical presentation of cardiac ischemia via a coronary steal mechanism . a 64-year - old man with a history of congestive heart failure and stage iv chronic kidney disease secondary to uncontrolled hypertension and diabetes mellitus presented with three days of orthopnea and one night of intermittent left shoulder pain radiating to the back . physical examination revealed a heart rate of 99 beats per minute and blood pressure of 149/71 mm hg with an oxygen saturation of 94% on 4 liters of oxygen . laboratory analyses were significant for a hematocrit of 24% and creatinine of 3.8 mg / dl . twelve - lead electrocardiography ( ecg ) revealed sinus rhythm at 94 beats per minute and a new lbbb . an acute myocardial infarction was suspected and the patient was taken for emergent cardiac catheterization , which showed stenosis of up to 40% of the mid - lad coronary artery and mild disease in other vessels . in addition , a fistula was identified connecting the lad to the distal main pa ( figures 1(a ) and 1(b ) ) . transthoracic echocardiography demonstrated moderate global systolic dysfunction with a left ventricular ejection fraction of 40% as well as a moderate - sized pericardial effusion without echocardiographic evidence of increased intrapericardial pressure . a regadenoson nuclear perfusion stress imaging study performed after 4 days of diuresis and a blood transfusion was negative for inducible ischemia . furthermore , an ecg on the day of the stress imaging study showed resolution of the lbbb , and one week later the patient 's ecg showed recovery to near normal left ventricular ejection fraction and decrease in size of the pericardial effusion . we hypothesized that the small lad - pa fistula was not the sole cause of heart failure symptoms but was a contributor to the patient 's clinical presentation and ecg findings . the small fistula in this case steals from the lad - supplied myocardium , including the left bundle . by itself , the fistula was not sufficient to manifest clinically . however , in the setting of increased oxygen demand and decreased oxygen supply during the heart failure exacerbation , this coronary steal likely decreased the total oxygen supply in the lad territory enough to cause additional transient ischemia and lbbb . since he was previously asymptomatic without objective evidence of ischemia in the absence of myocardial oxygen supply and demand mismatch , we elected to continue close observation rather than closure of the fistula . coronary arterial fistulas are rare communications between coronary vasculature and thoracic vessels or cardiac chambers . the most frequent sites of drainage include the ventricles , pulmonary arteries , the coronary sinus , the superior vena cava , or the pulmonary veins . of the different types of fistulas , 42% originate from the left coronary tree and 17% drain into the pulmonary artery . fistulas are generally congenital in origin , but they can also arise from cardiac trauma , chest irradiation , cardiac surgery , coronary angioplasty , and endomyocardial biopsy . while fistulas are usually asymptomatic , their natural history can be variable . adult patients who develop symptoms often do so in the 5th or 6th decade [ 1 , 2 ] . common clinical presentations include dyspnea , congestive heart failure , angina , aneurysm , or myocardial infarction . whether or not a patient will develop symptoms is determined by the degree of fistula - induced volume overload , as well as the severity of left - to - right shunting secondary to fistula size and location . transesophageal echocardiogram may be useful in delineating the origin , course , and drainage of a fistula . therefore , in the presence of large shunts or even in asymptomatic patients , surgical or transcatheter ligation may be considered to prevent long - term sequelae such as steal , spontaneous rupture , heart failure , or myocardial ischemia [ 6 , 7 ] . transcatheter closure is first - line therapy in suitable anatomic cases due to lower cost , shorter recovery time , and reduced hospital stay . although coronary steal may have contributed to ischemia of the left bundle as evidenced by subsequent ecg findings , we can not exclude a rate - related bundle branch block . however , this is less likely since an ecg tracing 5 months earlier , at the same heart rate , revealed a normal qrs duration as did subsequent ecg tracings at similar heart rates . a right heart catheterization was not performed to assess filling pressures or to calculate a shunt fraction . even without direct measures of these pressures , it was still evident that the patient was in congestive heart failure by history and physical exam findings . he improved significantly with diuresis , suggesting elevated filling pressures also contributed to the supply - demand mismatch . the left ventricular systolic function may have also appeared decreased at presentation because of the septal motion abnormality from the lbbb . our patient , who presented with orthopnea , shoulder pain , and a new lbbb , illustrates that even a small coronary fistula may steal enough blood flow to mimic the symptoms of an acute myocardial infarction in the setting of a transient oxygen supply - demand mismatch . a coronary fistula , therefore , may not be the sole culprit lesion or an innocent bystander , but an accomplice in causing signs and symptoms of acute heart failure . the authors have no disclosures or financial conflicts of interest relevant to this paper to report .
coronary arterial fistulas are rare communications between vessels or chambers of the heart . although cardiac symptoms associated with fistulas are well described , fistulas are seldom considered in the differential diagnosis of acute myocardial ischemia . we describe the case of a 64-year - old man who presented with left shoulder pain , signs of heart failure , and a new left bundle branch block ( lbbb ) . cardiac catheterization revealed a small left anterior descending ( lad)-to - pulmonary artery ( pa ) fistula . diuresis led to subjective improvement of the patient 's symptoms and within several days the lbbb resolved . we hypothesize that the coronary fistula in this patient contributed to transient ischemia of the lad territory through a coronary steal mechanism . we elected to observe rather than repair the fistula , as his symptoms and ecg changes resolved with treatment of his heart failure .
the most common primary cardiac tumor is myxoma a benign neoplasm usually located in the left atrium . the most common malignant cardiac tumor is angiosarcoma , which can usually be found in the right atrium . due to scarce symptoms the factors which impede complete tumor removal include tumor location and growth causing infiltration of the right ventricle , the tricuspid valve , and the right coronary artery . the 40-year - old female patient was referred to the clinic with the diagnosis of a right atrial tumor . one month before , during a routine gynecological examination , the patient was diagnosed with a uterine tumor . after preliminary diagnostics , the patient underwent hysterectomy with adnexectomy . based on the histopathological examination of the removed material , angiosarcoma was diagnosed . control abdominal ultrasonography revealed a tumor focus in the liver , while echocardiography revealed a tumor in the right atrium . two days after the procedure , the patient was admitted to the clinic of cardiac surgery to undergo heart surgery . echocardiography performed at admission revealed a tumor in the right atrioventricular furrow 43 20 18 mm in size , infiltration of the right atrium and right ventricle with possible involvement of the right coronary artery , and fluid in the pericardial sac . further preoperative diagnostics included magnetic resonance of the heart , which revealed a right atrial tumor involving the atrial wall , infiltrating around the right coronary artery , and herniating into the lumen of the right atrium , as well as fluid in the pericardium ( fig . 1 ) . magnetic resonance of the heart the procedure was performed via median sternotomy , in normothermia , with the use of extracorporeal circulation . after the pericardial sac was incised , the pressure pushed out 300 ml of bloody fluid . the opening of the pericardial sac visualized a large tumor growing out of the right atrial wall ( fig . a substantial portion of the tumor was removed from the right atrium . a fragment of the lesion , approx . 10 20 mm in size , was left in the vicinity of the right coronary artery due to the infiltration of the coronary artery and the right atrium . the defect of the atrial wall was reconstructed with a fragment of the pericardial sac 5 5 cm in size . a pericardial patch was sutured onto the resected tumor fragment to prevent bleeding . despite the use of 4.0 sutures and teflon patches , the prevention of bleeding was not successful due to the delicate structure of the neoplastic tissue . in order to stop further bleeding , a 4.8 4.8 cm dressing covered with human blood coagulation factors ( tachosil , manufactured by the swiss company nycomed ) hemostasis monitoring revealed that another fragment of the tumor remained unsecured , and another tachosil dressing ( 3 2.5 cm in size ) was placed over this fragment . control cardiac echo performed before discharge revealed a small amount of fluid in the pericardial sac with no signs of compression , considerable saturation of the right atrial wall , and contractility disorders of the right ventricle . histopathological examination revealed the same histological type of the removed tumor fragment as in the uterus angiosarcoma . the late detection of the neoplastic lesions precluded a complete resection of the tumor from the myocardium . if the resected tumor mass had not been secured correctly , the patient would have faced the risk of a dangerous hemorrhage and cardiac tamponade . the delicate structure of the tumor precluded the provision of efficient hemostasis with traditional surgical methods . when they come in contact with the patient 's blood , these substances form a fibrin network which makes the sponge adhere to the bleeding surface . the dressing has found a wide spectrum of applications in many fields of general surgery ( inhibiting bleeding from parenchymal organs ) , thoracic surgery ( preventing not only bleeding , but also pleural adhesions and air leaks ) , and cardiac surgery [ 6 , 7 ] . based on our observations , tachosil may be used to control the hemostasis of delicate vascular structures that can not be removed completely .
primary malignant cardiac tumors are rare and are usually detected at an advanced stage of disease . their location and infiltration often hinder surgical resection . tissue sarcomas , especially angiosarcomas , are composed of irregular and delicate vascular tissue . the resection of such tumors from the heart is associated with a high risk of life - threatening bleeding that can not be stopped with traditional surgical methods . we present a case report of the application of a dressing containing human fibrin and thrombin in order to prevent bleeding during the partial resection of advanced cardiac angiosarcoma in a 40-year - old patient .
mycotoxins are secondary metabolites of molds which are associated with certain disorders in animals and humans . in addition to being acutely toxic , some mycotoxins are now linked with the incidence of certain types of cancer , and it is this aspect which has evoked global concern over feed and food safety , especially for milk and milk products . aflatoxin m1 ( afm1 ) is a hepatocarcinogen found in milk of animals that have consumed feeds contaminated with aflatoxin b1 ( afb1 ) , the main metabolite produced by fungi of the genus aspergillus , particularly a. flavus , a. parasiticus , and a. nomius . about 0.36.2% of afb1 in animal feed serious health concerns , many countries have set maximum limits for aflatoxins , which vary from country to country . the european community prescribes that the maximum level of afm1 in liquid milk should not exceed 50 ppt . however , according to the us standard , the level of afm1 in liquid milk should not be higher than 500 ppt . there have been several studies on afm1 concentration in milk samples in different regions of the world and also in iran , but this study was done to evaluate the occurrence of afm1 in milk distributed in mashad in northeast of iran in order to evaluate the potential of changing the regional standard on afm1 contamination of milk . in this study the afm1 content of pasteurized milk samples in retail stores in mashad ( northeast of iran ) was determined in fall 2011 . forty - two pasteurized milk samples ( 1000 ml milk packets , heat treated at 7274.4 for 1520 ) from different brands were collected by simple random sampling method . the samples were transported to the laboratory in an insulated container at about 4c and analyzed upon arrival . most of the reagents used to detect afm1 were contained in the ridascreen test kit , which included microtiter plate coated with capture antibodies , afm1 standard solutions used for the construction of the calibration curve ( 1.3 ml each 0 , 5 , 10 , 20 , 40 , and 80 ppt ) , peroxidase - conjugated afm1 , substrate ( urea peroxidase ) , chromogen ( tetramethylbenzidine ) , and stop reagent contains 1n sulphuric acid . the quantitative analysis of afm1 in pasteurized milk samples was performed by competitive elisa ( ridascreen afm1 , r - biopharm ) procedure as described by r - biopharm gmbh . prior to analysis of the samples , the elisa method was validated to ensure data quality . validation of elisa was carried out by determination of recoveries and the mean variation coefficient for fresh milk spiked with different concentrations of afm1 ( 5 , 10 , 20 , 40 and 80 ppt ) . the upper creamy layer was completely removed by aspirating through a pasteur pipette and from the lower phase ( defatted phase ) 100 l was directly used per well in the test . one hundred l of the afm1 standard solutions ( 100 l / well ) and test samples ( 100 l / well ) in duplicate were added to the wells of microtiter plate and incubated for 60 min at room temperature in the dark . after the washing steps , 100 l of the enzyme conjugate was added and incubated for 60 min at room temperature in the dark . fifty l of substrate and 50 l of chromogen were added to each well and mixed thoroughly and incubated for 30 min in the dark . following the addition of 100 l of the stop reagent to each well , the absorbance was measured at 450 nm in elisa reader ( elx-800 , bio - tek instruments , usa ) . according to the ridascreen kit guidelines , the absorbance values obtained for the standards and the samples were divided by the absorbance value of the first standard ( zero standards ) and multiplied by 100 ( percentage maximum absorbance ) . therefore , the zero standard is thus made equal to 100% , and the absorbance values are quoted in percentages . the values calculated for the standards were entered in a system of coordinates on semilogarithmic graph paper against the afm1 concentration in ppt ( figure 1 ) . the equation of the trendline in figure 1 is as follows : ( 1)y=0.016x21.940x+91.34 . data were analysed using excel 2007 and results reported as mean sd . the calibration curve and trendline equation prepared using excel 2007 . the standard solutions of concentration from 5 to 80 ppt afm1 were used to find calibration / standard curve . figure 1 gives the calibration curve of standard solutions of afm1 with concentrations of 5 , 10 , 20 , 40 , and 80 ppt by elisa analysis . analytical results showed that the incidence of afm1 contamination in pasteurized milk samples was low . although 97.6% of the samples were contaminated with afm1 , the toxin concentration was lower than iranian national standard and fda limit ( 500 ppt ) and only in three ( 1.6% ) of the samples afm1 concentration was greater than the maximum tolerance limit ( 50 ppt ) accepted by european union and codex alimentarius commission . the minimum and maximum contamination level of afm1 was found to be 6.4 and 71.4 ppt , respectively . the mean sd afm1 level in the analyzed samples of pasteurized milk was 23 16 ppt . the mean afm1 concentrations in milk in european , latin american , and far eastern diets have been reported by the joint fao / who expert committee on food additives to be 23 , 22 , and 360 ng / l , respectively . thus , the observed mean afm1 concentration in mashad milk samples was as high as the european and latin american and much lower than those reported for the far eastern diets . on the other hand , several studies have been done to determine afm1 contamination of milk in iran ( table 3 ) . the incidence of afm1 observed in the present study was lower than the incidence of afm1 reported by other authors [ 817 ] , yet , in all studies , the averages of toxin concentrations are below 100 ppt . the variations may be attributed to differences in region , season , and especially analysis method . based on the above results , especially later studies in mashad , the present situation is hopeful and might represent the possibility of altering standard limit of afm1 concentration in milk in iran . we suggest reduction of the limit as low as 100 ppt for raw milk .
the aim of this study was to detect the amount of aflatoxin m1 ( afm1 ) in pasteurized milk samples in mashad in northeast of iran . for this purpose , 42 milk samples were collected from retail stores during fall 2011 and analyzed for afm1 by enzyme - linked immunosorbent assay ( elisa ) technique . all the analyses were done twice . results showed presence of afm1 in 97.6% of the examined milk samples by average concentration of 23 16 ppt and contamination level ranging between 6 and 71 ppt . the concentration of afm1 in all the samples was lower than the iranian national standard and food and drug administration limits ( 500 ppt ) , and , only in 3 ( 1.6% ) samples , afm1 concentration was more than the maximum tolerance limit ( 50 ppt ) accepted by european union and codex alimentarius commission . according to our findings and previous studies , afm1 contamination of milk is not a concern in this region , and the regional standard of afm1 contamination in milk might be changed to lower than 100 ppt .
mycosis fungoides ( mf ) is a cutaneous t cell lymphoma ( ctcl ) characterized by infiltration of skin with patches , plaques and nodules composed of t - lymphocytes . it has various stages , premycotic , patch , plaques , nodules , tumours and erythroderma . tumour demblee is a variant of tumour stage , which develops from normal skin without prior patch or plaque stage . a 55-year - old hindu male , tobacco farmer by occupation , presented with multiple infiltrated plaques and nodules of 3 months duration over the face and scalp . the nodules started from ears and spread all over the face and scalp , increasing in size and number . patient had a history of severe itching episodes for past three years which were not relieved by antihistaminics . there was history of episodes of fever , weight loss , anorexia , and nausea for the past 2 months . multiple infiltrated plaques and nodules with few erosions and foul smelling superficial ulcers were present over face and scalp . the infiltrated skin over forehead , nose and ear lobules and loss of eyebrows gave a leonine face appearance [ figures 1 and 2 ] . the nodes were discrete , non - tender , mobile , and firm in consistency . loss of hair was seen on scalp , eyebrow , and axillary region . nodules plaques and erosions over face investigations revealed a hemoglobin level of 8.6% gm and the erythrocyte sedimentation rate ( esr ) of 110 mm / hour . chest x - ray , lymph node aspiration cytology and bone marrow examination did not reveal anything abnormal . ultrasonography of abdomen and pelvis and computed tomography ( ct ) scan of head and neck , chest , abdomen , and pelvis were normal . histopathological examination of the excision biopsy from a nodule over face showed a lymphocytic infiltrate in the papillary dermis and around the hair follicle and pilosebaceous unit . epidermotropism was noted with formation of well defined pautrier 's microabscess at places [ figure 4 ] . biopsy showing epidermotropism ( h and e , 40 ) biopsy showing clusters of atypical lymphocytes within the epidermis ( pautrier microabscesses ) ( h and e , 400 ) immunohistochemistry showed positive cd3 and lca markers as and cd 30 and cd 20 negativity . he termed it mycosis fungoides because of the resemblance of the lesions to mushrooms . in 1885 , vidal and brocq described mycosis fungoides d emblee for a patient presenting with skin tumours not preceeded by patch or plaques . in this type of mf mf is the most common type of ctcl and accounts for almost 50% of all primary cutaneous lymphomas . however other lymphoproliferative disease also involve the skin including ki-1 + anaplastic large cell lymphoma , peripheral t - cell lymphoma , cutaneous b - cell lymphoma , adult t - cell leukaemia/ lymphoma , t - cell lymphoid leukaemia and cutaneous hodgkin 's disease . incidence of mf has been estimated to range from 0.06 to 0.1 per 10,000 cancer cases per year in the usa . the term tumour d emblee is now falling into disrepute and these tumors may , in fact , be pleomorphic cd 30 negative cutaneous t - cell lymphoma ( peripheral t - cell lymphoma ) , which have undergone large cell transformation . many of these cases are likely to be classified by immunophenotyping as various types of non - mf t - cell lymphoma or even b - cell lymphoma of the skin . such type of mf d emblee has been reported rarely in past.[1214 ] many cases described as the demblee variant in the past may have represented other types of lymphomas . the cd 30 negative large ctcl and small/ medium sized pleomorphic ctcl have been described in literature to be presenting with tumors without prior or concurrent patches or plaques along with histological presentation sometimes similar to that of mf . the cd 30 negative large ctcl ( 5 year survival of 15% ) has a poor prognosis compared with small/ medium sized pleomorphic ctcl ( 5 year survival of 60% ) . usually the mean interval between appearance of skin lesions and definite diagnosis by histopathology is approximately 6 years , however , in our case it was only 3 - 4 months . the patient was treated with chop regimen [ cyclophosphamide , hydroxydaunorubicin ( doxorubicin ) , oncoverin ( vincristine ) , prednisone ] plus methotrexate . taking into account that this case of tumour d emblee also showed typical histopathological changes along with cd30 negativity and the eventual death of the patient within short span of time after the diagnosis , it is possible that the patient had cd 30 negative large ctcl , which could not be confirmed owing to limited resources in our hospital set up . this case is reported because of an acute and masquerading presentation of mycosis fungoides reiterating the fact that ctcl can pose an enormous diagnostic challenge .
mycosis fungoides is a cutaneous t - cell lymphoma characterized by infiltration of skin with patches , plaques , and nodules composed of t - lymphocytes . it is the most common type of cutaneous t - cell lymphoma and accounts for almost 50% of all primary cutaneous lymphoma . tumour d emblee is the term used for the patient presenting with skin tumors not preceded by patches or plaques . we report a rare case of mycosis fungoides d emblee variant with tumors of only 3 months duration without any preceding skin lesions .
pulmonary calcification , typically asymptomatic , can be caused by a number of diseases , most common being end - stage renal disease . we describe a case of pulmonary calcifications associated with chronic kidney failure , which has been improved by medical treatment . a 21-year - old man with a 4-year history of end - stage renal disease , on hemodialysis ( three 4-h sessions / week ) , secondary to untreated bilateral vesico - ureteral reflux and neurogenic reactive bladder . the patient was referred for a bone scan because of osteoarticular complaints , especially at the elbows with limited mobility , and he reported mild and nonspecific symptoms , especially no significant dyspnea . patient had high creatinine ( 428 mol / l ) ; serum phosphorus ( 4.51 mmol / l ) ; total alkaline phosphatase , 108 u / l and serum parathyroid hormone level was 1700 pg / ml . whole body bone scintigraphy images [ figure 1 ] , showed a diffuse uptake in both lungs , more intense in the right one , suggesting an extra - osseous pulmonary calcification and an increased radiotracer uptake in the soft tissues of the left arm and in the periarticular soft tissues surrounding the elbows . whole body bone scintigraphy with anterior and posterior projection performed 2 h after the injection of 18 mci ( 666 mbq ) of tc - methylene diphosphonate which showed a diffuse uptake in both lungs and an increased radiotracer uptake in the soft tissues of the left arm and in the periarticular soft tissues surrounding the elbows ( the hand positions are due to the limited mobility of the elbows ) chest radiograph performed in the light of the data of bone scan parathyroidectomy was declined by the patient . the medical therapeutic was chosen with dietary phosphorus restriction , noncalcium phosphate binders , calcimimetics , optimal control of secondary hyperparathyroidism , and intensive hemodialysis with a low - calcium dialysate . second bone scan was done 3 months later [ figure 3 ] revealed a significant decrease , without disappearance , in the lung 's uptake . second whole body bone scintigraphy with anterior and posterior projection performed 2 h after the injection of 18 mci ( 666 mbq ) of tc - methylene diphosphonate revealed a significant a decrease in the lungs uptake ( the hand positions are due to the limited mobility of the elbows ) uremic tumoral calcinosis predominates in periarticular soft tissues with preservation of the bone and joint structures . the clinical symptoms of mpc are usually mild that 's why this pathology is rarely diagnosed and patients with extensive calcification may be asymptomatic . chest x - ray findings in mpc are nonspecific and they are frequently normal . the diagnosis is confirmed by biopsy , but can be suspected by typical findings on a tc-99m - methylene diphosphonate bone scan , which is a sensitive and specific method for diagnosing . that why , some authors have recommended the use of bone scan in hemodialysis patients with pulmonary symptoms . finally , low glomerular filtration rate can cause hyperphosphatemia and an elevated calcium - phosphorus product . for patients with hyperparathyroidism , our patient declined parathyroidectomy and was treated medically with a significant regression of scintigraphic abnormalities .
pulmonary calcification is a subdiagnosed metabolic lung disease that is commonly asymptomatic and frequently associated with end - stage renal disease . we report a case of a 21-year - old man with a 4-year history of end - stage renal disease without respiratory symptoms . we discover incidentally on a bone scan a pulmonary calcification . parathyroidectomy was refused by the patient . after 3 months of medical treatment , a second bone scan was done , and we found a partial response .
healthy oral mucous membrane is normally of varying shades of red . when either the patient or the clinician notices areas of pigmentation , there is often an element of increased concern . focal lesions usually need an in - depth examination to exclude a melanoma , while diffuse lesions often have no specific histological features and do not generate prognostic perplexity . however , diagnosis of these lesions is important because they could be a sign of diseases with systemic implications such as peutz - jeghers syndrome or adrenal insufficiency . melanin is produced by melanocytes in the basal layer of the epithelium and is transferred to adjacent keratinocytes via membrane - bound organelles called melanosomes . the term melanoacanthoma was first used by mishima and pinkus in 1960 to describe a benign mixed skin tumor composed of basal and prickle cell keratinocytes and pigment - laden dendritic melanocytes . oral melanoacanthoma ( oma ) is a rare , benign pigmented lesion , brown to brown - black , well circumscribed , characterized by hyperplasia of spinous keratinocytes and dendritic melanocytes . we present a rare case of diffuse oral pigmentation which led us to diagnose it as oma . a 22 year old female patient came to our institution vaidik dental college and research centre , daman with a complaint of black pigmentations in the mouth since 2 months . patient reported that she was apparently alright 2 months back when she noticed black pigmentations in the mouth . the patient was concerned whether it was normal or some disease and so the patient reported to our institute . intraoral examination revealed brown black macular pigmentations widespread along the posterior part of left and right buccal mucosa [ figure 1 ] . it also showed involvement of retromolar areas , hard palate , soft palate and fauces . there was marked acanthosis with diffuse distribution of dendritic melanocytes in suprabasal layers of epithelium [ figure 2 ] . immunohistochemistry revealed diffuse nuclear and cytoplasmic immunoreactivity with for s-100 protein [ figure 3 ] . diffuse pigmentation involving hard palate , soft palate , bucal mucosa and retromolar areas marked acanthosis ( hematoxylin and eosin . the differential diagnosis of diffuse oral pigmentation include peutz - jeghers syndrome , addison 's disease , pigmentation due to smoking and medications , laugier - hunziker syndrome and oma . peutz - jeghers syndrome is an inherited , autosomal dominant disorder with variable inheritance , characterized by hamartomatous polyps in the gastrointestinal tract , mostly in the small bowel , and pigmented mucocutaneous lesions . in the present case , the patient had no previous or family history of any kinds of pigmented lesions or gastric intestinal polyposis . diffuse pigmentation of the oral mucosa and/or skin secondary to systemic drug administration is a well - recognized phenomenon . the patient did not give any history of medications including oral contraceptive and anti - malarial drugs . laugier - hunziker syndrome is an idiopathic macular hyperpigmentation of skin characterized by brownish black spots on oral mucosa including lips associated with longitudinal melanonychia of nails . although the pigmentation was diffuse and macular , the nails did not show any abnormalities . oma is a benign pigmented disorder of the oral mucosa , characterized by simultaneous proliferation of both melanocytes and keratinocytes . to emphasize the non - neoplastic nature of the disease , tomich and zunt suggested the term melanoacanthosis while reserving the designation melanoacanthoma for cutaneous tumors . the intra - oral site most commonly affected in oma is the buccal mucosa but involvement of other sites such as the mucosa of the lip , palate , gingiva and alveolar mucosa has also been reported . the clinical presentation is a brown to brown - black macular lesion , predominantly solitary , encountered in the younger age group with a distinct female predilection . oma is considered to be a reactive phenomenon and it has been suggested that masticatory and frictional trauma may play an etiological role . microscopically oma is characterized by the presence of numerous benign appearing dendritic melanocytes scattered throughout an acanthotic and mature squamous cell epithelium . in addition inflammatory cells with eosinophils may be present in the subjacent connective tissue stroma . an immunohistochemical analysis with melanocytic marker ( s-100 protein ) is an additional way to confirm . it is a benign condition and once the diagnosis is confirmed no treatment may be required . oma is a rare benign condition which must be distinguished from other diffuse pigmentations like peutz - jeghers syndrome , addison 's disease , pigmentation due to medication and laugier - hunziker syndrome . there is usually no treatment required in such cases and there have not been any cases of malignant transformation reported till date .
the clinical presentation of diffuse pigmentation can be alarming to the patient as well as the clinician . a histopathologic examination of a pigmented lesion is necessary in most of the cases in the oral cavity . oral melanoacanthoma is a very rare diffuse pigmentation with no specific treatment required . it shows increased number of dendritic melanocytes in an acanthotic epithelium . we present a rare case of diffuse pigmentation in the oral cavity whose diagnosis was done on the basis of clinical presentation and histopathology . also immunohistochemistry was done .
their occurrence was first described in 1670 by thilesus . however , at that time fistulas were a common complication of chronic and untreated cholecystitis . according to a 2005 study , 226 cases have been reported in total , with fewer than 25 in the last 50 years . the reduced incidence in current times can be attributed to more rapid diagnosis and treatment with antibiotics or surgery . although occurring in acalculous cholecystitis and carcinoma of the gallbladder , fistulas are still most commonly associated with gallstones [ 3 , 4 ] . obstruction of the cystic duct leads to an increase in gallbladder pressure and reduced perfusion with necrosis , which consequently causes gallbladder perforation . the contents of the gallbladder may then empty into the peritoneal cavity and an abscess may form or a fistula may develop through adherence to the duodenum , colon or abdominal wall , often via the fundus of the gallbladder . the right upper quadrant is the most common location for the exit tract of the fistula , but locations such as the gluteal region , umbilicus and right groin have also been documented . cholecystocutaneous fistulas are most often seen in elderly women over the age of 60 , likely due to coexistent disease and non - specific symptoms interfering with diagnosis . a white 85-year - old female with hypertension and a previous history of breast biopsy underwent endoscopic retrograde cholangiopancreatography with sphincterotomy after initially presenting on may 3 , 2011 with common duct stones . the patient was initially seen in the emergency department complaining of a 3-day history of sharp intermittent epigastric and right upper quadrant pain radiating to the central back . mild scleral icterus was noted , but there were no signs of jaundice or lymphadenopathy . her abdomen was soft , non - distended and mildly tender to palpation with a positive murphy 's sign . routine blood work demonstrated an elevated white blood cell count of 16.1 , no abnormalities on sma7 , elevated lipase > 3,000 , and elevated liver function testing including an alkaline phosphatase of 215 , a bilirubin of 41 , an ast of 100 , a ggt of 305 and an alt of 194 . clinical evidence of mild jaundice accompanied by blood work abnormalities and positive radiological signs led to the diagnosis of acute calculous cholecystitis , common bile duct stones up to 7 mm in size and biliary gallstone pancreatitis . she was treated conservatively with intravenous antibiotics and underwent endoscopic retrograde cholangiopancreatography with sphincterotomy for removal of several stones of various sizes . percutaneous cholecystostomy was then carried out for drainage of the gallbladder after development and medical control of atrial fibrillation . on june 1 , 2011 she was re - admitted to the hospital with a left lower lobe pulmonary embolism . on june 27 , 2011 the percutaneous drain was removed at her request . in early august 2011 , she re - developed right upper quadrant discomfort ; furthermore , she noted some purulent drainage from the percutaneous drain site and extrusion of approximately 30 gallstones . she had several follow - up ultrasounds which identified a fistulous tract measuring 0.78 cm in diameter communicating with the external opening in the right upper quadrant ( fig . an irregular hypoechoic area just inside the subcutaneous tissue measuring 4.1 2.7 cm was presumed to represent a contracted gallbladder . plans were made for laparoscopic cholecystectomy and management of her cholecystocutaneous fistula on february 22 , 2012 , once she finished her coumadin regiment . in the morning of the operation , on february 22 , 2012 , the patient 's inr was still elevated at 1.8 and the surgery was re - scheduled for a month later . on april 18 , 2012 the patient underwent laparoscopic cholecystectomy and fistula division ( fig . 2 ) . three additional gallstones were found in the gallbladder at the time of the operation . we present the case of an 85-year - old white female who was diagnosed with a cholecystocutaneous fistula that developed as a complication following removal of a percutaneous drain that was used to treat her acute cholecystitis . re - occurrence of her cholecystitis after drain removal and the presence of gallstones promoted the production of a fistula along the pre - existing tract of the drain . her concurrent treatment with anticoagulants for a pulmonary embolism delayed the definitive management of her cholecystitis and fistula . fortunately , the patient remained in reasonably good health throughout the waiting period from time of fistula diagnosis to surgery . more conservative approaches such as percutaneous cholecystotomy have been used in high - risk patients , leading to spontaneous closure of the fistula . however , in this case the fistula developed through the old drain tract , so surgical intervention was employed . as with uncomplicated cholecystitis , laparoscopic techniques are favorable compared to open surgery and thus a laparoscopic cholecystectomy was undertaken in this case . the gallstones removed during cholecystectomy were of orange - brown color consistent with cholesterol stones . although fistula formation is now a rare complication of cholecystitis , it remains a possibility and should be considered in the differential diagnosis of any fistulous tract in the right abdominal wall . we have demonstrated that previous percutaneous drainage of an acute gallbladder infection can promote the formation of such a fistula if the infection is not properly dealt with or re - occurs . physicians should be prepared to recognize this complication in patients after drain removal and prior to definitive surgery .
cases of cholecystocutaneous fistulas are now a rare occurrence as a result of rapid diagnosis and treatment . we present a case of cholecystocutaneous fistula developing after the removal of a percutaneous drain for the treatment of acute cholecystitis . re - occurring infection and presence of gallstones led to fistulization of the gallbladder fundus and the development of a tract along the path created by the drain . the patient presented with re - occurring right upper quadrant abdominal pain , purulent discharge from the fistulous opening and expulsion of multiple gallstones . she underwent laparoscopic cholecystectomy and fistula excision .
a 56-year - old man was hospitalized because of pain and numbness in his left hand since he slipped a week earlier . , we found that the motor and sensory functions of his left hand were normal . his laboratory data , including anti nuclear antibody , anti - neutrophil cytoplasmic antibody , and rheumatoid arthritis factor , were within normal limits . however , the laboratory analysis showed decreased protein c and s concentrations ( protein c antigen , 57% ; protein s antigen , 19% ) . the diagnostic work - up to determine the degree of lesion included a computed tomography ( ct ) angiography , which showed an intraluminal lesion of the left upper extremities vessel . in the ct scan , coincidently , we stumbled across a mass lesion on the aortic arch . for the evaluation of the incidental mass lesion , we performed a chest ct scan and transthoracic echocardiography . the ascending aorta and the aortic arch had intact intima and a normal size ( fig . 2 ) . in transthoracic echocardiography , we found a floating mass in the lesser curvature of the aortic arch ( fig . 3 ) . we decided to surgically remove this floating mass because of the risk of peripheral embolization , including thrombectomy for the brachial and radial artery occlusion of the left arm . a median sternotomy was performed , a venous cannula was inserted in the ra auricle , extracorporeal circulation was begun , and the central temperature was decreased to 25. the patient was then in total circulatory arrest . an incision was made in the aortic arch , and the 3.0-cm intraaortic mass was completely removed ( fig . the mass had no definite stalk , and its attachment site in the aorta was relatively normal . we also removed the thrombus of the left upper extremities through the brachial artery . in the postoperative peripheral angiography , one week later , the patient recovered without complications and was discharged on the regimen of warfarin . aortic thrombi , however , are another important cause of arterial thromboembolism . factors related to an arterial thrombus are arteriosclerosis , arterial dissection , trauma , malignant tumor , and hemostatic disorder . in this case , the patient had protein c and s deficiency , which induced a hypercoagulable disorder . the presence of pedunculated thrombi in the aortic arch as in this case is rare . the incidence of embolic events from mobile aortic thrombi is 73% . in this case , the patient had a thrombus in his left arm . they occur more commonly in patients of advanced age and those with several cardiovascular risk factors . in our case the most frequent location of thoracic aorta thrombi is the region of the aortic isthmus and the portion distal to the aortic arch , at the side opposite to the origin of the subclavian artery . ct and echocardiography can be used for the diagnosis of aortic thrombi . in particular , transthoracic and transesophageal echocardiography have high diagnostic accuracy and allow the assessment of the size , morphology , and anchoring site of the thrombus , as well as the characteristics of the aortic wall . further , to determine the cause of the thrombus , we should consider a survey for hypercoagulable disorder . a definite diagnosis requires histological and immunohistochemical studies . in a differential diagnosis with other mass lesions , such as tumors , the treatment of aortic thrombi is considered necessary because of the risk of a massive systemic embolization . thrombolysis can be a possible treatment , but there is a risk of thrombolytic agents selectively lysing the stalk of the lesion , releasing the bulk of the lesion into the systemic blood stream . we believe that in selective patients with acceptable surgery for cardiopulmonary bypass and definite systemic embolic events due to highly mobile aortic thrombi , surgical treatment has been successful . this case is reported in order to inform the readers of a rare case where floating thrombi in the aortic arch of patients with embolization were successfully treated surgically .
floating thrombi in the aortic arch are very rare and an unusual source of systemic embolism . herein , a case of a 3-cm thrombus in the aortic arch is reported . it was a floating , highly mobile thrombus attached to the lesser curvature of the aortic arch . the patients had a hypercoagulable disorder induced by protein c and s deficiency . the thrombus was operatively removed with a favorable outcome .
it is a benign melanocytic lesion typically < 2 mm in thickness , with an annual malignant transformation rate of one in 8,845.1 polypoidal choroidal vasculopathy ( pcv ) is a recurrent and relapsing chorioretinopathy characterized by grape - like subretinal vascular lesions associated with retinal pigment epithelium detachments ( peds).2 indocyanine green angiography ( icga ) is the gold standard for pcv diagnosis . however , patients with pcv are often mistaken for patients with exudative age - related macular degeneration and typical choroidal neovascularization ( cnv ) . we previously reported the first case in the literature of a stable pcv associated with nevus , which was managed conservatively.3 in this article , we performed a retrospective chart review following the written informed patient consent of a 78-year - old caucasian female , who had active , symptomatic pcv secondary to nevus , and was successfully treated with photodynamic therapy ( pdt ) . this case involved a 78-year - old caucasian female with a stable left - eye superotemporal extrafoveal pigmented nevus for 20 years . funduscopy showed a pigmented lesion measuring 4.83.2 mm in basal dimensions with overlying clumped soft drusen at the posterior pole along the 2 oclock meridian ( figure 1a ) , corresponding to a nevus at 2.0 disc diameters from fovea . a discreet orange nodule adjacent to the pigmented lesion on the nasal aspect and associated subretinal fluid ( srf ) was noted . optical coherence tomography ( heidelberg engineering , heidelberg , germany ) showed typical features of a flat nevus with ped associated with an underlying discreet polyp - like lesion at the nasal edge of the nevus and extensive srf ( figure 1d ) . fluorescein angiography ( figure 1b and c ) and icga ( heidelberg engineering ; figure 2a and b ) demonstrated early filling of a grape - like structure suggestive of pcv with leakage in the late phase . icga also revealed an associated small branching vascular network ( bvn ) in both early and late phases ( figure 2a and b ) . a diagnosis of pcv adjacent to nevus was made . due to the peripheral location of pcv and the presence of srf threatening the fovea intravenous verteporfin ( 6 mg / m ) was followed by standard - fluence pdt ( 50 j / cm ) at the center of the pcv with a spot size of 1,600 m over 83 seconds from a 689 nm laser . following treatment , srf was absent ; however , a persisting small ped was observed ( figure 1f ) . icga at 7 months showed an absence of leakage , with complete regression of the polypoidal lesion ( figure 2c and d ) . no active polyp could be detected on icga at 2 years , with a stable best - corrected visual acuity of 6/6 ( 0.0 logmar ) . studies have found that patients with choroidal nevus may develop typical cnv.47 however , pcv secondary to nevus has rarely been reported . we previously reported a case of a quiescent pcv arising from a stable choroidal nevus where the polyp was located above the nevus , however , between the retinal pigment epithelium ( rpe ) and bruch s membrane . this location of the pcv corresponded to a study by uyama et al on icga findings of japanese patients with pcv , in which they proposed that pcv may be a peculiar form of cnv beneath the rpe and above the bruch s membrane.8 similar to this , histopathological examinations revealed that vessels found at the margins of type-1 choroidal neovascular membranes tend to be matured and dilated , and therefore , pcv seemed to originate from longstanding type-1 ( occult ) cnv above bruch s membrane and is not a primary choroidal vascular disorder.9 we postulated that a choroidal nevus may cause chronic inflammatory or degenerative changes overlying rpe , which may result in the growth of type-1 cnv and eventually lead to pcv lesions developing adjacent to the nevus.3 in our case , optical coherence tomography and angiography revealed a discrete orange grape - like lesion below the ped with early filling and leakage in the late phase associated with a bvn and an srf , which were more typical of active pcv . some may argue the current case to represent malignant transformation of choroidal nevus , since srf was observed with an orange structure , which could be interpreted as pigment associated with choroidal melanoma . however , the pigmented lesion was flat , and the orange structure was adjacent to , not overlying , the lesion . moreover , our case responded well to pdt being applied at the center of the pcv , not over the pigmented lesion , and has remained stable for 2 years post - pdt . these features support our case to be pcv secondary to choroidal nevus rather than malignant transformation . various treatments including thermal laser therapy have been used for pcv.1015 icga - guided pdt was found to be effective in treating cnv associated with choroidal nevus ; however , variable outcomes have been reported.11 pdt is also one of the most effective treatments for pcv , resulting in acuity improvement , leakage reduction , and complete pcv regression.13,14 however , a high recurrence rate and minimal bvn regression have been documented , with possible complications such as subretinal hemorrhage and rpe tears.16 the favorable outcome of pdt in our case was most likely due to the extrafoveal location of pcv and the patient s good initial acuity . in pcv patients , vascular endothelial growth factor ( vegf ) was found to be elevated in rpe and vascular endothelial cells.17 intravitreal anti - vegf injections for pcv have been shown to be effective in reducing srf and improving vision ; however , vascular abnormalities have often persisted.12,15 a previous study reported that combination treatment with pdt and anti - vegf resulted in better acuity outcomes and a lower risk of developing pdt complications than photodynamic monotherapy.15 lowering vegf levels after pdt may be the key to preventing pcv recurrence and cnv development . however , koh et al reported that in follow - up visits of up to 6 months , although pdt combined with ranibizumab was superior to ranibizumab monotherapy in achieving complete regression of pcv , no difference was found between the combination treatment and the photodynamic monotherapy.12 in this report , we present the case of a caucasian female who developed symptomatic , active pcv at the edge of a stable choroidal nevus , which was successfully treated with one session of pdt , resulting in improved symptoms and fluid resolution , and no further treatment was required . icga is invaluable for diagnosing pcv and differentiating pcv from typical cnv , as pcv is a highly variable disorder . further studies are required to understand the mechanisms of pathogenesis and evaluate optimal treatment options .
we report a case of a caucasian female who developed active polypoidal choroidal vasculopathy ( pcv ) at the edge of a stable choroidal nevus and was successfully treated with verteporfin photodynamic therapy . no active polyp was detectable on indocyanine green angiography 2 years after treatment , and good vision was maintained . indocyanine green angiography is a useful investigation to diagnose pcv and may be underutilized . unlike treatment of choroidal neovascularization secondary to choroidal nevus , management of pcv secondary to nevus may not require intravitreal anti - vascular endothelial growth factor therapy . photodynamic monotherapy may be an effective treatment of secondary pcv .
the utilization of multiple pharmacologic agents is an essential component of modern day anesthetic practice . while there have been numerous advancements in recent years in both analgesic and amnestic medications available for an anesthesiologist to use in clinical practice , the cadre of neuromuscular blocking agents available in the united states has been stagnant . the ideal neuromuscular blocking agent is one that is rapidly acting , has minimal to no adverse effects , is independent of end organ metabolism , and allows for rapid and complete reversal of neuromuscular blockade . first , the deleterious effects of residual neuromuscular blockade in the postanesthetic care unit have been well studied and are clinically relevant . emphasis on operative efficiency and patient discharge has also been widely identified as an area for potential cost saving measures in modern healthcare settings . novel drug development has been proven to be a difficult and timely process as it has been over 20 years since a new nondepolarizing muscle relaxant has been introduced for clinical use . this paper will highlight some of the latest pharmacological advancements in the area of neuromuscular blockade . the enantiomers gantacurium and cw002 are two of the most recent neuromuscular blocking agents that have shown potential for clinical application . the appeal of these molecules is the ultra - rapid reversal of neuromuscular blockade via cysteine adduction and minimal systemic hemodynamic alterations with administration . gantacurium is an asymmetric alpha - chlorofumarate and is classified as an ultra - short acting nondepolarizing neuromuscular blocker . its pharmacologic properties have been established using both animal and human models with its ed95 found to be 0.19 mg / kg . maximum neuromuscular blockade using gantacurium was found to be within 90 s following administration of 1.5 ed95 with even faster onset at higher doses . this pharmacologic profile is comparable to that of succylincholine and could eventually serve as a replacement for a rapid depolarizing muscle relaxant . gantacurium chemical structure cw002 differs in structure from gantacurium by being symmetrical and lacking a chlorine at the fumarate double bond . these properties give cw002 a greater potency than gantacurium and an intermediate duration of action of approximately 30 min . using both animal and human models , , cw002 has minimal to no hemodynamic effects at administered doses well above its documented ed95 . the first is a slow ph - sensitive hydrolysis at the ester linkages of the molecules . this results in a t of 56 min and 495 min for gantacurium and cw002 , respectively . the second pathway for inactivation is much more rapid and has the greatest clinical implications . l - cysteine adduction results in a byproduct of extremely low potency that also subsequently undergoes hydrolysis to form inactive molecules . furthermore , of importance is that unlike conventional neuromuscular blockers , this pathway allows for complete reversal at any time after bolus administration of neuromuscular blockers . l - cysteine adduction terminates the relaxants action via inactivation and not by overcoming competitive inhibition . advancements in neuromuscular blocking agents have the potential to have significant impact on anesthetic care in the united states . the ability to rapidly and reliably induce and reverse favorable conditions for tracheal intubation and surgery can profoundly impact anesthetic care in ambulatory , inpatient , and emergent settings . all anesthesia providers will need to consider some of the advantages and potentially disadvantages of these new drugs in their practice in the future . this paper details some of the promising medications on the horizon for clinical use . continued research is needed in the most important area of neuromuscular modulation in clinical practice .
pharmacological advances in anesthesia in recent decades have resulted in safer practice and better outcomes . these advances include improvement in anesthesia drugs with regard to efficacy and safety profiles . although neuromuscular blockers were first introduced over a half century ago , few new neuromuscular blockers and reversal agents have come to market and even fewer have remained as common clinically employed medications . in recent years , newer agents have been studied and are presented in this review . with regard to nondepolarizer neuromuscular blocker agents , the enantiomers gantacurium and cw002 , which are olefinic isoquinolinium diester fumarates , have shown potential for clinical application . advantages include ultra rapid reversal of neuromuscular blockade via cysteine adduction and minimal systemic hemodynamic effects with administration .
the ingestion of instruments or materials used in various dental procedures may occur accidentally in dentistry . foreign bodies vary in size and shape and range from burs , posts , root pieces , teeth , orthodontic brackets , endodontic instruments ( files , broaches ) , impression materials , implant components , and restorations [ 1 , 2 ] . some objects are made of materials that lack radiopacity , which makes them impossible to identify and locate ; diagnostic bronchoscopy / endoscopy or computed tomography for localization is then required [ 3 , 4 ] . foreign body ingestion is a commonly seen accident in emergencies , usually in children ( 80% ) , elderly , mentally impaired , or alcoholic individuals ; whereas it may occur intentionally in prisoners or psychiatric patients [ 58 ] . rarely foreign body ingestion results in serious complications , such as intestinal perforation , bleeding , obstructions or impactions . the patient position in the dental chair as well as the patient s medical history is important in preventing serious complications . usually instruments entered the gi tract pass asymptomatically and atraumatically within 2 days to 4 weeks . however there are many potential sites for impactions , among them the ileocecal valve is the most common site . approximately seventy five percent of perforations occur at or near this site or at the rectosigmoid junction [ 6 , 9 ] . only 1 percent of ingested foreign bodies cause an intestinal obstruction requiring surgery [ 10 , 11].the aim of this article is to document implant screwdriver ingestion along with its consequences and to offer guidance for prevention and management . a 69 year - old male patient presented with a chief complaint of missing upper left second molar and mandibular left first molar teeth ; for which implant placement was planned . implant placement was done successfully and patient was recalled after 4 months for second stage surgery . during second stage surgery while placing a gingival former on the implant in the region of upper left second molar , the screw driver accidentally slipped from the operator s hand . the operator made an unsuccessful attempt to retrieve the instrument by making the patient spit . the case was attended by a gastroenterologist and immediately pa chest and abdominal radiograph were taken confirming the screwdriver to be in stomach ( fig 1 ) . an endoscopy was planned , which was performed under local anesthesia but the instrument could not be retrieved . patient was kept under observation and advised to consume fiber - rich food to enhance intestinal motility and to regularly examine his stools . on the second day again radiographs were repeated and this time screwdriver was located in the intestine ( fig 2 ) ; the very same day the patient passed the ingested screwdriver in his stool ( fig 3 ) . it must be emphasized that preventing complications of foreign body ingestion and aspiration is of great importance . this applies to the identification of at risk patients by means of comprehensive clinical examination and thorough patient history taking . in our case the patient was senile and apprehensive ; there was difficulty in screwing the gingival former in the maxillary posterior region of upper left second molar , from where the instrument slipped . as the patient was in the supine position direct access to oropharynx was eminent although rare , ingestion / inhalation of a foreign body may lead to serious complications ; therefore , immediate radiographic evaluation is a must including pa / lateral chest , lateral neck and abdominal radiographs . usually , most ingested foreign bodies are expelled in stools without causing any complications in several days to several weeks ; for which conservative management of serial radiographs and fiber rich diet is an initial protocol [ 14 , 15 ] . if serial radiographs depict the same location of foreign body or there is a sign of abdominal tenderness or hemorrhage then most likely there is retention , obstruction or intestinal perforation for which an invasive procedure is indicated and has to be done as early as possible through gastroscopy for its retrieval . the mallampati score is assessed by asking the patient ( in a sitting posture ) to open his / her mouth and protrude the tongue as much as possible .the anatomy of the oral cavity is visualized ; specifically , whether the base of the uvula , faucial pillar and soft palate are visible . depending on whether the tongue is maximally protruded and/or the patient asked to phonate , the scoring may vary . class iv : only hard palate visible it is emphasized that all practitioners should take preventive steps during treatment like placement of gauze screen across the oropharynx , tying ligature ( dental floss ) to instruments , adjusting chair position ( sitting position prevents aspiration / ingestion and supine position increases the risk of swallowing ) and should be able to manage the patients in emergency situations . sending a patient home in the belief that a foreign body that slipped into the oropharynx has been swallowed and should a foreign body be ingested / aspirated , the patient must be examined clinically and radiographically ; diagnosis must be performed immediately by a specialist and patient and his family members must be informed . class iv : only hard palate visible it is emphasized that all practitioners should take preventive steps during treatment like placement of gauze screen across the oropharynx , tying ligature ( dental floss ) to instruments , adjusting chair position ( sitting position prevents aspiration / ingestion and supine position increases the risk of swallowing ) and should be able to manage the patients in emergency situations . sending a patient home in the belief that a foreign body that slipped into the oropharynx has been swallowed and will pass through the gut may be associated with complications and lead to litigation . should a foreign body be ingested / aspirated , the patient must be examined clinically and radiographically ; diagnosis must be performed immediately by a specialist and patient and his family members must be informed . this case report illustrates a case of foreign body ingestion in an anxious senile patient having mallampati class 1 , during implant restoration in the posterior maxilla ; proper preventive measures may prevent such complications .
one of the complications during a routine dental implant placement is accidental ingestion of the implant instruments , which can happen when proper precautions are not taken . appropriate radiographs should be taken to locate the correct position of foreign body ; usually the foreign body passes asymptomatically from gastrointestinal tract but sometimes it may lead to intestinal obstruction , perforations and impactions . the aim of this article is to report accidental ingestion of 19 mm long screw driver by a senile patient .
non - hodgkin 's lymphomas ( nhl ) primarily involving the orbit , is relatively uncommon . we report a case of orbital lymphoma in a 62-year - old male with rare histopathological findings secondary to transformation of once cell type into another . tissue diagnosis and molecular studies led to revelation of diffuse large b cell lymphoma evolving from malt lymphoma . proliferation of two morphologically and phenotypically different b cells resulting in malignancy has not been found in the orbit so far . the orbit is a rare primary site for non - hodgkin 's lymphoma ( nhl ) , accounting for 1% of primary presentations and an estimated 5 - 14% of all extra nodal presentations . however , lymphomas are the most common primary orbital tumor in adults 60 years of age and older . transformation from marginal zone mucosa - associated lymphoid tissue ( malt ) lymphoma to a different form of lymphoma is a very rare occurrence , and has not been previously described in orbital lymphomas . we present a case of bilateral orbital malt lymphoma that transformed into a diffuse large b - cell lymphoma ( dlbl ) , both of which were incidentally found to be coexistent at the time of diagnosis . a 62-year - old caucasian male with a past medical history of hypertension and diabetes , presented with bilateral eye protrusion since 1 week . he had been experiencing double vision and throbbing frontal headaches for the past 2 weeks . physical examination was remarkable only for bilateral exophthalmos [ figure 1a ] , without any palpable lymphadenopathy . magnetic resonance imaging ( mri ) of the orbit revealed 3.8 3.0 1.5 cm enhancing orbital mass involving the lateral and superolateral aspect of the peripheral right orbit as well as an irregular 1.1 1.1 2.4 cm mass along the inferior aspect of left orbit [ figure 1c ] . also noted were abnormal signal enhancements within the lesser sphenoid wing and the anterior body of the mandible on the right . given this constellation of findings , there was suspicion for lymphoma or other metastatic disease . ( a ) photograph of the patient showing bilateral swelling and proptosis of the eyes , more prominent on the right side . ( b ) photograph of the patient after 1 month of chemotherapy , showing significant improvement on both sides . red arrows showing lateral hyperintense infiltration in both orbits biopsy of right eye mass was done that revealed extensive soft tissue involvement by two separate lymphomatous populations [ figure 2a and b ] , comprising small and large neoplastic lymphocytes . the large cells were positive for cd20 b cells with co - expression of cd10 bcl6 and mum-1 . by corresponding flow cytometry the small lymphoma cells were cd20 + b cells with co - expression of bcl2 and aberrant cd43 and partial cd5 . b cell immunoglobulin gene rearrangement by polymerase chain reaction ( pcr ) was done to detect clonality . peaks migrating at 318 , 255 , and 274 bases were present on b - cell immunoglobulin heavy chain ( igh ) . in addition , there were peaks present migrating at 195 and 273 bases on b - cell immunoglobulin kappa light chain ( igk ) . gene rearrangement studies were also done on the different aberrant lymphocyte population with diagnosis of malt lymphoma . those areas also shared common peaks at 318 and 255 bases in igh as well as 273 bases in igk light chain . ( a ) low magnification view of lacrimal gland histopathology , showing two morphologically different lymphocytes . ( b ) high power magnification shows malignant large lymphocytes in the lower half with abundant cytoplasm , round nuclei with prominent nucleoli and occasional mitoses . seen in the upper half of the figure are small lymphoid cell with a narrow rim of clear cytoplasm and condense chromatin . this is consistent with malt lymphoma metastatic work - up with computed tomography ( ct ) scans and positron emission tomography ( pet ) scan was negative . he was started on rituximab and cyclophosphamide , doxorubicin , vincristine , and prednisone ( chop ) . by the time of his 1-month follow - up , the orbital swelling had disappeared repeat pet is negative for any metastatic disease , and only residual disease is present in the orbit . orbital lymphomas ( ol ) usually present with eyelid swelling , palpable eyelid mass , diplopia , proptosis , and lid erythema . the most commonly infiltrated structures are found within the superior - lateral quadrant , such as the superior rectus muscle , lateral rectus muscle , lacrimal gland , and eyelid . systemic lymphoma has been diagnosed in 67% of patients with bilateral orbital tumor and in 34% with unilateral orbital tumor . majority of the ol are malt type lymphomas , accounting for 40% to 70% of the cases . although initially described in gastric mucosa and in association with helicobacter pylori infection , malt lymphomas have subsequently been observed to arise in other epithelial structures , including the thyroid , parotid gland , lung , uterus , and breast , as well as in the orbit . age greater than 60 years , elevated lactate dehydrogenase ( ldh ) , and coexistent other malignancies , have been identified as independent risk factors for transformation into aggressive lymphomas . management of orbital lymphoid tumors includes surgical excision , chemotherapy , immunotherapy , or radiotherapy , depending on the size , shape , location , and grade of the lesion and on the systemic status of the patient .. some aggressive tumors may require chemo - immunotherapy as in this case . what is unusual about our case is that it combines two morphologically and phenotypically different mature b - cell lymphomas arising in the same tissue . in the pathological sample it may represent either two different co - existent lymphomas or a transformation from one to the other . in this case , immunoglobulin gene rearrangement studies revealed common peaks at 318 bases and 254 bases in the igh , and 273 bases peak in the igk light chain , in both malt lymphoma as well as dlbl . these findings suggest a common initial clonal process evolving from malt lymphoma to a diffuse large b cell lymphoma with light chain switch ( from lambda to kappa ) . transformation of malt lymphoma is a rare event , and there are only a few cases of this occurrence that have been reported , but to our knowledge , no such pathological transformation has been identified in the orbit .
context : non - hodgkin 's lymphomas ( nhl ) primarily involving the orbit , is relatively uncommon . rarely two pathologically different nhl cell types have been found to be coexistent.case report : we report a case of orbital lymphoma in a 62-year - old male with rare histopathological findings secondary to transformation of once cell type into another . tissue diagnosis and molecular studies led to revelation of diffuse large b cell lymphoma evolving from malt lymphoma.conclusion:proliferation of two morphologically and phenotypically different b cells resulting in malignancy has not been found in the orbit so far . they are usually aggressive tumors and require chemo - immunotherapy .
a 64-year - old male was admitted with sudden onset of right - sided hemiparesis , headache , gait disturbance , and recurrent vomiting . the patient regularly took amlodipine besylate ( 5 mg once a day ) for his blood pressure , but nothing for his diabetes . on examination , his pulse was regular , his blood pressure was 160/100 mmhg , and his temperature and respiratory rate were normal . a neurological examination revealed right - sided hemiparesis ( mrc grade : upper / lower , iv / iv ) , right horner syndrome , and ataxia of his right limbs . on sensory examination , pinprick and temperature sensations were decreased on the right side of his face and the left side of his body . position and vibration senses were also decreased on the right side of his body . on the second day in hospital , his right side became hemiplegic ( upper / lower , ii / ii ) with flaccid tone and hyporeflexia . during the next 7 days , his tone returned , the deep tendon reflex increased , and he developed a right - side babinski response . diffusion - weighted and t2-weighted brain mri performed 24 hours after the onset of his condition revealed a high - intensity area in the right lateral medulla extending from the rostral medulla to the upper cervical cord , and in the right cerebellum in the territory of the medial branch of the posterior inferior cerebellar artery ( pica ) ( fig . 1 ) . mr angiography disclosed suspicious narrowing of the proximal and distal portions of the right vertebral artery and hypoplasia of the left vertebral artery ( fig . low - dose aspirin ( 100 mg once a day ) and clopidogrel ( 75 mg once a day ) were initiated for secondary prevention . on the basis of the mr angiography findings , we recommended digital subtraction angiography and angioplasty , but the patient refused any other treatment modalities . the patient was discharged 36 days after the ischemic accident with hemiparesis ( upper / lower , iii / iii ) , ataxia of his right side , and residual sensory impairment . ipsilateral hemiparesis with symptoms and signs of lateral medullary infarction were first described by opalski in 1946.1 he reported two patients with ipsilateral hemiplegia , ataxia , horner syndrome , diminished facial sensation , and diminished superficial sensation of the contralateral side . pathologic and neuroradiologic findings have identified the causal lesion of opalski 's syndrome.2,3 in the present case , the lesion causing ipsilateral hemiparesis was located in the upper cervical cord , also involving the corticospinal tract below the decussation . the arterial supply of the medulla arises from the vertebral artery , pica , and anterior and posterior spinal arteries . the pica and vertebral artery supply the lateral medullary area , and branches of the vertebral artery are distributed to practically the entire lateral medullary region between the medullary pyramids and the fasciculus cuneatus at the caudal medullary level . norrving and cronqvist examined the pattern of vascular occlusion in lateral medullary infarctions , and found that the most common vascular lesions involved the vertebral arteries.5 the relative sizes of the vertebral arteries vary considerably , and in approximately 10% of cases one vessel is so small that the other is essentially the only artery supplying the brainstem and cerebellum . when the one vertebral artery responsible for supplying the major source of the blood flow is occluded , the resulting infarction is more severe than in the case of bilaterally competent vertebral arteries.6 therefore , in the present case we may consider that the right vertebral artery was responsible for supplying the major source of blood flow and that an artery - to - artery embolism with right vertebral arterial atherosclerosis resulted in coexisting cerebellar and medullary lesions extending to the rostral cervical cord . several previous reports of opalski 's syndrome2 - 4 attributed these clinical observations to observed focal ischemic lesions of the medulla . dhamoon et al . reported one autopsy case with severe atherosclerosis and thrombosis in the proximal and distal sections of the right vertebral artery in opalski 's syndrome.2 in the present case , acute ischemic lesions extended to the rostral medulla and encompassed the medial pica territory of the cerebellum , and mr angiography showed suspicious severe stenosis or near occlusion of the right vertebral artery , and hypoplasia of the left vertebral artery . conventional angiography and angioplasty could not be performed due to patient 's refusal . in addition , considering that intrinsic pica ( in situ branch artery ) disease can also cause lateral medullary lesion with multiple cerebellar involvement,7 we can not exclude the possibility of in situ pica occlusion .
a 64-year - old man presented with sudden onset of right - sided hemiparesis , headache , gait disturbance , and recurrent vomiting . a physical examination revealed right - sided hemiparesis , right horner syndrome , ataxia of the right limbs , and diminished sensation on the left side of his body . diffusion - weighted mri revealed an acute right lateral medullary infarction extending from the rostral medulla to the upper cervical cord , and an acute cerebellar infarction in the territory of the medial branch of the posterior inferior cerebellar artery . magnetic resonance angiography revealed suspicious severe stenosis or near occlusion of the proximal and distal parts of the right vertebral artery , and hypoplasia of the left vertebral artery . we diagnosed ipsilateral hemiparesis with lateral medullary infarction ( opalski 's syndrome ) and concomitant cerebellar infarction .
accidental ingestion of foreign bodies like coins , fish bones , plastic toy parts , batteries and needles are common in toddlers and pre - school children . the battery cells are potentially hazardous as they cause chemical mucositis and because of their capability to generate electric current . the mucosal damage starts early and may lead to life - threatening complications in long - standing cases . a male child aged 1 year and 9 months was brought to our center by the caregivers with history suggestive of accidentally swallowing a computer battery cell at home 4 h before . the parents provided the history of living in a one - roomed dwelling of a slum , which also served both as a computer hardware assembling and repairing workshop of the father and , usually , was cluttered with computer accessories . history suggested that the child had ingested a battery cell while playing in the vicinity of the dismantled spares of computer sets and started retching , vomiting , coughing and choking and turned pale soon after swallowing something . the father had brought along with him a sample of the battery cell to support the suspicion , which resembled a five rupees coin with smooth edges . a high - risk informed consent was taken after counseling the caregivers for the procedure , explaining in detail the risks with the advantages and disadvantages involved in anesthesia and the course of actions of further interventions . in the preliminary history and clinical examination , the child had dehydration and mild stridor , but showed no batter marks on the body to exclude a case of child abuse . x - ray pa view of the chest and neck was performed immediately in the emergency room , which confirmed the impaction of the foreign body [ figure 1 ] . x - ray ( pa view ) showing foreign body the toddler was rushed to the observation room of the emergency services at our center . a fluid line was promptly started with ringer lactate and a bolus dose of hydrocortisone and ceftriaxone was administered with adequate precautions , while the preparation was pursued to shift the child to the emergency operation theater . a pediatrician was called without delay to assess the clinical status , including hemodynamic stability of the child . the pre - operative logistics was in favor of general anesthesia on the anticipation of use of rigid endoscope if flexible endoscopy fails and a tracheostomy in case of respiratory distress . the child was intubated orally by the anesthesiologist and the pediatric flexible esophagoscope was negotiated . the battery cell was noticed to be impacted in the cricopharynx , which is the most common site of impaction for foreign bodies . because of limited edematous and congested space , instrumentation was difficult and the single battery slipped repeatedly due to smooth surface , but was removed safely without trauma to the surrounding structures . an infant feeding tube was inserted immediately under direct supervision . on inspection , the foreign body was found to be discolored , with corrosion of one surface , as we could remove it before it could cause severe reaction to the tissues [ figures 2 and 3 ] . extracted foreign body opposite side of the corroded foreign body the child was kept in the pediatric intensive care unit to observe for immediate complications like dysphagia , odynophagia , mucositis causing stridor and aspiration in cases of delayed intervention . antibiotics , steroids and anti - inflammatory drugs were given as a 5-day course and a nasogastric tube was left in situ for a week , after which the patient could swallow well before discharge . the child was followed - up for the last 1 year and was free of long - term complications . foreign bodies in the esophagus are a routine emergency dealt by the otolaryngologist in daily practice . more the delay in extraction of the leaking battery cells , greater is the damage due to liquefaction necrosis of mucosa and the chance of stricture and pressure necrosis . there were no major complications after endoscopic removal . in the turkish retrospective study on the ingested foreign bodies in children having endoscopic removal , general hospital poison control center , of 25 patients with button battery ingestion , it was noted that impacted cell needed immediate esophagoscopic removal . at the department of radiology , klinikum rudolf virchow , charlottenburg , frg , button - type batteries ingested were removed from the esophagus and the upper gastrointestinal tract of 13 children by means of the fe - ex ogtm - technique . in all cases , the button cells were easily detected and retracted under fluoroscopic control with the magnet without post - operative setback . litovitz in his review of 56 cases advocated immediate removal of cells to prevent further complications and use of nasogastric tube to prevent strictures . still , the researchers are of the opinion that the management of ingested foreign bodies in children is not standardized . in the present case , the accidental battery cell impaction was referred to a teaching hospital emergency within 4 h. the kid was examined and diagnosis was confirmed promptly . the narrow inlet of cricopharynx was the site of impaction for this type of larger foreign bodies . primary outcome measure was immediate endoscopic removal with non - invasive technique as the utmost priority to prevent known secondary complications by the prompt extraction . the strength of the study is that the case of foreign body impaction had undergone optimum management within the resource constraint set up of eastern indian tertiary care hospitals . further , the case has been reported with precise details , including the follow - up . to the horizon of our knowledge , no previous study had been reported of children with computer battery cell in the cricopharynx of a toddler in eastern india . foreign bodies are dealt with by different authors differently , but the basic method is prompt endoscopy and atraumatic removal . further , controversies move around the logistics of general anesthesia , use flexible or rigid endoscope and a tracheostomy in case of respiratory distress . future research directions should move around research collaboration in this part of the country for the underlying mechanisms of foreign body ingestions by socioclinical research . the caregivers need to be educated on the art of rearing of children by continuing parent education to prevent all the domestic and peridomestic accidental occurrences . they should be taught on the early identification of ingested foreign bodies , and the optimum intervention is by prompt endoscopic removal of foreign bodies under general anesthesia as the safe and effective method in children to prevent erosion and perforation of the gastrointestinal tract . training of the primary health care staff with clinical audit are needed for handling such emergencies without referring them to other centers , which can save morbidities with long - term complications .
a computer lithium battery cell was impacted in the cricopharynx of a 1 year and 9 month - old child . the battery cell was safely removed with the pediatric flexible oesophagoscope under anesthesia without trauma to the surrounding structures . the lithium battery cell is potentially dangerous due to its ability to cause chemical damage to the mucosa and cause early inflammation and edema leading to dysphagia and respiratory obstruction . hence , it should be promptly extracted without delay to prevent catastrophe . the toddler recovered uneventfully in the immediate post - operative period and was followed - up for the last 1 year without any complication . to the horizon of our knowledge , no previous case was reported with computer battery cell in the cricopharynx of a toddler in eastern india . in conclusion , parent education is important in the early detection of foreign bodies and their interventions .
although many case reports have described the presence of a variety of abdominal organs found in spigelian hernias , there are no reports of an incarcerated appendix repaired laparoscopically . the use of laparoscopic technique in this case provided easy identification of the incarcerated structure and allowed us to perform an appendectomy without a large incision and with minimal dissection of the abdominal wall . a 71-year - old female presented to our clinic with a 10-year history of right lower quadrant pain . multiple imaging studies over this time period , including ct and mri , failed to reveal the etiology of her symptoms . in the week prior to presentation , the patient noticed a new bulge at the site of her chronic pain . the patient was taken urgently to the operating room for a laparoscopic spigelian hernia repair . upon laparoscopy , she was found to have an incarcerated appendix in the hernia ( figure 1 ) . after the appendix was carefully reduced , it was noted that the tip of the appendix was necrotic and an appendectomy was performed . the fascial defect was quite small and could be closed in a tension - free manner . due to the inflammation present and the appendectomy performed , the hernia was therefore repaired primarily with nonabsorbable monofilament sutures and buttressed with a biological mesh underlay . postoperatively the patient made good progress and was discharged on postoperative day 5 after return of bowel function . the patient was seen in outpatient clinic twelve days after discharge from the hospital and remains pain - free six months later . the incidence of spigelian hernias is low , but they are the most common type of spontaneous lateral ventral hernia . they are defined by protrusion of the peritoneal sac , an organ , or preperitoneal fat through the spigelian aponeurosis which is located between the semilunar line laterally and the lateral aspect of the rectus muscle medially . ( 1 ) the most common presenting symptoms are pain and the presence of a lump . however , these are notoriously difficult to diagnose on physical exam given that they are often obscured by abdominal fat and the external oblique aponeurosis . ( 2 ) spigelian hernias can be repaired in a traditional open fashion or laparoscopically , but the basic tenets of hernia repair must be applied in both situations . laparoscopic repair of spigelian hernias has been shown to be a viable option in the setting of elective repair , although one study recommended extraperitoneal repair in cases in which there is no known incarceration . ( 3,4 ) laparoscopy has also been reported as a method of diagnosis of spigelian hernias in cases where preoperative workup did not provide a definitive diagnosis . additionally , the use of laparoscopic repair with mesh has been reported for repair of an incarcerated small bowel . ( 5 ) multiple intra - abdominal organs have reportedly been found in spigelian hernias , but the presence of an appendix is very rare . ( 6,7,8 ) all prior reports of appendix - containing spigelian hernias have repaired the defect using open technique . in this case , the use of intraperitoneal laparoscopy allowed easy identification and subsequent management of the incarcerated appendix with minimal dissection of the abdominal wall structures .
spigelian hernias are rare , making up only 1 - 2% of all hernias . like other hernias , they may contain abdominal contents but are more likely to be incarcerated due to the small size of the fascial defect.(1 ) we describe here the case of a 71-year - old female with a 10-year history of right lower quadrant pain that remained undiagnosed despite multiple imaging studies . prior to presentation the patient developed a new bulge and increasing pain at this site ; an ultrasound revealed the presence of a bowel - containing hernia . the patient was taken urgently to the operating room for a laparoscopic spigelian hernia repair , and was found to have an incarcerated appendix in the hernia . after the hernia was reduced , an appendectomy was performed and the hernia was repaired with biological mesh . postoperatively , the patient did well , and her pain resolved .
white cottony mycelia formed on the stems of the diseased plants , which became tough and formed abundant amounts of brown spherical sclerotia on the surface of the mycelial mat . initially , stem rot disease affected only a small number of garlic plants ; however , the disease gradually spread and caused large - scale damage . the symptoms of the disease began in may , when the canopy of the garlic plant became densely covered , blocking air flow and light . in late may , relatively high air temperatures and frequent rain fall favored disease development . according to farmers who had experience with this disease , the disease becomes troublesome only when environmental conditions favor disease development . stem rot disease of garlic is a soil - borne disease , the inoculum potential of which increases in continuously mono - cropped areas . it is presumed that the debris of infected plants is main inoculum source of the disease . abundant sclerotia are formed on the stems and bulbs of infected plants , and the sclerotia are overwintered and invade the root , crown , and stem when garlic is planted ( fig . the causal organism of garlic stem rot was isolated from the sclerotia formed on the stems of the infected plants . the surfaces of the collected sclerotia were disinfected with a 1% naocl solution for 1 min , rinsed with sterilized water 3 times , and blotted with 5 layers of flame - sterilized filter papers ( 90 mm ) . the surface - sterilized sclerotia were placed on potato dextrose agar ( pda ) medium and incubated at 25 for 4 days . the fresh mycelia grew out from sclerotia on agar surface were cut with spatula and transferred to new pda and incubated at 25. the causal organism was cultivated in pda for 3 wk , and the morphology of the hyphae and clamp connection structure and sclerotia formation were examined under light microscopy ( table 1 ) . the mycelia were white and grew fast and prosperously on the surface of the pda and had a cottony appearance . 1d ) , were tan to brown in color , and were generally spherical , although some were irregularly shaped . after a 4-day incubation period on pda at 25 , a typical clamp connection structure was observed in the hyphae ( fig . the diameter of the hyphae ranged from approximately 4 to 8 m , and the optimal temperature for growth of the fungus was 30. the causal organism of stem rot disease in garlic and the associated symptoms agreed with the findings of kishi , and the mycological characteristics of the fungus agreed with those reported by mordue . the pathogenicity of the fungus was examined in a garlic plant grown in 1/5000a wagner 's pots . three bulbs of health garlic were planted in wagner 's pots ( 10 replication ) in october 2008 . the inoculum of the fungus was prepared by mixing fungal mycelia and soil 5 kg of sandy loam soil was sieved and autoclaved at 121 for 30 min . and repeatedly autoclaved 3 times at 5-day intervals . the mycelial mat of the test fungus grown on pda for 7 days was harvested . the harvested mycelial mat from 30 petri dishes ( 9 cm in diameter ) was mixed thoroughly with 5 kg of sterilized soil in a plastic container ( 56 35 13 cm ) . the mixture containing the mycelial mat and the soil was dried in the shade for 15 days and then powdered . the powdered mixture was preserved in a green house and used as inoculums ; 200 g of soil inoculum was placed on top of the wagner 's pots . after infestation of inoculum , 500 ml of tap water was added to each pot and covered with one layer of newspaper to maintain sufficient soil moisture . the inoculated pots were kept separately in a green house and were observed for disease symptoms . seven days after inoculation , white mycelia on the stems of the garlic and premodia of sclerotia were observed . as time progressed , the premodia of sclerotia developed into typical brownish sclerotia , and the white mycelia on the stem and soil surface had typical stem rot symptoms ( fig . , many diseases caused by sclerotium rolfsii in various crops have been reported [ 3 - 6 ] . the disease described in this article and the mycological characteristics of the causal organism were in line with these reports . on the basis of the results obtained in this study , the author suggested that the disease observed was stem rot of garlic caused by s. rolfsii saccardo . the isolate obtained from garlic in this study was deposited in the korean agricultural culture collection and was assigned the registration number kacc no .
stem rot disease was found in garlic ( allium sativum l. ) cultivated from 2008 to 2010 in the vegetable gardens of some farmers in geumsan - myon , jinju city , gyeongnam province in korea . the initial symptoms of the disease were typical water - soaked spots , which progressed to rotting , wilting , blighting , and eventually death . white mycelial mats had spread over the lesions near the soil line , and sclerotia had formed over the mycelial mats on the stem . the sclerotia were globoid in shape , 1~3 mm in size , and tan to brown in color . the optimum temperature for growth and sclerotia formation on potato dextrose agar ( pda ) medium was 30. the diameter of the hyphae ranged from approximately 4 to 8 m . typical clamp connection structures were observed in the hyphae of the fungus , which was grown on pda medium for 4 days . on the basis of the mycological characteristics and pathogenicity of the fungus on the host plants , the causal agent was identified as sclerotium rolfsii saccardo . this is the first report of stem rot disease in garlic caused by s. rolfsii in korea .
a 10-year cohort study found a 1.33-fold relative risk of death in a schizophrenic population compared with a control population ; the leading cause of death was cardiovascular disease . in patients with myocardial infarction and cardiac failure , reduced heart rate variability is one of the predictive factors of increased risk of cardiac death ; this reduced heart rate variability might be due to the anticholinergic effects of psychotropic drugs . thioridazine , an old and widely prescribed neuroleptic drug which was recently withdrawn , was associated with 75 % of 49 deaths in a patient group taking a single antipsychotic drug regimen ; its potential for qt prolongation had already been reported in 1963 . unexplained sudden death in young adults has been linked to the prescription of antipsychotics other than thioridazine . electrocardiographic modifications due to psychotropic drugs include prolongation of the pq interval ( atrioventricular blocks of different degrees of severity ) , widening of the qrs interval ( bundle branch block ) , st - segment changes ( repolarization disturbances ) , and prolongation of the qt interval . drug - induced long qt syndrome is an underestimated adverse drug effect : morbidity and mortality associated with a prolongation of the qt interval currently constitute the most frequent cause of drug withdrawal from the market or in 1920 , bazett found that the repolarization phase was related to ventricular systole , and that its duration was mainly influenced by the heart rate . bazett 's formula corrects the qt interval with an approximation for a rate of 60/min as follows : qtc = qt/rr , expressed in seconds ( figure 1 ) . prolongation of the qt interval is considered to be a surrogate marker for the risk of developing a particular type of ventricular tachyarrhythmia called torsades de pointes ( tdp ) , which may be recognized on the electrocardiogram ( ecg ) as a twisting of the qrs axis ( figure 2 ) . prolongation of the qt interval was reported in 8 % of 495 psychiatric inpatients . in an unpublished study in 1 000 inpatients under 65 years of age , serious cardiac events and sudden death occured more often at high doses of haloperidol , droperidol , sertindol , and methadone ; hence , drug - induced qt interval ( repolarization phase ) prolongation is mainly considered as a dose - dependent adverse reaction . psychotropic drugs block several potassium currents ( eg , iks and ikr ) during repolarization ( phases 2 and 3 during the action potential ) , resulting in a prolonged qt interval on the ecg with an increased risk of developing tdp similarly , eight phenotypes of the congenital long qt syndrome are recognized . the most frequent phenotypes are for potassium channels kcnq1 ( or kvlqt1 ) coding long qt type 1 ( lqt1 ) and kcnh2 coding lqt2 ; for sodium channels , scn5a is responsible for the lqt3 phenotype . drugs such as methadone , amitriptyline , haloperidol , and sertindole promote qt prolongation by blocking the herg potassium channels . as for class ic antiarrhythmic drugs , such as flecainide and propefanone , haloperidol also blocks sodium channels , and displays a quinidine - like effect by slowing sodium influx into myocytes . all drugs enhancing the qt interval prolongation should not be prescribed in patients with congenital long qt . furthermore , several psychotropic drugs block in vitro calcium channels of the l - type and may cause bradycardia and heart block through negative inotropic effect . in contrast to low - voltage calcium ion channels ( t - type ) located in pacemaker cells , highvoltage channels of the l - type modulate conduction through the sinoatrial pathway and the atrioventricular node . this mechanism may explain the unusual occurrence of second - degree sinoauricular ( mobitz type ii ) or atrioventricular block during clozapine prescription ( figure 3 ) . moreover , atrial fibrillation is also reported as an unusual adverse reaction during clozapine treatment . inherited defects of ion channels responsible for congenital long qt syndrome ( which are not always apparent on the ecg ) , polymedication , methadone maintenance , hypokalemia , hypomagnesemia , and history of cardiovascular disease are risk factors that increase the clinical consequences of the ion - channel effects of psychotropic drugs . however , age as a single factor does not seem to contribute substantially to the risk of cardiac adverse drug reactions . besides the qt interval prolongation and other major ecg modifications such as atrioventricular block and intraventricular conduction delay of different degrees of severity , other serious cardiovascular adverse reactions which are not dose - dependent are associated with psychotropic drugs . several deaths , from myocarditis and cardiomyopathy during clozapine therapy were reported in physically healthy young adults . the who database shows that clozapine is significantly more frequently reported in relation to cardiomyopathy and myocarditis ( figure 4 ) than other drugs . myocarditis and cardiomyopathy were also associated with chlorpromazine , lithium , fluphenazine , risperidone , and haloperidol , but these associations need to be further investigated in order to establish whether they are causal . the above information indicates that ecg monitoring should be performed during hospitalization and ambulatory treatment , at least when multiple psychotropic drug regimens , methadone maintenance treatment , and other predisposing factors for qt prolongation are present at admission . we particularly recommend regular cardiac and ecg monitoring in patients receiving clozapine , high - dosage antipsychotics , tricyclic antidepressants , drug regimens with potential interactions , or in clinical situations recognized as promoters of qt prolongation . further electrocardiographic studies in psychiatric patients , systematic recording of case reports , and data mining in pharmaco vigilance systems will help establish the magnitude of cardiac adverse reactions to psychotropic drugs .
rates of cardiovascular morbidity and mortality in psychiatric patients are higher than in the general population : it is estimated that those who suffer from schizophrenia have a life expectancy approximately 20 % shorter than those who do not , and this difference is not fully accounted for by suicide or accidental death.1 cardiovascular adverse effects of psychotropic drugs are common , and potentially harmful.2 the most serious cardiovascular consequences of psychotropic drugs are arrhythmias and sudden death , which principally result from torsades de pointes following progressive qt intervall prolongation . less severe cardiac adverse drug reactions are extremely common . orthostatic hypotension , vasodilatation with transient collapse , and reflex sinus tachycardia due to 1-adrenoceptor blockade and to anticholinergic effects occur at therapeutic dosages of several psychotropic drugs . postural hypotension was found in 77 % of patients receiving antipsychotic medication versus 15 % receiving placebo , and a correlation was found with drugs dosage.3 furthermore , antipsychotic drug is associated with an increased risk of hip fracture with a relative risk of 2 ( confidence interval [ ci ] , 1.6 to 2.6 and accounts for a third of all falls in nursing homes.4,5
an 18-year - old caucasian male was admitted for headache and abdominal pain , without diarrhea , of 3 weeks duration . the diagnosis of burkitt s lymphoma , stage d , noncleaved diffuse type was established ( figure 1a ) . chemotherapy was initiated with cyclophosphamide , oncovin , methotrexate , and prednisone ( comp ) . computed tomography ( ct ) scans with and without contrast and magnetic resonance imaging ( mri ) of the head was obtained secondary to the presence of headache in order to evaluate for central nervous system involvement . cerebrospinal fluid ( csf ) analysis revealed normal protein and glucose levels with 220 wbc s per high - powered field , which exhibited malignant morphologic characteristics . the csf tested negative for cryptococcal antigen and venereal disease research laboratory ( vdrl ) test , with no growth on bacterial and fungal cultures . subsequent bone marrow biopsy and hiv testing were also negative . based on elevated opening pressure and the presence of hydrocephalus on neuro - imaging , the patient underwent ventricular - peritoneal shunting . despite improvement in the hydrocephalus , ten days after the shunting procedure the patient developed diplopia for which the ophthalmology service was consulted . on examination patient had hypertropia of the right eye ( figure 1b ) , maximal on left gaze with beilschowsky test positive confirming right superior oblique palsy . visual fields , color vision and optokinetic nystagmus were normal . pupils were 3 mm , equal , and reactive to light ( figure 2a ) . his general condition deteriorated and fourteen days later he developed a right 3rd cranial nerve palsy and expired two days later . the patient s symptom of diplopia can be attributed to the palsy of the right superior oblique muscle . the long intracranial course of the trochlear nerve renders it vulnerable , which in this case was most likely affected by the elevated intracranial pressure . incidently , the patient had no pupillary constriction on near gaze with preserved accommodation and convergence ; the light reflex , however , was also maintained . these reflexes are mediated by the oculomotor nerve and associated parasympathetic fibers originating from the edinger - westphal nucleus ( bron et al 1997 ) , however , the basis of this finding is difficult to explain . it has been suggested that neurons in the primate pretectal olivary nucleus are solely related to the pupillary light reflex and that the cortical projections to this pretectal nucleus are related to this reflex and do not play a role in the pupillary near response ( zhang et al 1996 ) . the differential diagnosis of this finding is difficult , as iarp itself is a rare clinical entity . csf testing confirmed the absence of neurosyphilis and meningitis , leaving the presence of malignant - appearing white blood cells ( wbcs ) as the primary abnormality in addition to the elevated opening pressure . thus , we propose that infiltration from the burkitt s lymphoma and/or increased intracranial pressure due to hydrocephalus may have caused compression over the anatomical pathway serving the efferents of the near pupillary reflex . the elevated intracranial pressure was most likely secondary to impaired csf absorption at the arachnoid villi , which is most likely related to the presence of malignant wbcs in the csf . an autopsy on this patient could have assisted in clarifying the pathology behind this manifestation .
we present a case of an 18 year old white male with burkitt s lymphoma who was operated on for hydrocephalus and subsequently referred for evaluation of new onset diplopia . on examination , his visual acuity ( va ) was 20/20 in both eyes with a right superior oblique palsy . his pupillary reaction to light was intact while on near gaze there was no constriction of the pupils , bilaterally . the other two responses of the near gaze triad ie , convergence and accommodation were present . these findings were suggestive of an inverse argyll robertson pupil ( iarp ) , a rare entity in the literature . we could not find a specific cause attributable to this manifestation in this patient , though we feel it may be secondary to infiltration from burkitt s lymphoma and/or compression from elevated intracranial pressure of the efferent pupillary near reflex pathway .
for successful endodontic treatment , thorough knowledge of root canal morphology along with its variation is mandatory . the main objective of endodontic therapy is to prevent and when needed to cure endodontic disease and apical periodontitis . to achieve these goals , locating , cleaning and shaping ingle lists the most frequent cause of endodontic failure as apical percolation and subsequent diffusion stasis into the canal.1 variations in form of aberrant canal configurations , accessory canals , bifurcation , isthmuses , and anastomoses are often difficult to identify , thus creating a problem for endodontic treatment . inadequate knowledge regarding variations of root canal system may be a major cause of the failure of root canal system . endodontic treatment of single rooted teeth is usually simple as these teeth usually have single root canal . many studies have examined the root canal systems of these single - rooted teeth , confirming that it is not as simple as it may appear to be on standard periapical radiographs.2 however variations in form of presence of extra canal in mandibular incisors have been documented by various researchers in the past . vertucci in 1974 classified the canal configuration of mandibular incisors into four types:3 type i : single canal is present from the pulp chamber to the apex.type ii : two separate canal leaves the pulp chamber , but join short of the apex to form one canal.type iii : one canal leaves the pulp chamber , but it divides into two within the body of the root , the canals merge again to exist as one canal.type iv : two separate and distinct canals are present from the pulp chamber to apex . type ii : two separate canal leaves the pulp chamber , but join short of the apex to form one canal . type iii : one canal leaves the pulp chamber , but it divides into two within the body of the root , the canals merge again to exist as one canal . type iv : two separate and distinct canals are present from the pulp chamber to apex . various investigators have studied root canal system in mandibular incisors and reported following findings:4 although some of the morphological variations may depend on different ethnic backgrounds , two canals should be expected in about one - quarter for mandibular incisors . this proportion is not found clinically by practitioners during root canal treatment due to the failure of the dentist to recognize the presence of the second canal.5,6 the case report presented here has a striking feature of presence of extra canal in all the mandibular incisors in the same patient . a 45-year - old male patient reported to the department of conservative dentistry of chatrapati shahu maharaj shikshan sanstha dental college , aurangabad , from maharashtra state in india with the chief complaint of pain with lower anterior region . history revealed that the patient had a dull aching , intermittent pain mainly at night hours since 1 year . on clinical examination edge to edge the incisal bite with severe attrition was seen associated with mandibular anteriors . the intraoral periapical ( iopa ) showed a loss of lamina dura with 31 and 41 and periodontal space widening with 32 and 42 suggesting chronic apical periodontitis ( figure 2 ) . non - surgical endodontic treatment was planned with exploration , cleaning , shaping and filling of the root canal of all the mandibular incisors . root canal access opening was prepared through incisal edges initially with round diamond bur and later with round - end cutting tapered diamond in an oval shape with larger labio - lingual extension in an isolated condition with application of rubber dam . all the incisors when endodontically explored were found to have two separate canal orifices extending into two canals and joining short of the apex to continue as one ( vertucci s type ii canal morphology ) , thus showing one apical opening . the working length was determined for all the canals with apex locator as well as radiographically . complete chemo - mechanical preparation of all the teeth was done by hand instrumentation and use of 3% sodium hypochlorite irrigation . all the canals were then filled with intracanal calcium hydroxide for 1 week . on the next appointment , the intracanally placed calcium hydroxide was removed and obturation done with gutta - percha by lateral condensation technique . post - treatment radiographs were taken with conventional radiograph as well as orthopantomogram , cone beam computed tomography ( cbct ) technique with the consent of the patient ( figures 3 - 6 ) . dr . hermann prinze wrote object of the clinical dentistry is to institute preventive measures to relieve suffering , and to cure disease . to gain this goal clinician should have sound knowledge of dental anatomy , differential diagnostic modulates.7 during interpretation of diagnostic radiographs if there is sudden change in canal radiodensity , narrowing of canal space , abrupt disappearance of canal space , this gives us a clue for need of one extra angulated radiograph to diagnose an extra root or canal.8 uma et al . studied 50 extracted mandibular incisor for canal and isthmus morphology radiographically and concluded that type i and type iii canal configuration are much commoner than type ii canal which are rarely found.9 sinzianna scarlatescu et al . studied 32 extracted mandibular incisors in a south eastern romanian population by using color detector and a tooth - clearing technique . they concluded that type i root canal configuration ( 65.5% ) , type iii was found in 25% cases , type ii in 6.3% and type vii in 3.1%.10 mandibular incisor root canal system has either ovoid or ribbon shaped with a single canal in the range of 71.8 - 73.6% and double canal in the range of 26 - 28.1% . hence to achieve success in endodontic therapy it s important to locate , shape and obturate these extra canals . a common reason for not locating a second canal in mandibular incisors is an inadequate access opening into the tooth that leaves a lingual shelf of dentine over the second ( usually the lingual ) canal.11 therefore , it may be necessary to modify the conventional access preparation to permit better visualization and instrumentation of additional canal even at the emphasis of compromising the crown structure.12 this case report highlights the importance of thorough knowledge of root canals and its variations . extra canals in mandibular incisors are not a rare entity as suggested by few investigators . detailed knowledge and at times the modification of access opening is needed . the practitioners should be aware of how many canals to expect , their location , length and relationship to each other . wilson and henry have suggested that the access opening must be widened labio - lingually as well as inciso - gingivally to locate the extra canal , if any.13 it s also emphasized that radiographs taken from different angles are a must to anticipate the presence of extra canal .
single rooted tooth are considered to be the easiest for root canal treatment . the literature has documented cases with single rooted tooth showing more than single canal . understanding of root canal morphology thus is an important aspect to be considered for successful endodontic treatment . the case presented here shows a rare entity of having double canals in all the mandibular incisors in a single patient .
external ventricular device ( evd ) is useful in monitoring intracranial pressure ( icp ) in patients with severe traumatic brain injury and abnormal computed tomography ( ct ) scan of the head . however , the presence of evd is a well - known risk factor for infection of central nervous system ( cns ) with the incidence of 118% . acinetobacter baumannii has emerged as an important nosocomial infectious agent due to its ability to tolerate desiccation and to accumulate diverse mechanisms of resistance . there are few reports of successful use of tigecycline for treatment of multidrug resistant ( mdr ) a. baumannii meningitis . colistin is potentially nephrotoxic , which increases with higher cumulative dose and in patients with preexisting renal impairment . here , we report a successful microbiological cure of evd - associated mdr acinetobacter ventriculitis using intravenous and intraventricular colistin together with intravenous tigecycline . a 75-year - old patient presented with the history of decreased level of consciousness and weakness in left half of the body following trivial fall . glasgow coma scale ( gcs ) was 7 ( e1v1m5 ) with bilateral equal and reacting pupils . after 3 days , patient had progressive drop in gcs to 4 ( e1m3vt ) . evd was in proper position and functioning . on the day 4 , he developed high - grade fever ( up to 103f ) . analysis of cerebrospinal fluid ( csf ) sample revealed total count of 630 cells / mm with 90% polymorphs with sugar of 20 mg / dl and protein of 180 mg / dl . csf sample was sent for culture and sensitivity which revealed a. baumannii sensitive only to colistin and tigecycline and resistant to all other antibiotics including carbapenems . intravenous colistin was started at the dose of 2 million iu 8 hourly together with 200,000 iu through intraventricular route daily . tigecycline was administered intravenously at a loading dose of 100 mg followed by 50 mg twice daily . csf culture after 3 days of starting colistin and tigecycline was negative with decrease in cell count to 25 cells / mm . worsening of renal function , intravenous dose of colistin was decreased to 2 million iu 24 hourly on the day 5 when creatinine reached 4 mg / dl . intravenous colistin and tigecycline was continued for 14 days when the family members of patient decided to withdraw the support due to anticipated poor neurological outcome despite microbiological cure of ventriculitis . possess an impressive armamentarium of resistance mechanisms rendering it resistant to almost all commercially available antibiotics . colistin is widely used for the treatment of infections by mdr gram - negative rods . however , intravenous administration of colistin is associated with a very low cns transfer of only around 5% . a concomitant intraventricular administration is required for treatment of severe ventriculitis in patients with evd . the literature review suggests a minimum intrathecal dose of 125,000 iu daily as suggested by the guidelines of infectious disease society of america and may possibly increase to 250,000 iu daily . duration of intraventricular treatment < 7 days is possibly associated with higher mortality . in our case , it was administered for 8 days . combination of intraventricular and intravenous therapy was chosen as intraventricular therapy alone is not sufficient to treat evd - related ventriculitis due to mdr gram - negative pathogen . kidney injury is related to total cumulative dose and duration of therapy . as in our patient , older and more severely ill patients are at higher risk for nephrotoxicity , and it independently predicts higher mortality . tigecycline has demonstrated good in vitro activity against mdr a. baumannii , but current evidence does not support its use as monotherapy . hence , tigecycline was used as a component of multidrug and multi - route therapy for our patient . despite successful microbiological clearance , the bundle of measures such as education of intensive care unit personnel , meticulous intraventricular catheter handling , and csf sampling only when clinically necessary routine replacement of drainage catheter on the 7 day is associated with significantly decreased incidence of evd - associated infection . intraventricular and intravenous colistin combined with intravenous tigecycline may be effective in microbiological clearance of bacteria . bundle of measures should be implemented in all patients with evd and at risk of mdr infection to prevent the disastrous outcomes .
acinetobacter baumannii is an important cause of nosocomial ventriculitis associated with external ventricular device ( evd ) . it is frequently multidrug resistant ( mdr ) , carries a poor outcome , and is difficult to treat . we report a case of mdr acinetobacter ventriculitis treated with intravenous and intraventricular colistin together with intravenous tigecycline . the patient developed nephrotoxicity and poor neurological outcome despite microbiological cure . careful implementation of bundle of measures to minimize evd - associated ventriculitis is valuable .
in 1997 , nozaki et al . ( 1 ) described subdiaphragmatic intrasellar meningiomas that originated from the sellar turcica ; two of which only originated from the floor of the sellar turcica . since then , only one case of intrasellar meningioma from the sellar floor has been reported in the english literature ( 2 ) . the pituitary gland is reported to be covered by two distinct structures : the capsule and dura ( 3 ) . although meningiomas can originate from anywhere in the sella turcica , subglandular meningiomas are extremely rare ( 1 ) . we report a case of subglandular meningioma probably originating from the floor of the sella turcica along with a review of the pituitary fossa anatomy . the imaging revealed an intrasellar mass measuring approximately 1.8 1.7 cm with a slightly upward bulge , homogenous isointense signals on t1- and t2-weighted images , and a focal inhomogenous signal on t2-weighted images ( fig . on contrast - enhanced t1-weighted images , the lower portion of the sellar mass was greatly enhanced , and the upper portion , lesser enhanced ( fig . initially the upper portion was thought to be a pituitary adenoma and the lower portion , normal pituitary gland . when an endonasal transsphenoidal operation was performed to remove the tumor , a bulging tumor on the sellar floor the normal pituitary gland was elevated and shifted to the left anterolateral side by the mass . after the tumor was removed , the diaphragma sellae was identified at the top of the sellar turcica . on histological examination , the low - lying intrasellar lesion was confirmed as a meningioma , while above it was the normal pituitary gland ( fig . in 1969 , hardy and robert ( 4 ) described a separate type of intrasellar meningioma originating from the inferior aspect of the diaphragma sellae . in 1985 , al - mefty et al . ( 5 ) proposed that diaphragmatic meningiomas and a tuberculum sellae meningiomas were separate entities . in 1995 , kinjo et al . ( 6 ) classified diaphragm sellae tumors according to their site of origin from the diaphragm : type a originated from the upper leaf of the diaphragma sellae anterior to the pituitary stalk ; type b from the upper leaf of the diaphragma sellae posterior to the pituitary stalk ; and type c from the inferior leaf of the diaphragma sellae . in 1997 , nozaki et al . ( 1 ) summarized observations in the literature on 18 operatively confirmed pure subdiaphragmatic intrasellar meningiomas originating from the dura of the sella turcica . they included type c diaphragma sellae meningiomas according to kinjo 's classification and intrasellar meningiomas from the other side of the sella , such as the floor and anterior or lateral wall . they established the origin of 9 meningiomas ; 6 from the inferior leaf of the diaphragma sellae ( the same as type c diaphragma sellae meningiomas ) , 2 from the floor of the sella turcica , and only one from the anterior wall of the sella turcica . one further example from the floor of the sella has been reported since then ( 2 ) . to the best of our knowledge , only three cases of intrasellar meningiomas originating from the floor of sella turcica have been reported . it is important to differentiate a diaphragm sellae meningioma from a pituitary macroadenoma because they require different surgical approaches . ( 7 ) emphasized that , most of the intra- and suprasellar macroadenomas could be approached by the transsphenoidal route , while diaphragma sellae meningiomas might require a craniotomy . for diaphragma sellae meningiomas , the transcranial - transsphenoidal approach is preferred for a type c meningioma and the cranio - orbital approach for type a and type b meningiomas ( 6 ) . on the other hand , the transsphenoidal approach is advocated for all subdiaphragmatic meningiomas ( 8) , or should be tried first , irrespective of whether the lesion is a meningioma or a pituitary adenoma ; even if it has a small suprasellar extension ( 2 ) . according to cappabianca et al . ( 7 ) , it is essential for the diagnosis of type c meningiomas that the diaphragma sellae be displaced upwards with the normal pituitary gland visible below . our case also showed upward bulging of the complex of the diaphragma sellae and pituitary gland , with the meningioma located below the pituitary gland with a broad - based attachment to the sellar floor . the pituitary gland is reported to be covered by two distinct structures ; a capsule and the dura in the pituitary fossa ( 3 ) . at each inferolateral edge of the pituitary fossa , the thick dura of the inferior wall splits into two thinner layers that form a y shape . one of the arms of the y forming the lateral wall of the pituitary fossa is directed superiorly , while the other arm continues as the sphenoidal part of the medial wall of the cavernous sinus and extends to the lateral limit of the sinus ( 3 ) . it is difficult to differentiate intrasellar meningiomas from intrasellar tumors , which can include pituitary adenoma , pituicytomas , intrasellar germinomas , craniopharyngiomas , aneurysms , and metastases . calcifications are a feature of intrasellar meningiomas , craniopharyngiomas , and aneurysms , but they are not typical features of adenomas . necrotic or cystic changes can be found in most types of intrasellar tumors except for meningiomas . meanwhile , imaging findings on the angioarchitecture of aneurysms allow them to be differentiated from other tumorous lesions . the intrasellar mass in our case was treated by the transsphenoidal approach because our preoperative diagnosis was a pituitary adenoma . during the operation , the lower part of the intrasellar mass was found to be subglandular meningioma following a frozen biopsy , which was successfully removed , while leaving intact normal pituitary gland in the upper part of the intrasellar mass . in conclusion , they probably originate from the dura in the sellar floor , while most intrasellar meningiomas that are located in the subdiaphragmatic and supraglandular area originate from the diaphragma sellae . we report a case of intrasellar and subglandular meningioma along with a review of the literature .
most intrasellar meningiomas are located in the subdiaphragmatic and supraglandular region because they originate from the diaphragma sellae . subglandular meningiomas located under the pituitary gland are extremely rare . intrasellar meningiomas in the subdiaphragmatic and subglandular region probably originate from the dura in the sellar floor . we report a case of a subglandular meningioma along with a review of the literature .
isolated iliac artery aneurysms are extremely rare , representing only 0.03% of a series of 26 251 autopsy patients , mostly associated with atherosclerosis and with other aetiological factors including trauma and infection . presentation is variable including incidental findings during surgery , abdominal or rectal examination , acute rupture , ureteric obstruction , sciatic nerve compressive neuropathy or pelvic vein compression . gluteal artery aneurysms are also rare , more commonly affecting the superior gluteal artery in association with penetrating trauma , with those of the inferior gluteal artery usually associated with pelvic fractures [ 3 , 4 ] . although often asymptomatic , presentations may include progressive gluteal swelling , a mass mimicking a gluteal abscess , a tumour or be associated with sciatic nerve compression symptoms . a 67-year - old , non - communicative man was referred to plastic surgery with a right - sided subcutaneous gluteal haematoma and low haemoglobin after a minor fall 28 days previously . despite there being no obvious bleeding points after two surgical haematoma evacuation procedures , interspersed with days of haemodynamic stability , the patient required repeat blood transfusions . on saturday ( day 2 ) , a 500 ml haematoma was evacuated at our institution under general anaesthesia via a 4 cm incision . there were no identifiable bleeding points and he was transfused 2u of packed red cells peri - operatively , increasing his hb to 8.7 g / dl . the patient was haemodynamically stable , throughout sunday ( day 3 ) , with no clinical features of recurrence . on monday ( day 4 ) , the haematoma recurred and his hb dropped from 8.7 g / dl to 4.9 g / dl , prompting a second transfusion of 4 units of packed red cells , increasing his hb back to 8.7 g / dl . on tuesday ( day 5 ) , a further hb drop to 6.4 g / dl prompted transfusion of 2u of fresh frozen plasma 3u of red cells and surgical haematoma evacuation ; however , there was no identifiable active haemorrhage . gastroscopy and colonoscopy were performed due to a past medical history of haematemesis ; however , no recent / active bleeding was identified . the patient continued to be haemodynamically stable throughout thursday ( day 7 ) , abdomen and pelvis contrast ct indicated two bleeding sites arising from the internal iliac artery region . one was identified arising from the posterior division of the right internal iliac artery , at the inferior gluteal artery ( fig . less prominent bleeding point was noted at the division level of the internal iliac artery into its anterior and posterior branches , anterior to the sacroiliac joint ( fig . selective angiography was also performed , identifying a large pseudoaneurysm arising from the inferior gluteal artery and endovascular repair was undertaken ( fig . the right common iliac artery was accessed via a left common femoral artery puncture and a combination of five 2 5.0 mm figure 818 pushable coils and two vortx-18 pushable coils of 3 2.5 mm and 4 4.0 mm respectively ( boston scientific , massachusetts , usa ) were deployed to embolize the inflow and outflow vessels ( fig . a further 200 mls of blood were evacuated from the right buttock wound post - embolization . haemostasis was achieved successfully and wound closure undertaken on the following monday ( day 11 ) . ( b ) second site of haemorrhage at the level of division of the internal iliac artery into its anterior and posterior branches , anterior to the sacroiliac joint . figure 2:interventional angiogram : deployment of seven coils to embolize the inflow and outflow vessels of the inferior gluteal artery pseudoaneurysm . ( b ) second site of haemorrhage at the level of division of the internal iliac artery into its anterior and posterior branches , anterior to the sacroiliac joint . interventional angiogram : deployment of seven coils to embolize the inflow and outflow vessels of the inferior gluteal artery pseudoaneurysm . we present a diagnostically challenging case of recurrent subcutaneous gluteal haematoma , uniquely associated with 2 bleeding points relating to the internal iliac system . at surgery internal iliac and gluteal artery aneurysms are extremely rare , often traumatic in origin and predominantly classified as pseudoaneurysms [ 1 , 3 , 4 ] . they may be slow - growing and present late either as an incidental finding or with gluteal swelling and bruising , compressive neuropathy or compression of surrounding structures . radiological investigations including duplex , ct and mri may facilitate bleeding course identification , shape , size and position relative to surrounding structures . angiography however is diagnostically invaluable in delineating the anatomy of the arterial branches involved and in facilitating intervention [ 5 , 8 ] . aneurysms of the internal iliac artery and branches have a poor prognosis if left untreated . estimated mortality ranges between 50% and 75% , when in isolation , due to rupture and fatal haemorrhage [ 1 , 5 ] . traditional treatment includes open surgical artery ligation both proximal and distal to the aneurysm to minimize recurrence risk due to collateral circulation . due to the potential difficulty in identifying sites of haemorrhage , such as in our reported case , endovascular angiographic repair using embolization although this carries the incontestable advantage of being minimally invasive over surgery , embolization alone may be insufficient for pseudoaneurysm treatment as it is less likely to relieve exerted pressure on surrounding structures , while coils and stents may dislodge and migrate . this case uniquely reports recurrent gluteal haematoma in a non - communicative patient which manifested 28 days following minor , non - penetrating and non - fracture - associated trauma . furthermore , although the rare internal iliac artery system pseudoaneurysm has been reported , no similar report exists in the literature with two bleeding sources and with this mechanism of injury . despite repeated requirements for low hb associated blood transfusions , albeit interspersed with days of haemodynamic stability , exclusion of relevant history - related bleeding sources , and by endoscopy and two surgical explorations , it was only until contrast ct scanning was requested that two separate bleeding sources were identified and successfully treated by endovascular coil embolization . this report provides an important variant and lesson to supplement current literature and understanding of more diagnostically challenging cases of an extremely rare presentation ; it highlights the importance of maintaining a high index of suspicion and investigating the possibility of multiple bleeding points in cases of subcutaneous gluteal haematoma , even when the presentation is several weeks following minor non - penetrating and non - fracture - associated trauma . this research received no specific grant from any funding agency in the public , commercial or not - for - profit sectors .
isolated iliac artery aneurysms are extremely rare . gluteal artery aneurysms are also rare , more commonly affecting the superior gluteal artery in association with penetrating trauma , with those of the inferior gluteal artery usually associated with pelvic fractures . we discuss a diagnostically challenging presentation of recurrent subcutaneous gluteal haematoma due to two separate internal iliac artery - associated bleeding points . a 67-year - old man was referred , from a peripheral hospital , with a right - sided subcutaneous gluteal haematoma . this manifested 28 days following minor non - penetrating , non - fracture - associated trauma . despite repeat blood transfusions , albeit interspersed with days of haemodynamic stability , and despite exclusion of relevant bleeding sources at endoscopy and two surgical explorations , it was only until contrast ct scanning was requested that both bleeding sources were identified and successfully treated by endovascular coil embolization . this provides an important variant and lesson to supplement current literature and understanding of more diagnostically challenging cases of an extremely rare presentation .
sclerosing polycystic adenosis ( spa ) is a rare salivary gland lesion that was first described by smith et al . . most cases of spa have originated in the parotid and submandibular glands , with a few cases of minor salivary gland origin . we present a case of right nasal cavity spa in a 49-year - old man with nasal obstruction . this is the first case in the english literature of spa involving the nasal septum . although spa has a favorable outcome , the histologic findings of spa include atypia ranging from mild dysplasia to carcinoma in situ , which leads to difficulties in diagnosis and subsequent management . thus , spa could be included as a possible etiology of a mass in the nasal septum . a 49-year - old man presented with a 1-year history of right nasal obstruction and occasional epistaxis . the mass arose from the right nasal septum and bled easily on touch ( fig . 1 ) . a computed tomographic scan of the paranasal sinuses showed a polypoidal mass with attachment to the bony nasal septum without erosion into the surrounding structures ( fig . a margin of normal mucosa and the underlying perichondrium was widely excised . grossly , the tumor was a firm pedunculated mass , measuring 2.11.51.1 cm , with a peduncle 4 mm in length . the cut surface was lobular and glistening , containing a few tiny visible cysts < 1 mm in diameter . the microscopic examination revealed a subtle lobular proliferation of dilated ductal components with cystic changes surrounded by abundant dense hyalinized hypocellular collagen stroma with a mild inflammatory cell infiltration . immunohistochemical staining for -smooth muscle actin demonstrated myoepithelial cells surrounding ductal epithelium and the epithelial cells lining ducts showed focal immunoreactivity for estrogen receptor ( fig . 4 ) . together , these findings were characteristic histopathologic features of spa . the patient was discharged the next day , and the postoperative course was uneventful . postoperatively , no wound complications were observed and no recurrence was observed at the 12-month follow - up examination . smith et al . described nine cases of histologically - distinct lesions of the salivary gland and suggested the term spa for these rare lesions . they described the histologic features as a generally lobular proliferation of ductal and acinous elements , cystic ducts with frequent apocrine- and sebaceous - like cells , eosinophilic intracytoplasmic granules within some acinar - type cells , intraductal epithelial hyperplasia with occasional collagenous spherulosis , and dense fibrosis that is frequently nodular . thirty - four tumors arose in the parotid gland , three envloved the submandibular gland , and four arose in the oral cavity mucosa [ 2 - 5 ] . based upon previous reports , spa presents over a wide range of age ( 9 to 80 years ) , with a slight female preponderance . recurrence occurs in almost one - third of cases and there have been no reports on metastasis or mortality from these lesions . spa is a rare lesion of unknown etiology , morphologically resembling fibrocystic changes of the breast . . postulated that these lesions represent a pseudoneoplastic condition that results in both fibrosis and epithelial poliferation . however , a molecular study using the human androgen receptor assay for clonality analysis has demonstrated that this lesion is clonal and given the 30% clinical recurrence rate and hyperplastic , atypical features , spa is a neoplasm rather than a reactive process . histologically , spa is well - circumscribed , unencapsulated , and composed of lobules of proliferating ductules with occasional apocrine metaplasia , ductal hyperplasia , and focal cystic spaces within a dense collagenous hypocellular stroma . the glandular epithelial cells exhibit a spectrum including non - descript , apocrine , foamy vaculolated , and mucinous cells . the ductal epithelial atypia , ranging from mild dysplasia to carcinoma in situ , can be found in 40%-75% of cases . the differential diagnosis includes polycystic disease , sclerosing sialadenitis , and benign and malignant glandular neoplasias . the acinar or ductal proliferation , apocrine metaplasia , and dysplasia suggest the possibility of acinar cell carcinoma , oncocytic carcinoma , or adenocarcinoma . in spa , the lobular architecture is typically maintained , the atypical nests are rimmed by myoepithelial cells , and the invasive , destructive growth pattern of a carcinoma is lacking . however , if clinicians and pathologists are not familiar with spa , there is high chance of misdiagnosis . treatment for spa is surgical excision . recurrence occurs in almost one - third of cases and is likely due to incomplete surgical excision or multifocal disease , rather than true recurrent tumors . even through no invasive carcinoma or metastasis has been reported , if we consider that the precise nature of this uncommon lesion is unknown , it is advisable to follow patients for a long period . in conclusion , this is the first reported case of spa of nasal minor salivary gland origin . although atypia ranging from mild dysplasia to carcinoma in situ can be found in some cases , spa has a favorable outcome . because of the infrequency of occurrence and histologic similarity of this lesion to others , it is important for otolaryngologists to familiarize themselves with spa to reduce the likelihood of misdiagnosis .
sclerosing polycyctic adenosis ( spa ) is a rare lesion of unknown etiology morphologically resembling fibrocystic changes of the breast . to date , approximately 41 cases of spa have been reported . most cases of spa have originated in the parotid and submandibular glands , with a few cases of intra - oral minor salivary gland origin . this is the first reported case of sclerosing polycystic adenosis of nasal minor salivary gland origin . the differential diagnosis of spa includes polycystic disease , sclerosing sialadenitis , and benign and malignant glandular neoplasias . although atypia ranging from mild dysplasia to carcinoma in situ can occur in some cases , spa has a favorable outcome . it is important to be familiar with spa to avoid aggresive treatment that results from a misdiagnosis . we present a case of a 49-year - old man who had 1-year history of right nasal obstruction .
a 52-year - old man with significant history only of remote non - injection drug use presented with neck pain , 2 weeks of mild fevers , chills , night sweats , unintentional weight loss , dyspnea , and dry cough . on physical examination , he appeared non - toxic with poor dentition . magnetic resonance imaging done for the neck pain was concerning for cervical discitis , while a computed tomography ( ct ) chest scan done for his pulmonary symptoms was concerning for multiple septic pulmonary emboli . initial blood cultures grew gram - positive cocci in chains . because of concerns of infective endocarditis ( ie ) , ampicillin and ceftriaxone were started . a transthoracic echocardiogram ( tte ) showed a thickened pulmonic valve ( pv ) leaflet with a pulmonary artery systolic pressure of 58 mmhg . a subsequent transesophageal echocardiogram ( tee ) revealed a 2-cm , mobile , linear pv mass with moderate , eccentric pulmonic regurgitation ( fig . ct angiogram showed a pulmonary embolism distal to the right main pulmonary artery with distal propagation . meeting duke criteria for ie , he was discharged on a 6-week course of ceftriaxone via a tunneled catheter . overall , his prognosis was more promising due to early detection and no ensuing complications . right - sided infective endocarditis ( rsie ) is a rare phenomenon that predominantly involves the tricuspid valve , with or without involvement of the pv . however , isolated pv vegetation is an extremely rare event that typically occurs in 1.52% of patients hospitalized with ie ( 1 ) . from 1960 to 2005 , there were only 45 reported cases of pve in structurally normal hearts ( 2 ) . proposed mechanisms for lower incidence of rsie are attributed to the right heart valves having lower prevalence of congenital malformations , differences in the endothelium and vascularity , lower pressure gradients leading to lower jet velocities and reduced stress on the right - sided endocardium as well as reduced oxygen content in the venous blood ( 3 , 4 ) . identifying risk factors for rsie helps heighten clinical suspicion to make an earlier diagnosis , which improves outcomes . major risk factors are ivdu ( 76% ) , congenital cardiac defects , sepsis , alcohol abuse , diabetes , pacemaker , and central venous catheter implantation . in about 28% of the cases no specific risk factor is identified ( 2 , 46 ) . in our case , management and subsequent prognosis of this unique entity is determined by early and high diagnostic suspicion . this was particularly difficult in this case because of the dominating extra cardiac signs and symptoms . presentation of rsie often mimics primary pulmonary symptoms , such as fevers , dyspnea , and cough , with or without a normal chest x - ray ( 7 ) . in roughly 50% of reported cases , there was an associated pulmonic regurgitation murmur ( 8) , which was not appreciated in the above patient . a pv thrombus can not be ruled out ; however , our clinical suspicion was high for pv vegetation given our patient 's presenting symptoms in addition to the presence of multiple septic emboli . in detecting pv vegetations , tee has higher sensitivity and specificity in comparison to tte . the most common microbial culprits are staphylococcus aureus , coagulase - negative staphylococcus , and group b streptococcus , with septic pulmonary emboli being seen in approximately 75% of patients ( 911 ) . in non - complicated cases , initial management ( 12 ) should be with antibiotics only , as response is often seen within 6 weeks of therapy ( 13 ) . a retrospective study has shown that vegetations less than 12 cm in rsie commonly respond to medical treatment ( 14 ) . indications for surgery include persistent bacteremia and fevers despite appropriate parenteral antibiotic therapy , recurrent pulmonary emboli , locally invasive infections such as a perivalvular abscess , cardiovascular instability , or progressive valve destruction and incompetence leading to heart failure . prosthetic valve replacement or prosthetic annular implantation and non - prosthetic kay 's or devega 's annuloplasty , bicuspidization , valvectomy , debridement of the infected area , or excision of the vegetation ( 12 ) . overall , the prognosis for rsie including isolated pve is generally better than left - sided ie , with the latter carrying a higher mortality rate ( 15 ) . we document a rare case of isolated pve that had a non - specific presentation . evaluation of the patient 's risk factors and a careful history are key in earlier detection of rsie . this can help reduce hospital - related costs via avoiding complications and even the need for surgery . hence , high index of suspicion is required as early diagnosis can drastically improve outcomes while avoiding complications and preventing the need for surgery . in our case , prognosis was favorable due to early detection and no ensuing complications . the authors have not received any funding or benefits from industry or elsewhere to conduct this study . informed consent was obtained from the patient and his family for educational use of the below mentioned data and no personal patient information has been disclosed . none of the authors have any financial or personal bias that would inappropriately compromise the publication of this work .
we discuss a unique case of a 52-year - old man with no history of intravenous drug use or dental procedures who presented with neck pain , 2 weeks of fevers , chills , night sweats , cough , and dyspnea found to have isolated pulmonic valve ( pv ) endocarditis . the patient did not have an associated murmur , which is commonly seen in right - sided infectious endocarditis . a transthoracic echocardiogram showed a thickened pv leaflet , with subsequent transesophageal echocardiogram showing a pv mass . speciation of blood cultures revealed streptococcus oralis . in right - sided infective endocarditis , usually the tricuspid valve is involved ; however , in our case the tricuspid valve was free of any mass or vegetation . the patient did meet duke criteria and was thus started on long - term intravenous antibiotics for infectious endocarditis . the patient 's symptoms quickly improved with antibiotics . a careful history and evaluating the patient 's risk factors are key in earlier detection of infective endocarditis ( ie ) . because of early detection and a high index of suspicion , the patient had no further complications and did not require any surgery . in conclusion , clinical suspicion of right - sided ie should be high in patients who present with persistent fevers and pulmonary symptoms in order to reduce the risk of complications , and to improve outcomes .
a 19-year - old male presented with an additional opening on the ventral surface of the penile shaft along with a normally placed urethral meatus at the tip of the glans . there was no history of swelling or stone impaction at the site of the opening . the only surgical history was of a ceremonial circumcision at the age of 5 years that was done by a local priest . the glans was normal in size and shape with a normally placed external urinary meatus . there was a small opening near the corona on the ventral aspect of the penile shaft , just lateral to the midline . there was no visible chordee and the length of the penis was normal . at the time of micturition , the patient voided mainly through the normally placed meatus along with dribbling of urine from the ventral opening ( fig . 1 ) . the results of investigations , such as hemogram , biochemical profile , urine analysis , abdominal ultrasound , intravenous pyelography , and other radiological investigations to rule out coexistent anomalies , were normal . there was no anorectal malformation . , an infant feeding tube could be easily passed into the fistulous opening via the external urinary meatus . there was a lack of spongiosus tissue around the fistulous tract , whereas the distal urethra had normal spongiosus covering . the tract was excised , and double layered repair of the defect was done ( fig . the patient was able to pass urine normally after removal of the catheter and the fistula had completely healed . a review of the literature indicates that there are two variants of congenital anterior urethrocutaneous fistula . the more common type is associated with chordee , a dorsal hood , distal hypospadias with or without a distal urethral or spongiosal defect , and anorectal anomalies . the rarer variety , as in the case reported here , develops in an isolated fashion with no chordee or hypospadias and an intact distal urethra and spongiosum . only 24 cases of this variety have been reported in the english literature to date . all cases apart from one were described in the pediatric age group [ 1 - 8 ] . there was no mention of the effect of this deformity on the patient 's virility or his ability to procreate . our patient had normal erectile function and was a father , thus proving that this deformity has no effect on the reproductive function of the patient . the patient did not seek medical advice for his condition until late because of the minimal symptoms associated with his condition . considerable confusion about this entity exists , and there have been claims that it is in fact a result of trauma during circumcision . however , due to the lack of urothelium in the pathological examination of the circumscribed skin and also because of the reports of its existence in uncircumscribed patients , it is now being considered as a separate entity . the arrest of the growth of the proximal urethra and compensatory overgrowth of the glandular urethra has been proposed to be the mechanism for the formation of the isolated variety of fistula . a deficiency of spongiosum in the fistula , as occurred in our case , can also be explained by this , because only the proximal urethra is associated with the formation of corpus spongiosum [ 2,4 - 6 ] . before deciding on surgical correction , probing , dye studies , and cystourethroscopic examination may be helpful in substantiating the diagnosis . several methods of surgical repair have been described in the literature , including local skin flap , preputial skin flap , denis brown urethroplasty , thiersch - duplay urethroplasty , primary closure , buccal mucosal graft , and tubularized incised plate urethroplasty with dartos flap [ 1 - 8 ] . the method of repair must be individualized depending on the location and the size of the fistula . in the present case , the fistula was small , and thus primary closure was used for repair with success . in larger fistulas , the incidence of recurrence following repair is about 17% as per the reported literature ( 4 recurrences in 24 cases ) [ 1 - 8 ] . to conclude , isolated congenital anterior urethrocutaneous fistula is a condition that can be easily and effectively managed . increased awareness , good history , meticulous examination , and imaging studies can help in the correct diagnosis and treatment of this rare clinical entity .
isolated congenital urethrocutaneous fistula of the anterior urethra is an extremely rare anomaly in which , along with a normal urethra and meatus , a fistula is present . these cases usually present in the pediatric age group . to date , only one such case has been described in the adult population . we describe one such case in an adult and discuss the management of this uncommon anomaly .
a thermophilic fungus , a. flavus k-03 from poultry farming soil ( kim , 2003a ) was cultivated on a mineral salt medium ( 0.5 g nano3 , 1.0 g k2hpo4 , 0.5 g mg so47h2o , and 0.01 g feso47h2o per liter ) supplemented with 0.2% peptone and 1.0% glucose at 40 , ph 9.0 for 15 days . keratinase was purified from the culture filtrate by carboxylmethyl cellulose ion exchange and sephadex g-100 chromatography ( 2 85 cm ; pharmacia , sweden ) , and the purity and the protein concentration were determined by sds - page analysis as previously described ( bradford , 1976 ) . a suitable amount of enzyme had been diluted into 0.2 m calcium chloride solution under continuous stirring . hydrolyses of azo - keratin and azo - casein ( sigma ) were determined as previously described with some modifications ( lin et al . , 1992 ; kim , 2003b ) . the free amino group released by keratinolysis was estimated by the ninhydrin method ( rosen , 1957 ) . no enzyme activity was observed in the final preparation and stored at 4. after immobilization , the excess enzyme keratinase was collected for reuse . as shown at table 1 , approximately 100 g of purified keratinase was immobilized on beads . in free and soluble forms , immobilization reduced the keratinolytic and caseinolytic activity by 71~78% . in comparison with chemically immobilized keratinase ( lin et al . , 1996 ) , the specific activity of bio - immobilized keratinase was improved from 17 to 29% when casein was used as the substrate . soluble keratinase and immobilized keratinase ( 1.5 g and 20 g , respectively ) were added into 50 l of 50 mm buffers viz . citric acid / na2hpo4 for ph 4~6 , nahpo4/na2hpo4 for ph 6~8 , tris - hcl for ph 7~9 , glycine / naoh for ph 9~11 , and nahco3/naoh for ph 11~12 , and incubated at 4 for 15 min . after pretreatment , 0.8 ml of 0.5% azo - casein dissolved in 50 mm tris - hcl buffer ( ph 9.0 ) was added to measure the remaining activity of free and immobilized keratinase . comparing the stabilities of soluble and immobilized keratinase at 40 , 10 g of keratinase and 0.3 ml of immobilized keratinase were added separately into two tubes containing 10 ml of 50 mm tris - hcl buffer , ph 9.0 . as shown in fig . 1 , both free and immobilized keratinase showed sensitivity in acidic condition , but were less sensitive in alkaline ph . comparing with soluble keratinase , the immobilized keratinase was much more stable in extreme ph values . a high ph ( ph 12 ) did not decrease keratinase activity of both forms , while acidic ph did . in both cases , the highest hydrolysis of feather was achieved at ph 8.5 . however , when casein was used as the substrate , both forms of keratinase showed the highest activity at ph 9.0 . the shift in ph optimum is most likely due to differences in substrate solubility , steric hindrance , etc . 2 , the immobilized keratinase showed higher heat stability than the free enzyme , indicating that the enzyme stability of the immobilized keratinase was significantly improved . the half - life of immobilized and free keratinase was approximately 50 and 15 h , respectively . after three - day incubation at 40 , the immobilized keratinase maintained 77% activity , whereas the soluble enzyme was 18% active . free keratinase ( 10 g ) and immobilized keratinase ( 0.3 ml ) were added separately into the tube of azo - casein and incubated at 40 with rotation . a 5.0 ml aliquot from each reaction mixture was taken every 2 h , and casein hydrolysis was measured . during a seven - day reaction , immobilized keratinase hydrolyzed casein continuously , and it retained 48% of activity by day 7 . however , free keratinase lost its activity after two days , probably because of autolysis and denaturation ( lin et al . , 1992 ) . this suggests that immobilized keratinase has a higher durability than free enzyme , allowing an extended reaction period ( fig . immobilized keratinase is able to hydrolyze soluble casein and keratin and the insoluble feather keratin . with increased heat stability and ph tolerance , immobilized keratinase for example , bioreactors with immobilized keratinase can convert ground feathers to peptides and amino acids . the results of the present study demonstrated that the level of keratinase attachment is low . therefore , further studies on improving binding ability of keratinase to various carriers are currently in progress .
extracellular keratinase isolated from aspergillus flavus k-03 was immobilized on calcium alginate . the properties and reaction activities of free and immobilized keratinase with calcium alginate were characterized . the immobilized keratinase showed proteolytic activity against soluble azo - casein and azo - keratin , and insoluble feather keratin . heat stability and ph tolerance of keratinase were greatly enhanced by immobilization . it also displayed a higher level of heat stability and an increased tolerance toward alkaline phs compared with free keratinase . during the durability test at 40 , 48% of the original enzyme activity of the immobilized keratinase was remained after 7 days of incubation . the immobilized keratinase exhibited better stability , thus increasing its potential for use in industrial application .
to report a severe ocular complication initiated ten days after intravitreal administration of bevacizumab ( avastin ) , in a patient with exudative age - related macular degeneration ( amd ) . ten days after intravitreal injection of 1.25 mg avastin , the patient manifested acute loss of vision with excruciating pain . an extensive choroidal detachment was evident in close contact with the lens , which necessitated an emergency sclerotomy with reconstruction of the anterior chamber . five months after the injection , the patient complained of mild pain , photophobia , and visual acuity deterioration from the fellow eye . the diagnosis of sympathetic ophthalmia was suggested and treated with intravitreal injections of triamcinolone acetonide every three months with good response , complicated by elevation of intraocular pressure which we managed with ahmet valve implantation . serious ocular complications after intravitreal of avastin can not be excluded , including massive choroidal hemorrhage and sympathetic ophthalmia of the fellow eye . intravitreal administration of bevacizumab ( avastin ; genentech , inc . , san francisco , ca ) , a humanized monoclonal antibody to vascular endothelial growth factor ( vegf ) , has been reported to help in age - related macular degeneration ( amd)13 and in regression of retinal and iris neovascularization.4,5 a 75-year - old male without history of ocular surgical intervention had an intravitreal injection of 1.25 mg bevacizumab ( avastin ) in the right eye ( re ) because of exudative age - related macular degeneration . the patient had mild hypertension and was on metoprolol 50 mg tablets ( lopressor ) bid . the patient did not have bleeding tendency , history of liver disease , and was not on anticoagulants . ten days after intravitreal injection , he manifested acute loss of vision with excruciating pain in the re and he was admitted to our hospital . on presentation , his best - corrected visual acuity was no light perception ( nlp ) in the re and 3/10 from the left eye ( le ) . the conjunctiva was congestive , the cornea edematous , and the anterior chamber ( ac ) was collapsed ( figure 1a ) . an extensive choroidal detachment was evident in close contact with the lens ( figures 1b , 1c).the intraocular pressure in the patient s re was 55 mmhg , while he was on oral carbonic anhydrease inhibitor , drops of xalatan once daily ( latanoprost ) , and cosopt bid ( fixed combination of dorsolamide hydrochloride and timolol maleate ) . the fundus examination of the le revealed extensive confluent soft drusen at the posterior pole . the intravenous administration of carbonic anhydrease inhibitor and hyperosmotic agent failed to reduce the intra - ocular pressure and to alleviate the pain . an emergency sclerotomy with reconstruction of the ac with viscoelastic was performed two days later . two months later , the eye was painless , hypotonous , and there was detachment , residual subretinal fibrosis , and hemorrhage ( figure 1d ) . five months after the injection , the patient complained of mild pain , photophobia , and visual acuity deterioration in his fellow eye . on presentation his best - corrected visual acuity in the le was 1/20 , and the fundus examination revealed extensive confluent drusen at the posterior pole with edema of the optic disc and flare and cells in the vireous . a fundus photograph ( figure 2a ) and fluorescein angiography were inconclusive due to dense cataract ( figures 2b , 2c ) . the diagnosis of sympathetic ophthalmia was suggested . since the patient had a subtotal gastrectomy for uncontrollable bleeding from peptic ulcer 15 years ago , activated by an excessive salicylates intake , we opted for only topical and parabulbar steroids . three months later , his best - corrected visual acuity in the left eye was 2/10 while he still was under treatment . finally the patient overcame his fears and let us to proceed with intravitreal injections of triamcinolone acetonide every three months with a good response6,7 complicated by an elevation of intraocular pressure , which we managed with an ahmet valve implantation . systemic administration of bevacizumab causes serious and , in some cases , fatal adverse reactions . hemoptysis has occurred in patients with non - small cell lung cancer treated with chemotherapy combined with avastin.8 in a small study , the incidence of serious or fatal hemoptysis was 31% in patients with squamous histology and 4% in patients with adenocarcinoma receiving avastin compared to cases treated with chemotherapy alone . it is advised that patients with a history of recent hemoptysis should not receive avastin.8 when data were pooled from two randomized phase ii studies utilizing bevacizumab with 5-fu / leucovorin , and also in a third treatment arm of bevacizumab/5-fu / lv of a randomized phase iii study , a few toxicities were uniformly encountered in all of the above studies.9 the reported toxicities were grade 3 ( medically manageable ) hypertension ( 3%16% ) , hemorrhage ( 2%9.3% ) , gastrointestinal perforation ( 1.5% ) , arterial thromboembolism ( 3.8% ) , wound - healing problems ( 1%2% ) , and proteinuria ( 1%2%).9 the reported ocular complications of intravitreal injection for armd so far , are tears of retinal pigment epithelium1013 and complications related to the injection site mainly , as conjunctival hyperemia and subconjunctival hemorrhage.14 our case demonstrates a serious ocular complication initiated ten days after intravitreal administration of avastin in a patient with exudative amd .
purpose : to report a severe ocular complication initiated ten days after intravitreal administration of bevacizumab ( avastin ) , in a patient with exudative age - related macular degeneration ( amd).patients and method : case report.results:ten days after intravitreal injection of 1.25 mg avastin , the patient manifested acute loss of vision with excruciating pain . an extensive choroidal detachment was evident in close contact with the lens , which necessitated an emergency sclerotomy with reconstruction of the anterior chamber . four months later , the eye proceeded to phthisis bulbi . five months after the injection , the patient complained of mild pain , photophobia , and visual acuity deterioration from the fellow eye . the diagnosis of sympathetic ophthalmia was suggested and treated with intravitreal injections of triamcinolone acetonide every three months with good response , complicated by elevation of intraocular pressure which we managed with ahmet valve implantation.conclusion:serious ocular complications after intravitreal of avastin can not be excluded , including massive choroidal hemorrhage and sympathetic ophthalmia of the fellow eye .
in 2009 , a 52-year - old woman presented with a single lesion on her nose , which started as a papule , referred to sedighe tahereh clinic , isfahan , iran . the lesion had existed for a period of 14 months and was slowly increasing in size , enlarging to a plaque . the diagnosis of leishmaniasis was confirmed with a positive smear of the lesion showing leishmania bodies about 1 year before . all five members of her family had had a history of proven leishmaniasis . in the past medical history , the patient was a renal failure case since 11 years before and received a renal transplant 4 years after the diagnosis of renal failure . she was receiving oral mycophenolate mofetil ( 2 g daily ) and cyclosporine ( 100 mg daily ) . a 33 cm indurated ulcer with elevated borders was present on the tip of her nose ( figure 1 ) . her therapeutic plan was intralesional glucantime injection ( approximately 1 ml of 1.5 g vial per week , intralesional injection ) . after completing a therapeutic course of 20 sessions receiving intralesional glucantime injections , she was considered as glucantime therapy resistant . the occurrence of malignant neoplasms in sites of scars is an infrequent but well - known phenomenon.5 although the coexistence of cutaneous leishmaniasis and bcc may have been coincidental , some studies suggest that an association between these two entities does exist.6 leishmaniasis can directly or indirectly alter the diagnosis and course of different malignancies.7 there are reports of bcc in chronic leg ulcers.8 cases of bcc developing in a leishmania scar have also been documented,9 but to our knowledge , cases of both leishmaniasis and bcc in the same site and the same lesion are rare.10 however , in this case , solid organ transplantation and long term immuno suppressive therapy should be considered as risk factors for malignancy . advances in effective immuno suppression after organ transplantation have led to increased risk of malignancies , particularly skin cancers11 including squamous cell carcinoma , basal bcc and malignant melanoma.12 thus , malignancies should be considered in the differential diagnosis of leishmaniasis lesions difficult to treat . the possible role of cutaneous leishmaniasis , as a predisposing factor for skin cancer , should also be kept in mind . aa was the main therapeutic physician and helped write the manuscript . i m and pk contributed in writing the manuscript .
leishmaniasis is a protozoan infection due to organisms of the genus leishmania . the differential diagnosis of cutaneous leishmaniasis includes arthropod bites , basal cell carcinoma ( bcc ) and other malignancies . bcc is the most common form of skin cancer . we present a case of cutaneous leishmaniasis resistant to standard intralesional glucantime injection in an immunocompromised patient , which was proved to be bcc after surgical excision .
the chapel hill consensus conference classification system classifies vasculitis according to vessel size and characteristic clinical and histopathological features . anti - neutrophil cytoplasmic antibodies ( anca ) have been used to further characterize the small - vessel vasculitides . large - vessel vasculitis comprises fewer clinical syndromes than medium- or small - vessel vasculitides , most commonly giant cell arteritis and takayasu s arteritis . large vessel involvement in anca - associated vasculitis is uncommon and , when present , often portends a poor prognosis , with outcomes involving aortic dissection , rupture and , possibly , stenosing arteritis . we describe a case of asymptomatic large vessel involvement in a biopsy - proven canca - associated small - vessel vasculitis . a 64-year - old greek man , previously well , presented with a 4-week history of fever , malaise , dysuria and haematuria . a trial of antibiotics ( roxithromycin and cephalexin ) from his local doctor had not improved his symptoms . at presentation relevant physical findings included crackles at the left lower lung field and minimal oedema in the lower extremities . initial laboratory investigations demonstrated a serum creatinine of 92 mol / l , with elevated inflammatory markers ( c - reactive protein 29 mg / l ; erythrocyte sedimentation rate 93 mm / h ) . initial urine microscopy revealed heavy isomorphic haematuria without cellular casts and an escherichia coli infection on culture . after treatment of the infection , urine microscopy demonstrated > 1000 10/l dysmorphic red cells and 24-h urine collection revealed significant proteinuria ( 1.05 g / day ) , with a creatinine clearance of 33 ml / min . a blurred outline of the abdominal aorta was revealed on computerized tomography ( ct ) scan , and subsequent ct aortogram revealed near circumferential soft tissue oedema of both the thoracic arch and inferior to the origin of the renal arteries . ( b ) ct scan of abdominal aorta at 2 months ( inflammation indicated by arrowheads ) . renal biopsy confirmed the presence of a pauci - immune , necrotizing crescentic glomerulonephritis with fibrocellular crescents present in 40% of the glomeruli . a positive anca titre was subsequently detected with granular cytoplasmic staining showing specificity for proteinase-3 ( 55 u / ml ) . oral cyclophosphamide ( 1.5 mg / kg / day ) and intravenous methylprednisolone ( 500 mg / day ) were commenced ; however , within a week , the patient developed diffuse alveolar haemorrhage . he required a total of six plasma exchanges > 10 days . despite treatment , the serum creatinine continued to rise ( peaking at 650 the patient was discharged on cyclophosphamide ( 1.5 mg / kg / day ) and prednisolone ( 60 mg / day ) . two months after discharge , investigations revealed a serum creatinine of 167 mol / l ( canca 30 u / ml ) , figure 2 . clinical remission was evident after 3 months treatment with cyclophosphamide , and methotrexate was commenced after the patient proved intolerant to azathioprine . twenty months later , the patient has remained well with a serum creatinine of 115 mol / l , with negligible haematuria ( 5 10/l erythrocytes on urine microscopy ) and proteinuria ( protein : creatinine ratio 0.010 g / mmol ) . there is no evidence of aortic vasculitis on ct scan , although the mid - thoracic and abdominal aorta remain mildly tortuous and ectatic . rbc , red blood cells ; hpf , high power field ; pcr , protein : creatinine ratio ; esr , erythrocyte sedimentation rate ; crp , c - reactive protein . anca - associated large - vessel vasculitis ( alv ) has rarely been reported in european and north american populations . it is possible that common pathological processes are involved in both large- and small - vessel vasculitis . intimal injury may be the initial insult , progressing to inflammation of the medial layer and then adventitia , with resulting transmural aortitis . alternatively , the large vessel wall changes seen on imaging may be a result of vasculitis of the vasa vasorum of the aortic wall [ 35 ] . the presentation of anca - associated large - vessel vasculitis is diverse but appears intrinsically different from the stenosing lesions of takayasu s vasculitis [ 69 ] . case reports of wegener s granulomatosis with large vessel involvement also include periaortitis , with resultant aortic aneurysm and dissection [ 2 , 10 ] . given the infrequent number of reports , there is no consensus on the management of patients with alv . however , apart from surgical intervention for dissection or rupture , the finding of large vessel involvement does not appear to require additional treatment . most patients have been managed with an extended period of immunosuppression using corticosteroids and cyclophosphamide , following similar regimens for small vessel disease . our patient underwent a follow - up ct aortogram 2 and 20 months after diagnosis . for patients with significant renal impairment , contrast ct scans may risk contrast - induced nephropathy , and alternative options include magnetic resonance imaging or positron emission tomography scanning . while anca - associated disease is often considered to be limited to small vessels , this case highlights that large vessels can also be affected . its true frequency is unknown as involvement appears to be asymptomatic ; however , though not seen in our patient , it can be associated with a poor outcome due to vessel rupture or dissection . this case illustrates the need for awareness of potential large vessel involvement in anca - associated small vessel disease .
anti - neutrophil cytoplasmic antibody ( anca)-associated vasculitis is traditionally viewed as a small vessel disease . we report a patient with canca antibodies directed against proteinase-3 with asymptomatic aortic involvement , in combination with diffuse alveolar haemorrhage and pauci - immune , necrotizing crescentic glomerulonephritis . a review of the literature is discussed .
fibrinogen is the primary substrate of the coagulation system and is fundamental to hemostasis . fibrinogen falls to critical levels soon after the onset of major trauma hemorrhage but is not considered part of routine clotting assays . to maintain the integrity of coagulation function it is recommended that fibrinogen is replaced when it falls below 150 to 200?mg / dl . early recognition and replacement has the potential to rapidly reverse trauma - induced coagulopathy , arrest hemorrhage and improve outcomes . schlimp and colleagues have demonstrated that it is possible to estimate fibrinogen in the emergency department using widely available point - of - care assays . the literature contains numerous reports of improved outcomes when early high - dose plasma is administered as part of a massive hemorrhage protocol . fibrinogen supplementation with either cryoprecipitate ( the uk or usa ) or fibrinogen concentrate ( europe ) is often delayed or considered second line in the empiric delivery of hemostatic coagulation therapy . early fibrinogen supplementation is commonplace in postpartum hemorrhage and cardiac surgery , with only limited data indicating a potential therapeutic benefit in trauma . each unit of plasma contains approximately 500?mg fibrinogen , and therefore the efficacy of large - volume plasma transfusions in massive hemorrhage protocols may in part be due to restoration of fibrinogen levels . for this reason , early fibrinogen replacement is the subject of two pilot randomized controlled trials in the uk ( cryostat ) and austria ( fitic ) due to report later this year . fibrinogen falls early , rapidly reaches critical threshold values relative to other coagulation factors and is associated with higher transfusion requirements and increased mortality . schlimp and colleagues have confirmed that hypofibrinogenemia is common in major trauma and is an almost universal problem in those patients presenting with hemoglobin < 8?g / dl . the identification of early hypofibrinogenemia requires a laboratory assay ; for example , the clauss method . fibrinogen levels are rarely available to the trauma physician in a clinically relevant timeframe and thus fibrinogen supplementation ( for example , cryoprecipitate ) is often delayed . rotational thromboelastometry and thromboelastography provide a more rapid and global assessment of coagulation and can provide an estimate of the contribution of functional fibrinogen to clot strength . these tests are expensive , however , and although available at point of care they require further modification , simplification and validation before this technology has global appeal for the trauma community . this study has shown it is possible to risk stratify patients for low or critical fibrinogen levels , using hemoglobin and base excess that are rapidly available in emergency trauma care . however , even with the addition of the injury severity score , which is not available within the first few hours of care , the regression model only accounted for 51% of the variation in fibrinogen . other important , iatrogenic and patient factors therefore contribute significantly to the depletion of fibrinogen in trauma hemorrhage . the mechanism by which fibrinogen loss occurs in trauma continues to be the subject of ongoing debate and research . there is limited evidence to support a consumptive process such as disseminated intravascular coagulation , although clearly fibrinogen will be utilized because the coagulation system is activated following hemorrhage . acidosis and hypothermia compound trauma - induced coagulopathy and have profound effects on fibrinogen breakdown and synthesis , which is supported by the findings of the current study that demonstrated critical and low levels of fibrinogen in 81% and 63% of shocked patients , respectively . finally , resuscitation with gelatins and hydroxyethyl starch solution reduce the concentration of fibrinogen through dilution and interfere with fibrin polymerization . fibrinogen is thought to only contribute approximately one - third of viscoelastic strength to the overall clot , with platelets being the major determinant of clot firmness in rotational thromboelastometry / thromboelastography . estimation of fibrinogen deficit alone risks missing the global derangement of hemostasis typified by trauma - induced coagulopathy . the estimation of fibrinogen levels by schlimp and colleagues reminds us that fibrinogen loss is not only rapid and significant in trauma but is detectable in the emergency department . metabolic acidosis , injury severity and hemorrhage reduce fibrinogen , but other endogenous and iatrogenic factors contribute to the depletion of this primary substrate of coagulation . rapid identification of hypofibrinogenemia should be routine in all injured patients and a priority in major trauma hemorrhage , either through estimation or functional assessment with rotational thromboelastometry / thromboelastography . understanding the mechanism by which fibrinogen is lost and the efficacy of early fibrinogen replacement are research imperatives , and are likely to yield significant therapeutic benefit . rd and kb both received departmental funding for study support costs from haemonetics corporation ( braintree , ma , usa ) and equipment / reagent grants from tem innovations gmbh ( munich , germany ) .
fibrinogen is fundamental to hemostasis and falls rapidly in trauma hemorrhage , although levels are not routinely measured in the acute bleeding episode . prompt identification of critically low levels of fibrinogen and early supplementation has the potential to correct trauma - induced coagulation and improve outcomes . early estimation of hypofibrinogenemia is possible using surrogate markers of shock and hemorrhage ; for example , hemoglobin and base excess . rapid replacement with fibrinogen concentrate or cryoprecipitate should be considered a clinical priority in major trauma hemorrhage .
endoscopic mucosal resection ( emr ) is an endoscopic alternative to surgical resection of mucosal and submucosal neoplastic lesions and intramucosal cancers . lesions limited to the mucosa and the superficial layers of the submucosa appear to be the most amenable to endoscopic cure . the techniques for emr can be broadly divided into two groups : suction and non - suction techniques . this topic will provide an overview of useful accessories for colorectal emr such as cap and band . this technique is most commonly performed with a transparent cap ( disposable distal attachment ; olympus , tokyo , japan ) attached to the tip of the endoscope . cap - assisted colonoscopy ( cac ) uses a transparent plastic hood attached to the tip of the colonoscope to flatten the semilunar folds and improve mucosal exposure . several studies have examined that cac facilitated shortening of the cecal intubation time in difficult cases , and was more sensitive for detecting adenomas than was conventional colonoscopy.1,2 a meta - analysis and systemic review suggests that a transparent cap on the end of the colonoscope may give a marginally faster cecal intubation time compared with standard colonoscopy . it also suggests that there is a better polyp detection rate and less pain with the cap.3,4 cac may reduce the time required for colonoscopic emr of each polyp and may also improve the polyp detection rate.5 this technique is most commonly performed using a distal attachment fitted to the distal end of the endoscope , with saline solution with a low concentration of epinephrine injected underneath the lesion . the lesion was snared and drawn into the cap using the suction function of the endoscope , and then ligated and resected using electrocautery . the most serious complication of this technique may be perforation due to the lack of submucosal saline injection and too much suction . therefore , large volume injection , which creates a large bleb and potentially reduces the risk of perforation , is recommended.6 moreover , emr using a cap ( emr - c ) is effective at removal of carcinoid tumor which is diagnosed increasingly each year . it is widely accepted that rectal carcinoid tumors with a diameter of 10 mm or less can be treated with local excision , including endoscopic resection.7 complete resection of rectal carcinoid tumors , however , is difficult to achieve with conventional endoscopic resection techniques because these tumors often extend into the submucosa . resection via polypectomy or conventional emr is often associated with resection margin involvement , which necessitates further intervention.8,9 the rate of positive resection margin for tumor is lower in the group of emr - c than conventional polypectomy group.10 - 12 and secondary endoscopic treatment for remnant lesions of rectal carcinoid tumors after primary emr or polypectomy is technically difficult because of fibrosis of the residual tissues . emr - c , a method to resect the submucosal layer by suction by using a transparent cap , may be feasible as a salvage treatment a variation of the suction technique is the band and snare procedure . during the band ( endoscopic ligator ; conmed , new york , ny , usa ) and snare procedure , tissue is banded using an esophageal variceal banding device and then snared off in the standard fashion.14 after submucosal injection beneath the lesion to elevate it away from the muscularis propria , the lesion was aspired into the ligator device and the elastic band was then deployed . next , snare resection was performed below the band with a blended electrosurgical current ( fig . the resection specimen was then removed by aspiration into the cap or by retrieving it with a grasping forceps . after resection of the specimen , the ulcer floor was endoscopically closed with clips to prevent postoperative bleeding and perforation . as with emr - c , complete resection of rectal carcinoid tumor is important but is difficult to achieve with conventional endoscopic resection techniques because these tumors often extend into the submucosa . resection via polypectomy or conventional emr is often associated with resection margin involvement . among various endoscopic resection techniques , endoscopic submucosal resection with a ligation device ( cac is more sensitive for detecting adenomas and may reduce the time required for colonoscopic emr of each polyp . emr - c and emr with a ligation device ( or esmr - l ) may be a superior method to conventional emr for removing small rectal carcinoids .
endoscopic mucosal resection ( emr ) is an endoscopic alternative to surgical resection of mucosal and submucosal neoplastic lesions . prior to the development of knives , emr could be performed with accessories to elevate the lesion . after the development of various knives , en bloc resection was possible without other accessories . so , recently , simple snaring without suction or endoscopic submucosal dissection using knife in the epithelial lesions such as adenoma or early mucosal cancer has been performed . however , for easy and complete resection of subepithelial lesions such as carcinoid tumor , a few accessories are needed . complete resection of rectal carcinoid tumors is difficult to achieve with conventional endoscopic resection techniques because these tumors often extend into the submucosa . the rate of positive resection margin for tumor is lower in the group of emr using a cap ( emr - c ) or emr with a ligation device ( emr - l ) than conventional emr group . emr - c and emr - l ( or endoscopic submucosal resection with a ligation device ) may be a superior method to conventional emr for removing small rectal carcinoid tumors .
visual acuity was 20/20 in the right eye and 20/25 in the left eye . slit lamp examination demonstrated clear cornea in both eyes , no anterior chamber cells in the right eye , and rare cells in the left eye . fundus examination of the left eye revealed a large 7 4 disk diameter ( dd ) irregular lesion superonasal to the optic nerve consisting of retinal whitening with granular appearance and characteristic isolated satellite the diagnosis of cytomegalovirus retinitis was made based on the fundus examination and clinical history . the patient was admitted for induction therapy with intravenous ganciclovir , 5 mg / kg , and was re - started on highly active antiretroviral therapy ( haart ) therapy . 1a superonasal cmv retinitis lesion with retinal granular necrotic appearance and few hemorrhages at the time of diagnosis . b two weeks after diagnosis , inferior posterior subhyaloid precipitates ( psps ) were observed . c fundus photo showing the psps below inferotemporal arcade . d spectral - domain optical coherence tomography showing multiple psps a superonasal cmv retinitis lesion with retinal granular necrotic appearance and few hemorrhages at the time of diagnosis . b two weeks after diagnosis , inferior posterior subhyaloid precipitates ( psps ) were observed . d spectral - domain optical coherence tomography showing multiple psps one week later , white granular subhyaloid opacities located 2 dd below the inferotemporal arcade and away from the main cmv lesion were observed on fundus examination ( fig . a spectral - domain optical coherence tomography ( sd - oct ) demonstrated hyperreflective round opacities clearly located in the space between the posterior hyaloid and the retina . twelve days after presentation , the superonasal cmv lesion matured into a grayish gliotic scar with several atrophic retinal holes and a localized rd . one month after the diagnosis of cmv and 2 weeks after the appearance of the rd , the sd - oct showed that the posterior hyaloid was still attached to the retina over the area of cmv lesion , bridging the retinal holes and keeping a localized rhegmatogenous rd stable ( fig . b showing the posterior hyaloid attached to the retina with overlying inflammation a fundus photo showing thinned retina and rd . b showing the posterior hyaloid attached to the retina with overlying inflammation four months after the diagnosis , while still on oral valganciclovir maintenance dose , the localized rd remained stable with attached posterior hyaloid ( fig . the psps resolved , but a new small necrotic lesion appeared superior to the optic nerve . a week later , another satellite lesion developed , and the patient s oral valganciclovir dose was increased from 900 mg daily to 900 mg twice daily . in addition , an intravitreal ganciclovir injection ( 2 mg/0.05 ml ) was administered ( fig . one week after intravitreal injection of ganciclovir , the rd progressed threatening the macula and lens - sparing pars plana vitrectomy ( ppv ) with silicone oil and ganciclovir implant ( vitrasert ) was performed . four months after the surgery , vision was 20/25 and retina was attached under silicone oil ( fig . 3a , b four months after the diagnosis , spectral - domain oct cuts through the superonasal cmv lesion demonstrate detached thin necrotic retina with multiple discontinuities and persistent hyaloidal attachment bridging the retinal holes . c fundus photography demonstrates two small new necrotic retinal lesions above the optic nerve and resolution of the inferior pspsfig . b oct showing attached macula a , b four months after the diagnosis , spectral - domain oct cuts through the superonasal cmv lesion demonstrate detached thin necrotic retina with multiple discontinuities and persistent hyaloidal attachment bridging the retinal holes . c fundus photography demonstrates two small new necrotic retinal lesions above the optic nerve and resolution of the inferior psps a fundus photos 2 months after ppv with silicone oil and ganciclovir implant . a 41-year - old healthy white female presented with blurry vision and floaters in the right eye for 4 weeks . she was referred by an outside ophthalmologist for possible acute retinal necrosis and was initiated on valacyclovir hydrochloride ( valtrex ) 1 g three times daily . the patient had a history of three episodes of shingles involving her scalp , right torso , and leg . best corrected visual acuity was 20/25 od and 20/20 os . slit lamp examination demonstrated diffuse white keratic precipitates in the right eye with 2 + anterior chamber cell and no cell in the left eye . dilated fundus examination showed 3 + vitreous cell in the right eye and extensive retinal necrosis with whitening and hemorrhage superiorly ( fig . c , d sd - oct demonstrating the posterior subhyaloid precipitates a right eye with extensive retinal necrotic lesion with overlying hemorrhages . c , d sd - oct demonstrating the posterior subhyaloid precipitates oct evaluation of the right eye demonstrated no evidence of fluid in the macula , but demonstrated the presence of psps overlying the retina away from the retinitis lesion , along the inferotemporal arcade ( fig . the patient underwent an anterior chamber tap for viral pcr studies and was treated with intravitreal ganciclovir 2 mg/0.05 ml . three weeks after presentation , she had received a total of four intravitreal ganciclovir injections and barricade laser superiorly by her local ophthalmologist . the keratic precipitates decreased and trace anterior chamber cell was present in the right eye . fundus examination showed laser demarcating and improved retinal necrosis with pigmentary changes and mild residual whitening and hemorrhages ( fig . the patient was continued on oral valganciclovir 900 mg twice daily and was scheduled for an immunology evaluation . 6laser demarcation is observed with residual whitening and retinal hemorrhages laser demarcation is observed with residual whitening and retinal hemorrhages such precipitates have not been previously well documented and reported ; therefore , the fundus photos and spectral - domain octs presented here are unique in the literature . the octs clearly show that these precipitates are located between the posterior hyaloid and the retina and therefore appear to be distinct from vitreous precipitates , which have been previously reported . the curvilinear organization of psps in the posterior segment suggest that they descent with gravity and collect along the attachment of the posterior hyaloid to underlying retina . these psps cleared during the course of treatment and therefore may potentially be followed as another clinical sign of response to therapy . we can speculate that because the psps clear with treatment , they are most likely made up of inflammatory cells . in conclusion , we present a novel finding of posterior subhyaloid precipitates , which were clearly documented by the spectral - domain oct . we expect that these cases will contribute to the further characterization of the cmv retinitis manifestation in the era of haart and oct imaging . the authors report no conflicts of interest . the authors alone are responsible for the content and writing of the paper . this article is distributed under the terms of the creative commons attribution license which permits any use , distribution and reproduction in any medium , provided the original author(s ) and source are credited .
purposethis study aims to report a novel finding of posterior subhyaloid precipitates ( psps ) in two patients with cytomegalovirus ( cmv ) retinitis.methodsa small case series was conducted.resultsclinical findings , treatment , and follow - up of two patients with cmv and psps are presented.conclusionsinflammatory precipitates may collect in the posterior subhyaloid space in acute cmv retinitis and resolve with treatment .
the incidence of duodenal atresia has been estimated at 1 in 6000 - 1 in 10,000 live births . duodenal atresia is thought to result from problems during embryo 's development , in which the duodenum does not normally change from a solid to a tube - like structure . we present here a case , which queries the embroyological basis of duodenal atresia possibly due to vascular accident . a 2-day - old preterm ( 33 weeks ) , weighing 1.3 kg neonate was referred to us from neonatal intensive care unit as it was prenataly diagnosed as a case of duodenal obstruction on ultrasonographic findings of polyhydraminos with the double - bubble appearance . on examination , the baby was alert , active , and had no obvious external morphological congenital anomalies . double - bubble appearance of duodenal atresia with total absence of distal bowel gas [ figure 1 ] . , it was revealed that duodenum was atretic and there was marked dilation of proximal duodenum . the upper 15 cm of jejunum had apple - peel configuration [ figure 2 ] . this part of bowel was spiraling around a retrograde vascular arcade , rest of the small and large bowel was normal . as only 15 cm of jejunum was having the apple - peel configuration with precarious blood - supply , we decided to resect this part of the bowel . end to oblique duodeno - jejunal anastomosis was done along with the plication of grossly dilated proximal duodenum [ figure 3 ] . the baby was on full breast feed by postoperative day 10 and discharged from hospital . x - ray flat plate abdomen - showing classical double - bubble appearance of duodenal atresia dilated duodenum and apple - peel jejunal atresia end to oblique duodeno - jejunal anastomosis done this defect is usually diagnosed prenatally via ultrasound or shortly after delivery by the presence of vomiting ( mostly bilious ) after each feed . approximately 50% of infants with duodenal atresia / stenosis have another anomaly , including cardiac , genitourinary , or anorectal defects , and annular pancreas . although no specific genetic abnormality is known to cause duodenal atresia , the number of reports of the anomaly occurring among siblings and among several generation of a family , as well as its frequent association with trisomy 21 , suggests one may be present . a severe form of intestinal atresia / stenosis is described as apple - peel deformity . this name is derived from the appearance of the intestine as it spirals around the blood - supply and resembles an apple - peel . the mortality of apple - peel atresia remains high though it has come down greatly in most of the reported series in last two decades . the embryological etiology of the duodenal atresia differs from that of small bowel atresia . in the 4 week of development rapid proliferation of the gut epithelium in the 6 week of development results in obliteration of the intestinal lumen . an error in this recanalization process is considered to be the embryological basis of duodenal atresia and stenosis . this differs from jejuno - ileal atresia , which is assumed to result from vascular disruption phenomenon during the later phases of gestation . apple - peel atresia is a rare variant of intestinal atresia in which the distal bowel is precariously supplied by a retrograde vascular arcade from the ileocolic , right colic or inferior mesenteric arteries . our patient with duodenal atresia had apple - peel configuration of upper 15 cm of jejunum . this association of duodenal atresia with apple - peel small bowel atresia is extremely rare . the presence of apple - peel atresia of upper jejunum being precariously supplied by retrograde vascular arcade and absent associated congenital anomalies suggest that duodenal atresia in our case resulted from vascular disruption phenomenon in late gestation . some literature in the past reportedly favored vascular accidents over recanalization defects in the cases of duodenal atresia . likewise , our case also with findings described channelizes our thoughts to an embryological variant duodenal atresia . duodenal atresia with the apple - peel appearance is a rare anomaly with limited cases and literature in the past . nevertheless , though the current understanding favors recanalization theory , but rarely vascular disruption phenomenon can cause duodenal atresia with the apple - peel appearance which may indicate insult occurring in late gestation .
classically , embryology of duodenal atresia has been linked to defect in recanalization process , while apple - peal atresia of small bowel has been considered as due to vascular accident during embryonic life . we present a 33 week preterm neonate with duodenal atresia with the apple - peal appearance of proximal jejunum for which resection of the jejunum with apple - peal configuration , plication of the duodenum , and duodono - jejunal anastomosis was done . thus , this rare case of ours questions the embryology of duodenal atresia with the apple - peal appearance suggesting it to be due to a vascular disruption phenomenon during embryonic life .
spider bite is endemic in parts of north america , mexico , tropical belt of africa and europe and can cause serious systemic manifestations . loxosceles spiders belong to the family loxoscelidae / sicariidae . of the 13 species of loxosceles loxosceles reclusa , or the brown recluse spider , is the spider most commonly responsible for this injury . dermonecrotic arachnidism refers to the local skin and tissue injury as a result of spider - bite . loxoscelism is the term used to describe the systemic clinical syndrome caused by envenomation from the brown spiders . cutaneous manifestations occur in around 80% cases around the site of bite , predominantly the lower limbs . the initial cutaneous manifestation is that of an erythematous halo with edema around the bite site . the erythematous margin around the site continues to enlarge peripherally , secondary to gravitational spread of the venom into the tissues . this gradually gives way to vesicles and finally a dark eschar or a necrotic ulcer . mild systemic effects such as fever , malaise , pruritus and exanthema are common , whereas intravascular hemolysis and coagulation , sometimes accompanied by thrombocytopenia and renal failure , occur in approximately 16% of those who receive the bite . rarely ( < 1% of the cases of suspected l. reclusa bites with a higher incidence in south american loxoscelism ) , recluse venom may cause hemolysis , disseminated intravascular coagulation , which can lead to serious injury and possibly death . although india is a home to a diverse array of arachnids , according to the latest updated list of spider species found in india , loxosceles rufescens is the only member of the loxosceles genus described in india . systemic envenomation ( especially renal failure ) from loxosceles bite has been rarely described from india . a 23-year - old male was bitten by a spider in the dorsal aspect of his right forearm . he developed a painful blister with red margins in the distal forearm , around the site of bite , which subsequently turned into a necrotic lesion . he had excruciating pain and erythematous swelling of the right hand and around the bite site . initial blood urea was 133 mg / dl , creatinine 3.4 mg / dl , serum na 131.6 meq / l and k 4.67 meq / l . hemoglobin was 6.2 g / dl , total leucocyte count 12,000/cmm ( n84l12e2m2b0 ) with normal platelet counts . he was referred to our hospital for worsening renal function and deteriorating skin lesions . on arrival there was blackish discoloration of the right distal forearm and hands and a gravitational pattern of involvement from the bite site down into the hands [ figure 1 ] . radial pulse was palpable and he had preserved sensation in the fingers of his right hand . though the spider was not brought , it was identified to be the brown recluse spider ( loxosceles spp ) based on the description and on showing representative pictures . gravitational pattern of dermonecrosis following the bite of brown recluse spider ( loxosceles spp ) , day 5 investigations revealed hemoglobin of 4.3 gm / dl with normal leucocyte and platelet counts with a reticulocyte count of 9.1% . he had advanced renal failure with normal electrolytes , serum urea 206 mg / dl and creatinine 6.6 mg / dl . liver function tests were normal with mild unconjugated hyperbilirubinemia ( total bilirubin was 2.4 mg / dl with unconjugated bilirubin of 1.9 / l , lactate dehydrogenase ( ldh ) 588 u / l ( normal range : < 200 u / l ) and serum haptoglobin was 18 mg / dl ( normal ) . he was discharged after 3 weeks with a daily urine output of 1.8 l , serum creatinine of 1.8 mg / dl , hemoglobin 9.2 gm / dl and ldh of 180 mg / dl . renal function had normalized ( serum creatinine 0.9 mg / dl , normal urine microscopy ) and ulcer had healed with desquamation of the involved skin [ figure 2 ] . healed skin lesion of the same patient at 6 weeks intravascular hemolysis was evidenced by low hemoglobin level , elevated reticulocyte counts with elevated unconjugated bilirubin , high serum ldh and low serum haptoglobin levels . the likely etiology of renal failure in our case is hemolysis leading to acute tubular injury . there was no evidence of myonecrosis / rhabdomyolysis as evidenced by normal serum cpk and urine myoglobin levels . although the spider was not brought by the patient for identification , the features were typical of loxoscelism , especially the gravitational pattern of dermonecrosis . most of the case series of loxoscelism have documented dermonecrosis with a few cases of hemolysis and rare cases of renal injury . wound debridement , elevation , application of ice and immobilization of the affected area may help ameliorate the extent of cutaneous damage . dapsone has been recommended by some authorities to treat dermonecrosis on account of its leucocyte inhibiting properties . patients exhibiting signs of systemic toxicity should be admitted and evaluated for evidence of coagulopathy and renal failure . it has been found that sphingomyelinase activity of the loxosceles toxin induces activation of an endogenous metalloproteinase , which then cleaves glycophorins thus rendering it susceptible to complement mediated lysis . in their study observed the transfer of complement - dependent hemolysis to other cells , suggesting that the loxosceles toxins can act on multiple cells . this observation can explain the relatively significant extent of hemolysis observed in patients with inoculation of small amounts of the toxin ( max 30 g ) . loxoscelism causes necrotic dermatologic injury through a unique enzyme ; sphingomyelinase d. loxosceles toxin has also been shown to have hyaluronidase , alkaline phosphatase and esterase activity . these cause degradation of the extracellular matrix and contribute to the spread of the toxin in tissue compartment . the dermatohistopathology of loxosceles bites include dermal edema , thickening of blood vessel endothelium , leukocyte infiltration , intravascular coagulation , vasodilatation , destruction of blood vessel walls and hemorrhage . renal injury in loxoscelism has been attributed to pigmentary nephropathy due to hemoglobin or myoglobin , secondary to hemolysis or rhabdomyolysis . however , in the absence of effective reporting systems , many fatal or near - fatal envenomations go unreported . careful clinical and entomological studies should be done to look into this neglected disease entity . it should be borne in mind that a case presenting with acute dermal inflammation or ulceration in a pattern , along with features of hemolysis or rhabdomyolysis or , in rare instances , acute kidney injury could be due to loxoscelism and it is a close mimicker of hemotoxic snake bite .
spiders of the loxosceles species can cause dermonecrosis and acute kidney injury ( aki ) . hemolysis , rhabdomyolysis and direct toxin - mediated renal damage have been postulated . there are very few reports of loxoscelism from india . we report a case of aki , hemolysis and a gravitational pattern of ulceration following the bite of the brown recluse spider ( loxosceles spp ) .
capecitabine ( xeloda ) is an orally administered antineoplastic pro - drug , which in tumour tissue is preferentially converted to 5-fluorouracil ( 5-fu ) by thymidine phosphorylase ( tp ) . it is used as adjuvant chemotherapy for metastatic breast and colon cancer . common dose - limiting side - effects of capecitabine include hyperbilirubinemia , nausea , vomiting , diarrhoea , bone marrow suppression and hand and foot syndrome ( hfs ) [ 13 ] . capecitabine - induced skin pigmentation may occur in the context of skin manifestations of hfs [ 49 ] . a 37-year - old woman ( phototype ii ) presented with irregular pigmented macules in the plantar regions . the lesions were roundish in shape , 0.20.5 cm in diameter and light to dark brown in colour with poorly defined margins ( figure 1 ) . dermoscopic examination showed parallel ridge pattern with preservation of eccrine gland apertures ( figure 2 ) . clinical history revealed that the patient had taken capecitabine ( xeloda ) in the last two months as adjuvant chemotherapy for recurrent breast cancer . hyperpigmentation was noticed after two cycles of therapy ( cycles consisted of 1250 mg / m twice daily for two weeks , separated by a 7-day interval ) . assuming that capecitabine was responsible for the lesions , we enquired whether the patient had ever previously had signs or symptoms of hfs . the patient replied that she had never previously experienced paresthesia , burning pain , erythema , swelling , blistering , desquamation or ulceration of the feet . to our knowledge , very few cases of capecitabine - induced hyperpigmentation have been reported [ 613 ] , and have mainly been in patients with phototype iv or v ( indians , africans , asians and aborigines ) , with only one case in a patient with phototype iii , suggesting a racial predisposition . in all eleven cases , hyperpigmentation involved palmar and plantar skin ; the oral mucosa was also involved in five cases ( four africans and one indian ) . in all cases , hyperpigmentation preceded hfs6 [ 811 ] , or was associated with palmo - plantar dysesthesia . indeed , many authors regard hyperpigmentation as one of the initial manifestation of hfs ( grade 1 hfs ) [ 79 ] . by contrast , we observed capecitabine - induced hyperpigmentation limited to the plantar regions in a phototype ii patient without any history , sign or symptom of hfs . hyperpigmentation is a well - known consequence of the antiproliferative drug 5-fluorouracil ( a capecitabine precursor ) , generally occurring when therapy is administered intravenously . in such cases , hyperpigmentation usually occurs on photo - exposed skin ( photomediated pathogenetic mechanism ) . however , our patient presented lentiginous hyperpigmentation on photoprotected areas , and there was no history of sun exposure since the start of the therapy . palms and soles are considered more sensitive to cytotoxic drugs due to the high proliferation rate of epidermal basal cells . in addition , there is evidence that capecitabinemetabolizing enzymes ( i.e. , tp and dihydropyrimidine dehydrogenase ) are not only expressed in tumour tissue but also in palmar and plantar skin . this suggests that drug metabolism was involved in our case more than racial predisposition or photo - sensitization . however , further studies are needed to assess the exact pathogenesis of hyperpigmentation induced by antiproliferative drugs manifesting in photoprotected areas in patients with phototypes i ii . , various authors have highlighted parallel ridge pattern not only in acral melanoma but also in certain benign plantar lesions , such as congenital or acquired acral nevi , subcorneal hemorrhage , dye - related pigmentation and drug - induced hyperpigmentation , especially in patients with phototypes iii vi [ 1317 ] . in particular , fracaroli et al emphasised the importance of obliteration of eccrine gland ducts by neoplastic proliferation of melanocytes in distinguishing malignant and benign acral pigmented lesions with parallel ridge pattern . indeed , the coexistence these two dermoscopic signs ( i.e. , parallel ridge pattern and acrosyringium obliteration ) is considered highly indicative of acral melanoma . however , it should be borne in mind that these two signs can also be detected in benign lesions , such as plantar hemorrhage ( e.g. , black heel ) , dye - related pigmentation and , as in the present case , drug - induced hyperpigmentation .
we report the case of a 37-year - old woman ( phototype ii ) who presented at our outpatient clinic with a two - month history of hyperpigmented plantar macules . medical history revealed that the patient had taken capecitabine in the past three months as adjuvant chemotherapy for recurrent breast cancer . dermoscopic examination of the plantar macules showed parallel ridge pattern with pigmentation in the furrows without obliteration of eccrine gland apertures . besides in acral melanoma , parallel ridge pattern can also be observed in benign plantar lesions , such as congenital or acquired acral nevi , subcorneal hemorrhage , dye - related pigmentation and drug - induced hyperpigmentation , especially in patients with phototypes iii vi . the few reported cases of capecitabine - induced hyperpigmentation have been associated with hand and foot syndrome in patients with phototypes iv v and palmar as well as plantar involvement .
( sun tzu , the art of war , c.500 bc ) the challenge of conducting high - quality clinical studies in the critically ill population is widely recognised . heterogeneity in patient populations and clinical practice , diagnostic uncertainty , concerns with consent , and the overlapping nature of the presenting illnesses are amongst the inherent difficulties . influential studies in this population therefore increasingly involve multicentre , collaborative efforts using rigorously defined inclusion criteria and outcome measures such projects aim to obviate the aforementioned difficulties and to reduce the influence of individual intensive care units ( icus ) and case - mix variations on the findings . ultimately , the quality of the data justifies the logistical challenge involved . for this reason , the recent article from the newly - formed irish critical care trials group is a welcome announcement of a further such alliance . the data presented describe 1,029 patients admitted to icu services in a 10-week period in 2006 , covering a slight majority of all icu beds in ireland . the data include national specialist centres and university teaching hospitals , as well as regional units . the overall pattern depicts a busy service , with 78% of admissions being emergent in nature , a mean sequential organ failure assessment score of 5.4 , and 70% of patients needing mechanical ventilation . previous research in ireland showing an icu bed occupancy rate of 97% , an unscheduled discharge rate of 23% , and frequent cancellation of elective surgery is consistent with this pattern . the icu readmission rate of 7.5% is perhaps attributable to these service realities as indeed may be the failure to collect data in four of the 14 units that entered the study , including 23% of relevant patients . while accepting that the missing patient data compromise the validity of the findings , the outcomes nonetheless appear compatible with international standards and indeed are broadly indicative of the success of modern intensive care medicine . the outcomes in the subgroups are more striking : over 75% of readmitted patients survived and , of the 93 patients with five or six systems failing on admission , just over one - half survived . compatibility with international outcomes is perhaps most evident from those diagnostic categories where standard , consensus definitions are established . for severe sepsis , the icu mortality of 24% compares with a reported 35% in england , wales and northern ireland for severe sepsis in the first 24 hours . for acute lung injury / acute respiratory distress syndrome , the irish mortality was 32% and the irish critical care trials group has previously shown that this is consistent with modern international studies in the protective ventilation era . although the numbers are relatively small ( n = 289 ) , an icu mortality of 38% for renal failure compares unfavourably with the hospital mortality of 26.3% reported by hoste and colleagues using the same criteria . nonetheless , the study arguably further validates these rifle ( risk - injury - failure - loss - end stage ) descriptors as outcome predictors in acute renal dysfunction . seasonal and regional variations can not be detected as data from a short collection period are pooled together . the use of icu mortality alone as a measure of outcome is not ideal , and more meaningful outcome assessment tools including hospital mortality should be utilised in future projects ; for example , patients who were refused readmission and who might have gone on to die in hospital wards will appear as survivors in such a crude analysis . future scientific publications should also avoid the irritation of new data introduction in the discussion of the findings . the value of the present data will be more fully realised when the irish critical care trials group produces further , hypothesis - testing studies . having taken advice from the anzics critical care trials group , the irish critical care trials group set out to achieve this epidemiological study to provide baseline information for research planning . the data provide insight into disease prevalence ( for example , of acute respiratory distress syndrome ) , and enable planning for the study duration and resource allocation once power analysis has indicated the size of the study population required . the demonstration of the willingness of team members to cooperate and of the capacity of information systems to gather and collate such information is a further key to such studies and collaborations . the authors identify an urgent requirement for audit resources to maintain the ambition shown by this study . participation in the uk 's intensive care national audit and research centre would be one option , opening up the possibility of a uk / irish database . the ability demonstrated by the irish critical care trials group study to establish a research ethos that straddles the relatively contentious border linking the irish republic with the united kingdom is scientifically encouraging . icu : intensive care unit ; rifle : risk - injury - failure - loss - end stage .
quality research , requiring large numbers of participants , in the intensive care unit ( icu ) population requires multicentre collaboration . although logistically challenging , this methodology reduces the influence of individual units and has greater validity and broader relevance to patients and practitioners . the nascent irish critical care trials group opens additional such opportunities . in the accompanying epidemiologic study , the group present data gathered over 10 weeks of 2006 describing 1,029 patients , from 10 irish icus representing over one - half of ireland 's critical care bed capacity . the data depict a busy service , with 78% of admissions being emergent and with a moderately high ( 7% ) readmission rate . while recognising that there were missing data , the outcomes in organ failure and sepsis where international definitions exist and the icu survival rate ( 83% ) were consistent with international standards . the achievement of this planned first epidemiological step lays the foundation for the conduct of prospective scientific studies . these studies might occur in ireland or in cooperation with other audit / scientific groups such as the uk 's intensive care national audit and research centre , the european critical care research network , or others . this brings us a small step closer to the prospect of global , high - volume studies in critical care .
ameloblastoma , a true neoplasm of enamel organ type tissue , is a slow growing but locally invasive benign epithelial tumor of jaws accounting for about 11% of odontogenic tumors . ameloblastoma occurs over a broad age range with average age range being fourth decade of life . radiographically , often presents as expansile lesion with a multilocular appearance and with thinning of the cortical plate . according to the 2005 histological classification of tumors of the world health organization , ameloblastoma histopathological variants include follicular , plexiform , granular , basal cell , acanthomatous and desmoplastic ameloblastoma . granular cell ameloblastoma ( gca ) is a less common lesion accounting for 35% of all histologic subtypes of ameloblastoma . this article attempts at highlighting this relatively less common tumor - gca involving the lower jaw . a 55-year - old male patient reported with a painless swelling on right side of the lower jaw . patient gave a history of swelling in mandibular right molar region 12 years back for which he got his molar extracted . he developed a swelling in the same region a year later and was treated for the local infection with incision and drainage . the swelling persisted for 6 years swelling and then gradually increased to the present size . extraorally , the swelling was ovoid in shape , firm in consistency and extended from right mandibular molar region to anterior region of the mandible . intraorally , the swelling extended from mandibular right third molar region to lower left first premolar area with obliteration of buccal and lingual vestibule [ figure 1 ] . panoramic view revealed a large multilocular radiolucency extending from lower right third molar region to lower left first premolar region [ figure 2 ] . an incisional biopsy was carried out which histopathologically revealed tumor cells arranged in form of follicles within a fibrous connective tissue stroma [ figure 3 ] . the follicles were lined by tall columnar ameloblasts like cells organized in palisaded pattern and with stellate reticulum like cells in the center . most of the follicles showed the presence of large granular cells forming the central mass of the tumor follicles [ figure 4 ] . the lesion was surgically excised , and the gross specimen was sent for histopathological examination . gross specimen revealed section of the mandible extending from lower right third molar region to lower left second premolar with buccal and lingual cortical plate expansion [ figure 5 ] . on further histopathological examination , clinical image shows intraoral swelling orthopantomogram showing multilocular swelling histopathological image shows follicles with central granular cells ( h and e , 10 ) histopathological image shows eosinophilic granular cells in the center of follicle and peripheral ameloblast like cells ( h and e ) central lesions develop more frequently in molar ramus region in mandible , in molar region in maxilla followed by maxillary sinus and floor of the nose . gca is a relatively rare lesion and in most instances , it is found as an admixture with other histologic patterns , particularly follicular subtype . there is a marked transformation of cytoplasm of stellate reticulum like cells so that they appear coarse granular and eosinophilic . , a layer of stellate reticulum may be seen at the periphery separating the granular cells from tall columnar cells . ultrastructural studies also suggest that these are lysosomal aggregates and are responsible for granularity of cells . lysosomal aggregation within the cytoplasm may be due to dysfunction of either lysosomal enzyme or lysosome associated protein involved in enzyme activation , enzyme targeting or lysosomal biogenesis . leading to accumulation of substrate that would normally be degraded in endosome lysosome system . it was also considered that lysosomes might have resulted from some genetic alterations of granular cells . it has been suggested that numerous lysosomes accumulate as a result of decrease in the ability of lysosomes to dispose the unwanted components that accumulates in the cell with age . an increase in fibronectin expression has been observed in granular cells indicating granular cells changes could be associated with an age - related transformation . authors suggest that the granularity in gca might be caused by increased apoptosis and associated phagocytosis by neighboring neoplastic cells . balaji et al . have reported occurrence of numerous apoptotic cell fragments with condensed nuclei in granular cell clusters using annexin v , a marker to detect early apoptosis . studies suggest a decreased expression of several apoptosis - related factors such as bcl-2 family proteins and p53 protein in ameloblastoma . immunohistochemical studies have revealed that these granular cells and tall columnar cell lining the follicle are cytokeratin positive indicating epithelial in origin . it has been suggested immunohistochemically granular cells are positive for cd68 , lysoyme and 1 antichymotrypsin but negative for vimentin , desmin , s-100 neuron - specific enolase ( nse ) and cd15 indicating cytoplasmic lysosomal aggregates are not of mesenchymal myogenic or neurogenic origin . some studies have suggested the expression of laminin-5 in granular cells and a weak expression in peripheral cells . the differential diagnosis of gca includes other oral lesions with a similar morphology of granular cell accumulation , including granular cell odontogenic tumor , granular cell tumor , and congenital epulis . granular cell odontogenic tumor is a rare odontogenic neoplasm with a tendency to occur in the mandibular posterior region and is composed of granular cells and nests or islands of the odontogenic epithelium . the granular cells are positive for vimentin , cd68 , lysozyme , muscle - specific actin , smooth muscle actin , calponin , neuron specific enolase , cd138 , and bcl-2 . granular cell tumor is relatively rare benign neoplasm that occurs in any part of the body including orofacial region . immunohistochemical studies of granular cell tumors suggest a neural or neuroectodermal origin of the granular cells . histologically congenital epulis does show the presence of large cells with eosinophilic granular cytoplasm within fibrous connective tissue stroma , which stain positively for phosphotyrosine and are s-100 negative . oncocytomas though rare in the jaws , possibility of occurrence can not be ruled out . in such rare cases , differential previous studies suggest gca to be most aggressive lesion , whereas some studies suggest that the granular cell are just a transitional phase in life cycle of ameloblastoma , starting with normal stellate reticulum like cells , leading to production of granules and finally leading to degeneration and formation of cystic areas . however , reichart et al . have reported a 33.3% recurrence rate for gca . a regular follow - up is important because of reports of recurrences even up to 8 years after initial treatment . understanding the nature of granular cells not only in gca but also in similar lesions will aid in differentiating as well as understanding the clinical behavior of the lesion .
ameloblastoma is an epithelial odontogenic tumor exhibiting diverse microscopic pattern that occurs singly or in combination with other patterns . this article describes a case of granular cell ameloblastoma ( gca ) involving mandible in a 55-year - old male . the possibility of granular component is there in other odontogenic and nonodontogenic lesions . sometimes dilemma exists in the diagnosis of such lesions . the purpose of this article is to unveil the hidden characteristics in gca , which might help in differential diagnosis of gca .
lupus erythematosus ( le ) panniculitis is a quite rare clinical entity characterized by one or several firm , asymptomatic , often fairly large subcutaneous nodules , as a manifestation of systemic le ( sle ) or discoid le ( dle ) . the inflammatory reaction in le profundus ( lep ) takes place primarily in the deep corium and the subcutaneous tissues leading to deep indurated nodules or sharply defined plaques . the overlying skin usually appears normal , but there may be erythema , atrophy , ulceration or poikilodermatous or hyperkeratotic changes . the lesions are most frequent on cheeks , but other sites of predilection are face , upper arms , hands , chest , buttocks , and thighs . a 24-year - old primigravida of 6 month gestation presented asymptomatic , multiple ulcerated lesions situated bilaterally over cheeks just in front ears . the lesions first developed as erythematous , painless nodules , oval - shaped , and firm in consistency ; gradually increased in size to reach the present size and ulcerate . examination revealed multiple erythematous , indurated plaques of about 2 cm 3 cm over both the cheeks with ulceration at base [ figure 1 ] . ( a and b ) multiple erythematous ulcerated lesions situated bilaterally over the cheeks besides routine investigations , special investigations , except anemia , like ana , anti - dsdna , anti - ro / ssa , and anti - la / ssb antibodies were within normal limits . the fat lobules showed patchy infiltrate with lymphocyte and plasma cells , and the adipocytes showed signs of coagulative fat necrosis with microcyst formation . the overlying dermis showed sparse superficial and deep perivascular lymphocytic infiltrate with mucin deposits [ figures 2 and 3 ] . the patient was started oral prednisolone 20 mg daily , which led to significant improvement . this was given for 2 weeks and then gradually tapered over a period of 2 months . after 2 months , lesions healed with atrophy [ figure 4 ] and did not show the occurrence of any new lesions till delivery . newborn was healthy , and blood was negative for ana , anti - dsdna , anti - ro / ssa , and anti - la / ssb antibodies . moderately dense lymphoplasmacytic lobular panniculitis with septal thickening ( h and e , 10 ) fat lobules showing patchy infiltration and signs of coagulative fat necrosis with microcyst formation ( h and e , 10 ) ( a and b ) lesions healed with atrophy after treatment lep is unusual but distinct clinical variety of le , commonly present in the third to sixth decades of life with a female : male ratio of 2:1 . the typical lesions are multiple indurated nodules or plaques involving the arms , face , buttocks , legs , chest , and less frequently the abdomen , back and neck , and breast . the histopathology is sufficiently characteristic to establish the diagnosis in the absence of other cutaneous or systemic lesions of le . . however , it is frequently accompanied by septal involvement ; and therefore , termed as mixed panniculitis . lupus panniculitis is often difficult to diagnose as other form of panniculitis may present similarly . the differential diagnoses of lep include panniculitis due to other connective tissue disorders like dermatomyositis or scleredema and weber - christian panniculitis or jessner 's lymphocytic infiltration , lyrnphocytoma cults , and sarcoidosis . lep may develop in association with dle or sle or may occur as an isolated phenomenon . aggarwal et al . reported a case of lep with associated mastitis but without any lesions of dle or sle . we found only three cases of lep in pregnancy even after extensive medline - based literature search . reported a case of fibrosing le panniculitis in a pregnant woman with anti - ro / ssa antibodies in mother and child . systemic steroids are generally not recommended for lupus panniculitis unless it is indicated for other manifestations of sle . malarials are contraindicated in pregnancy , our patient was treated with steroids and responded well . we reported an isolated case of lep with pregnancy which responded well to oral steroids . we reported an isolated case of lep with pregnancy which responded well to oral steroids . we reported an isolated case of lep with pregnancy which responded well to oral steroids .
a case of lupus erythematosus ( le ) panniculitis in pregnancy without any lesions of discoid le or systemic le is being reported . there were no systemic symptoms . her ana , anti - dsdna , anti - ro / ssa , and anti - la / ssb antibodies were within normal limits . diagnosis of lupus panniculitis was considered on clinical and histopathological grounds . the condition responded favorably to systemic steroid therapy .
stress induced hyperglycemia is a known complication of neonatal sepsis , but sometimes it may become very difficult to distinguish it from neonatal diabetes mellitus . we present a case of neonatal septicemia with stress induced hyperglycemia mimicking neonatal diabetes mellitus . extremely high blood sugar values with metabolic acidosis were noted in a term good weight baby caused a diagnostic dilemma . the present case report is about a 3 kg term neonate who presented on the 9 day of life with a history of fever for 2 days , lethargy and one episode of seizure . at admission , he was in a state of shock with severe dehydration . he was given two boluses of normal saline , radiant warmer care and intravenous antibiotics ( cefotaxime and vancomycin ) were started . the blood sugar was 1529 mg / dl and arterial blood gases revealed mild metabolic acidosis ( ph - 7.289 and hco3 - 11.5 ) . dehydration was corrected and thereafter considering the high blood sugar , the child was started on intravenous insulin infusion promptly at a dose of 0.1 iu / kg / h . on evaluation , there was thrombocytopenia ( platelet count-25,000 ) and serum procalcitonin was 3.10 ng / ml and c - reactive protein was 10 , which was high and suggestive of septicemia . lumbar puncture revealed a turbid cerebrospinal fluid ( csf ) , which was full of polymorphonuclear cells , csf protein was 80 mg / dl and sugar was 67 mg / dl . hence , a diagnosis of late onset neonatal septicemia with shock with stress induced hyperglycemia was considered . considering neonatal diabetes as another possibility due to very high blood sugars , child was evaluated further and it was found that serum insulin and glucagon stimulated c peptide levels were respectively 4.00 mcu / ml and 2.57 ng / ml , which were normal . child improved as acidosis and hyperglycemia gradually resolved , there were no more seizures and urine output was adequate . insulin infusion was slowly tapered as blood sugar normalized and it was stopped after 48 h. child maintained normoglycemia hereafter and did not require insulin infusion any more . magnetic resonance imaging brain was done at 45 days of life which revealed cerebral venous thrombosis of the transverse sinus with hemorrhagic infarct in left temporal region [ figure 1 ] . the child has been under follow - up for 6 months now with normoglycemia and no neurological sequelae . ( a ) axial t2 fluid attenuation inversion recovery image showing hemorrhagic infarct in left temporal region . ( b ) axial contrast - enhanced t1 image showing thrombus in the left transverse sinus hyperglycemia , defined as fasting blood glucose > 125 mg / dl is mostly seen in very low birth , small for gestation age infants and preterm babies receiving intravenous glucose infusion . this is attributable to altered metabolism associated with immaturity , as well as the need for continuous parenteral nutrition . in these children , it may be triggered by respiratory distress , surgery , neonatal pain , sepsis and other stressful events . hyperglycemia in preterm infants has been associated with increased rates of death , intraventricular hemorrhage ( ivh ) , sepsis and retinopathy of prematurity and with increased lengths of hospital stay . the problem of hyperglycemia is manifested due to an increase in the osmolality ( 1 mosm / l rise is noted with every 18 mg / dl increase in blood sugar ) ; particularly beyond 300 mosm / l . this is in the form of electrolyte disturbances , osmotic diuresis and in extreme form with ivh . the study by louik et al . had discussed that the fluctuations in cerebral blood flow velocity markedly increase the risk of ivh and such fluctuations may be partially caused by rapid change in osmolality . whether hyperglycemia causes ivh or vice versa is still controversial , but a plasma glucose level of more than 300 mg / dl is usually associated with a high risk of developing ivh . however , there is a paucity of data in indian population where more than 30% babies are low birth weight . in a study carried out on 1171 newborns admitted to nicu by chellani et al . , the mean blood glucose level was 271.8 75.33 mg / dl ( range : 175 - 400 mg / dl ) with a mean duration of hyperglycemia 60.6 15.44 h ( range : 48 - 96 h ) . in our case values as high as 1529 mg / dl were noted , which is uncommonly seen in term babies who are non - diabetic . stress factor responsible for causation of hyperglycemia includes sepsis due to escherichia coli and group b streptococci . it can also be associated with hyperglycemia by catecholamine , cortisol influences on the mobilization of glycogen , gluconeogenesis and insulin response . on the other hand , endotoxins and cytokines methyl - xanthine which is most often used in preterm babies causes an increase in camp level resulting in activated liver glycogenolsis . as per the cochrane database , it is possible that it is the sicker neonate , who is at higher risk for adverse clinical outcomes , who is more prone to hyperglycemia because of stress or metabolic injury to vital organs , especially the brain . it is also possible that it is hyperglycemia per se that is a cause of adverse clinical outcomes , especially if hyperglycemia is severe enough to cause significant plasma hyperosmolality resulting in fluid shifts from the intracellular to the extracellular fluid compartment . there is also some evidence of an adverse effect of severe hyperglycemia on neurologic outcome following cerebral ischemia in animals and adult humans as well as the neonate . thus , in the very low birth weight / extremely low birth weight neonate with hyperglycemia , it is important to determine the benefits and risks of treating the hyperglycemia by either reducing the rate of glucose infusion or by administering exogenous insulin . based on the above report it can be concluded that stress due to sepsis induced transient hyperglycemia in the newborn may present with extremely high blood sugar values and may mimic neonatal diabetes mellitus . it can be seen even in term babies , contrary to the belief that it occurs in preterm and small for gestation babies . considering the prognostic implications it may cause it is important that hyperglycemia is promptly treated by insulin infusion .
stress / sepsis induced transient hyperglycemia in the newborn may present with extremely high blood sugar values and may mimic neonatal diabetes mellitus . we present a case of neonatal septicemia with stress induced hyperglycemia mimicking neonatal diabetes mellitus . extremely high blood sugar values upto 1529 mg / dl with metabolic acidosis were noted in a term good weight baby causing a diagnostic dilemma . it can be seen even in term babies , contrary to the belief that it occurs in preterm and small for gestation babies . considering the prognostic implications it may cause it is important that hyperglycemia is promptly treated by insulin infusion .
staphylococcus aureus is a well - adapted human / zoonotic colonizer which can also cause a wide range of diseases and its treatment is becoming more difficult because of increasing rates of drug resistance both in hospital and community settings . community - acquired methicillin - resistant s. aureus ( ca - mrsa ) has been a subject of interest in the last decade . traditionally , the risk factors for ca - mrsa are chronic illness , history of recurrent use of antibiotics and intravenous drug abuse . moreover , new risk factors such as attendance in military service and day care centers , homosexuality , tattooing , contact sports , sharing soaps and towels , having pets and age 2 years have been proposed , yet several patients with ca - mrsa infections do not have any recognized risk factor for methicillin resistance ( 1 - 6 ) . in addition to epidemiological differences , ca - mrsa and hospital - acquired methicillin - resistant s. aureus ( ha - mrsa ) differ in antibiotic resistance pattern . ca - mrsa clones are usually resistant only to -lactams and erythromycin and are usually susceptible to co - trimoxazole , clindamycin and fluoroquinolones ( 1 , 2 , 5 , 7 ) . from a molecular perspective , ha - mrsa strains usually carry staphylococcal cassette chromosome mec ( sccmec ) types i - iii while ca - mrsa strains carry sccmec types iv or v ( 1 , 4 , 5 , 8) . the aim of this study was to determine the frequency of invasive ca - mrsa in children admitted to the pediatric wards of imam reza and ghaem teaching hospitals . in this retrospective observational study , data of s. aureus - positive cultures from sterile body sites ( i.e. blood , bone puncture , joint fluid and lymph node aspiration ) was gathered from microbiology laboratory files from march 20 2006 to march 19 2012 . the community or hospital origin of s. aureus infection was determined by hospital files and telephone communication with the patients parents according to the cdc criteria ( 1 , 6 ) . during this 6-year period , 23 invasive community - acquired s. aureus cultures were recovered from pediatric patients ( 8 girls and 15 boys ) . isolates were from blood ( n = 15 ) , synovial fluid ( n = 2 ) , bone puncture ( n = 3 ) and lymph node aspiration ( n = 3 ) . seventeen isolates ( 74% ) were resistant to methicillin . septic arthritis , osteomyelitis and sepsis ( table 1 ) the resistance rate for erythromycin , cotrimoxazole , ciprofloxacin , tetracycline , and genta - micin was 71% , 53% , 36% , 50% and 44% for ca - mrsa and 0% , 0% , 20% , 50% , 33% for community - acquired methicillin susceptible s. aureus , respectively . invasive staphylococcal infections like osteomyelitis , septic arthritis and pneumonia are common causes of admission to pediatric wards . however , based on our daily clinical observations , there is a high incidence of ca - mrsa in our institutions . this research confirms that the rate of methicillin resistance among invasive community - acquired isolates has been very high ( 74% ) in pediatric patients during the past six years . there is a high difference in the prevalence of ca - mrsa in different geographic areas . for example in the us , ca - mrsa prevalence varies between 15% in north eastern states up to 72% in southern states ( 3 ) . in middle east countries , the prevalence of ca - mrsa is 62% in saudi arabia ( 9 ) and 52% in turkey ( 10 ) , 11% in south of india ( 11 ) , and 74% in taiwan , ( 12 ) which is close to our estimation in this study . ca - mrsa was more common in boys in our study ( male / female ratio = 2 ) . also in a study of the nicu of texas children hospital , most subjects ( 65 of 89 ) were male ( 14 ) . sex difference in our study can be explained by considering the fact that bone and joint infections are more common in boys ( 15 ) . resistance of ca - mrsa to non--lactam antibiotics like co - trimoxazole and ciprofloxacin has been reported 0% and 10.7% in the us and 11% and 10% in saudi arabia , respectively ( 8 , 9 ) . in this study , high rate of resistance to co - trimoxazole and ciprofloxacin was observed among our invasive ca - mrsa isolates . interestingly , multidrug resistant ca - mrsa has been recently reported from other asian countries ( 16 ) . the rate of ca - mrsa in our community is very high and similar to asian ca - mrsa clones which are also resistant to co - trimoxazole and ciprofloxacin . although further epidemiological and molecular investigations are needed to confirm the results of this study , we recommend that in these two hospitals , for invasive community - acquired s. aureus infections treatment should be started with drugs like clindamycin , vancomycin , linezolid or daptomycin .
background and objectivecommunity - acquired methicillin - resistant staphylococcus aureus ( ca - mrsa ) is increasingly reported worldwide . we aimed to determine the frequency of invasive ca - mrsa in children admitted to the pediatric wards of imam reza and ghaem hospitals of mashhad , iran.materials and methodsin this retrospective study , data regarding s. aureus isolates from pediatric patients sterile body sites ( i.e. blood joint , bone and lymph node aspiration ) were retrieved in a time period from march 2006 to march 2012 . disc diffusion data was analyzed to determine the resistance pattern of the isolates , and differentiation between community - acquired and nosocomial s. aureus was done according to cdc guidelines.resultstwenty three invasive community - acquired s. aureus isolates from sterile body sites were identified , of which seventeen ( 74% ) were ca - mrsa . the ca - mrsa isolates showed high frequency of resistance to non--lactam antibiotics ( 71% to erythromycin 53% to co - trimoxazole , 44% to gentamicin and 36% to ciprofloxacin).conclusionin this study , the majority of invasive community - acquired s. aureus isolates were found to be ca - mrsa . therefore , we recommend that primary treatment should be with antibiotics such as clindamycin , vancomycin , linezolid or daptomycin for any invasive infection suspected to be caused by s. aureus in these two hospitals .
spinal cord teratomas are uncommon . excluding sacrococcygeal teratoma in neonates , teratomas in the spinal canal are rare . only a few central nervous system(cns ) delayed presentation of spinal teratomas has generally been associated with its slow - growing characteristics5 ) . dermoid tumors may rupture and fatty material may escape into the subarachnoid space , and/or ventricles , resulting in variable neurological symptoms6 ) . the symptoms are dependent on location and are due to the irritative effect on and/or compression of the adjacent structures3 ) aseptic meningitis may also occur15 ) . here , we report a rare case of a mature intradural spinal teratoma in an adult with concomitant additional intracranial lipid droplet dissemination associated with neurologic deterioration after iatrogenic rupture of the cyst portion occurs . iatrogenic rupture of cystic portion during spinal teratoma surgery may worsen intracranial lesion that previously exist . a 67-year - old man was transferred to our department from the urology department of our hospital . he complained of nocturia and frequent urination . during evaluation , an approximately 3 cm mass in the spinal canal abdominal ct imaging showed a fat - containing mass with mural calcification at the l2 level ( fig . the patient looked ill and had experienced aplastic anemia 2 years previously . on neurologic examination , an approximately grade 4 weakness was noted in both lower extremities . lumbar magnetic resonance ( mr ) imaging showed a hyperintense intradural mass on a t1-weighted image . after gadolinium enhancement , there was no significant enhancement of the mass in the conus medullaris and focal enhancement of the distal soft tissue component ( fig . after surgery , his lower - extremity weakness did not improve , and he could not walk without assistance . preoperative ct imaging showed low density lesion in both lateral ventricle and cistern . at that time postoperative brain mr imaging showed hyperintense lesion on a t1-weighted image on both the lateral ventricle and as well as slightly enlarged ventricles ( fig . we performed extra ventricular drainage ( evd ) , and his mental status recovered temporarily . however , his mental status again declined ; evd was performed on the other side after 7 days but , we could not retrieve tissue from the mass . during treatment , spinal cord teratomas are uncommon . only a few cns teratomas arise from the spinal cord . the prognosis of teratoids is poor and metastases at diagnosis are common , especially in neonates and children4 ) . teratomas are slow growing , but , often grow outside the spinal column and extend to the paraspinal and retroperitoneal spaces . mature teratomas are composed of well - differentiated elements , and immature teratomas are primitive elements derived from any or all of the three germ cell layers2 ) . mr imaging is considered as the gold standard for the detection of teratomas . total resection is desirable ; however , it is almost impossible because of the intimal adhesion between the teratomatous cyst and the circumferential nervous parenchyma . radical surgery is not recommended as the pathology is benign and recurrence rates are low even after subtotal resections1,11 ) . the surgical goal is subtotal resection for decompressing the cord and resecting the exophytic component5 ) , and excised tissues should be taken as extensively as possible to establish an accurate diagnosis7 ) . after subtotal resection , consecutive and long - term radiological follow - up is recommended16 ) . a case of spinal mature teratoma accompanying the intracranial dissemination of fatty droplets has been reported3,6,13,15 ) . dissemination of lipid droplets within ventricles occurs because of spontaneous , iatrogenic , or traumatic rupture . our case is similar to this previous case , although , an intracranial mass lesion was not confirmed histopathologically . the symptoms of intracranial lipid droplet dissemination were headache , chemical meningitis , seizure and mental deterioration . the cystic contents gain access to the ventricular system by way of retrograde flow through the foramen of luschka and magendie15 ) . it is possible that the central canal can serve as a pathway for ruptured material into the brain13 ) . ct and mr imaging are useful in diagnosing free fat in the subarachnoid space and intraventricular fat - fluid level . lipid is differentiated from cerebrospinal fluid by negative values ( -20 to -130 hounsfield units)15 ) . mr imaging is reliable in the evaluation of the presence of rupture and its extent in the subarachnoid spaces or into the central spinal canal10 ) . we report a rare case of a mature intradural spinal teratoma and concomitant intracranial lipid droplet dissemination in an adult . additional lipid droplet dissemination to intracranial space with neurologic deterioration after a spinal teratoma surgery should be considered when iatrogenic rupture of the cyst portion occurs . it might develop the symptom and worsen hydrocephalus , although asymptomatic lipid droplets dissemination was previously existed before lumbar spine surgery .
a teratoma is a neoplasm that contains tissues originating from three germ cell layers at ectopic sites . the embryology of teratomas remains unclear . teratomas are usually composed of cystic and solid components , and they are usually associated with syringomyelia . cystic lesions of teratomas may rupture in a spontaneous , iatrogenic , or traumatic manner . lipid droplets in the ventricles and subarachnoid space are rare . we managed a case of a spinal teratoma in the lumbar region in a 67-year - old man . he complained of nocturia , frequent urination , and difficulty in walking for 2 months . radiographic imaging revealed a lumbar spinal intradural mass . intracranial lipid droplets dissemination was also existed . the patient underwent surgery , and a diagnosis of mature teratoma was confirmed histopathologically . during the operation , the cystic portion of the intradural mass ruptured . during the hospital stay , the patient 's mental status declined . on radiological examination , slightly enlarged ventricle size was observed . dissemination of lipid droplets within ventricles occurs because of spontaneous , iatrogenic , or traumatic rupture . additional lipid droplet dissemination to the intracranial space associated with neurologic deterioration after a spinal teratoma surgery should be considered when iatrogenic rupture of the cyst portion occurs .
stress - responsive signaling pathways often mediate adaptive changes that favor microbial survival and propagation of the infection . therefore , many investigators have attempted to define microbial signaling pathways activated in response to the host . one of these pathways , the cyclic amp ( camp)/protein kinase a ( pka ) pathway , is closely associated with pathogenesis in many fungal species . although the central pka signaling elements are highly conserved in divergent fungi , the downstream effects of pka activation are often species specific . for example , candida albicans requires intact pka signaling to undergo the yeast - hyphal transition , a morphological change associated with its pathogenesis ( 1 ) . similarly , many endemic fungal pathogens use this pathway to initiate a temperature - regulated transition from an environmental mold to a pathogenic , yeast - like form ( 2 ) . in contrast , the opportunistic fungal pathogen cryptococcus neoformans employs pka to regulate the induction of phenotypes required for full virulence , such as polysaccharide capsule formation and laccase / melanin expression ( 3 ) . in each case , diverse fungal species appear to have coopted this conserved signaling pathway to regulate specific phenotypes required for their survival within the host . however , we have lacked a comprehensive understanding of the downstream targets of the pka pathway in pathogenic fungi . in a recent article in mbio , geddes et al . present an important series of investigations identifying pka - responsive proteins in c. neoformans ( 4 ) . using quantitative proteomics approaches , that study offers us significant insight into pka - dependent processes regulated by the fungal cell in response to various stresses , including many host - relevant signals . far from being a simple story , the downstream targets of pka in c. neoformans reported in these studies are components of multiple , varied cellular processes . the main highlighted result was the importance of pka in the ubiquitin - proteasome pathway and other processes related to the control of protein abundance . the authors specifically linked proteasome activation to expression of the main virulence determinant in this microorganism : polysaccharide capsule expression . additionally , the authors used various chemical inhibitors to explore translational prospects for their findings . interestingly , these observations in a unicellular , eukaryotic pathogen are consistent with recent reports of human neurodegenerative diseases in which pka signaling is similarly linked to proteasome activation . defects in human pka signaling result in altered protein turnover , contributing to increases in ubiquitin - protein aggregates associated with conditions such as amyotrophic lateral sclerosis and alzheimer s disease ( 5 , 6 ) . the effects of similar dysregulation of pka signaling in eukaryotic microorganisms have yet to be deeply explored , especially since most microbial studies to date have considered primarily the effects of pka in single cells growing in planktonic cultures . however , similarities in pka signaling between simple and multicellular eukaryotes might be better appreciated by studying microorganisms living in complex communities , such as biofilms . there is often inherent complexity in defining outputs from such a centrally acting signaling pathway . for example , in this paper , the authors note instances of similar cellular changes resulting from either activation or repression of pka signaling in c. neoformans . furthermore , the investigators noted the paradoxical observation that there was not always a direct correlation between transcript abundance and protein levels for individual , pka - responsive gene products . additionally , this type of proteomic analysis can be biased toward identifying the most abundant proteins in the cell , perhaps missing important changes in transient or low - abundancy proteins . however , the study by geddes et al . uses sensitive techniques to define microbial processes that are controlled by pka and , therefore , likely respond to host - derived stresses . their detailed proteomics analysis will be a considerable resource for investigators exploring pka signaling in varied eukaryotic pathogens and their infected hosts . as we better appreciate these adaptive responses of microorganisms attempting to survive within the host , we will gain new insight into novel diagnostic and therapeutic strategies to address challenging infectious diseases .
abstractthe protein kinase a ( pka ) signal transduction pathway has been associated with pathogenesis in many fungal species . geddes and colleagues [ mbio 7(1):e01862 - 15 , 2016 , doi:10.1128/mbio.01862 - 15 ] used quantitative proteomics approaches to define proteins with altered abundance during protein kinase a ( pka ) activation and repression in the opportunistic human fungal pathogen cryptococcus neoformans . they observed an association between microbial pka signaling and ubiquitin - proteasome regulation of protein homeostasis . additionally , they correlated these processes with expression of polysaccharide capsule on the fungal cell surface , the main virulence - associated phenotype in this organism . not only are their findings important for microbial pathogenesis , but they also support similar associations between human pka signaling and ubiquitinated protein accumulation in neurodegenerative diseases .
our patient presented with accelerated silicosis starting as gradually progressive shortness of breath for about 1 year , progressing to chronic type 1 respiratory failure . this was followed by development of full blown mctd , with combined features of rheumatoid arthritis , scleroderma and systemic lupus erythematosus with severe arthritis , myositis and joint contractures . this was a case report of a 32-year - old stone crusher presented with gradually progressive breathlessness , initially on exertion and later , also at rest for 1 year . at the same time , he also noticed raynaud 's phenomenon involving the fingers and toes . over the next 6 months , he developed a gradual binding down of the skin over the dorsa of hands , forearms , face and leg . the patient also had severe joint pains predominantly involving all joints of the hands , including the wrist joint and the knees bilaterally . patient had been working as a stone crusher for the past 6 years . on examination , the patient had diffuse hyperpigmentation , visible respiratory distress with a respiratory rate of 34/min with mild pallor . arterial blood gas analysis revealed type 1 respiratory failure with pao2- 54 mmhg , ph - 7.38 , paco2- 38 mmhg . testing for immune markers revealed antinuclear antibody ( ana ) positive-9 ( if assay ) , anti - double stranded - deoxyribonucleic acid positive , anti - scl70 igg antibody positive 86 u / l , anti - u1 ribonucleoprotein ( rnp ) antibodies positive in high titers , anti - cyclic citrullinated peptide and rheumatoid factor positive . contrast enhanced computed tomography of the chest was suggestive of diffuse intra and interlobular septal thickening with fibrosis and randomly situated nodules [ figure 1 ] . transbronchial lung biopsy was compatible with silicosis and revealed focal interstitial fibrosis interspersed with pigment laden macrophages and refractile material . low dose prednisonolone ( 20 mg od ) was given in view of the arthritis . methotrexate at a dose of 25 mg weekly was also started along with proton pump inhibitors and physiotherapy . his hypoxia has also improved on therapy , but he continues to be on supplemental oxygen . some patients have features of more than one rheumatic disease and thus do not fit into traditional classification . patients with combination of clinical finding similar to those of systemic lupus erythematosus , progressive systemic sclerosis , polymyositis , rheumatoid arthritis and with unusually high titers of circulating ana with specificity for nuclear rnp are considered to have mctd . hypotheses implicating modified self - antigens and/or infectious agents in the pathogenesis of mctd have been advanced , but none have been proven yet . crystalline silica ( quartz ) particles less than 1 are the most pathogenic in silicosis . this constitutes a permanent stimulus for the fibroblasts , which increase their production of collagen leading to cutaneous sclerosis , vascular occlusion and pulmonary fibrosis , which explains the myriad clinical features of scleroderma and mctd .
silica exposure has been implicated with the development of various connective tissue diseases . we report a case of 32-year - old stone crusher who developed silicosis with mixed connective tissue disorder ( mctd ) 6 years after exposure to silica . this association of silicosis with mctd has never been reported from the indian subcontinent , although the problem of this pneumoconiosis remains rampant . this rare association urges us to report this case .
blunt traumatic diaphragmatic rupture ( btdr ) is a life - threatening condition with an incidence of 0.8%1.6% in blunt trauma [ 13 ] . the diagnosis often happens to be late due to the absence of typical symptoms or other major injuries dominating the clinical aspect . an isolated btdr is rare and thus might be followed by a period of weeks or months not revealing any symptoms [ 2 , 5 ] . most btdr are located on the left side in the musculotendinous intersection [ 1 , 3 , 4 ] . herniation of colon , small bowel , or liver may occur and result in ileus , necrosis , and perforation [ 4 , 7 ] . we present a rare case of a 68-year - old female hospitalized in the neurological department due to parkinson disease . the examination showed a slightly reduced breath without any signs of pneumothorax or dyspnea , a decent pressure pain and a bruise . the chest radiograph displayed a herniation of bowel into the right hemithorax with consecutive ileus signs ( figure 1 ) . we performed a laparoscopic approach and found a 4 5 cm rupture of the right diaphragm with herniation of 1 meter small bowel . the transdiaphragmatic thoracoscopy displayed a collapsed lung and a dislocated rib fracture ( figure 2 ) . after irrigation of the thoracic cavity we made a direct laparoscopic strainless running suture with nonabsorbable tie ( 0/0 ethibond ) . the delay of the diagnosis was 4 days indicating the variety of the clinical appearance of the btdr . the missing typical symptoms can mask the severity of the injury and lead to increasing morbidity . although large prospective long - term studies regarding outcome after laparoscopic approach are still missing , laparoscopy was the method of choice in our case and safely performed , particularly when severe side injuries are absent . we could show , that laparoscopy has advantages in isolated diaphragmatic ruptures compared to traditional laparotomy , which is still preferred by some authors [ 8 , 9 ] . laparoscopy mostly allows easy reposition of herniated organs , sufficient inspection of the thoracic , and abdominal cavity and immediate laparotomy if necessary .
blunt traumatic diaphragmatic rupture ( btdr ) is a life - threatening condition with an incidence from 0,8%1,6% in blunt trauma , mostly located on the left side . the main prognostic factors are severe side injuries and the delay of diagnosis . we present a rare case of a 68-year - old female , with an isolated right diaphragm rupture . the diagnosis was done with a delay of 4 days by thoracic radiographs , which showed a herniation of small bowel into the right thoracic cavity . a reposition of the small bowel and a closure of the diaphragmatic defect by running suture were carried out laparoscopicly . although large prospective studies concerning the outcome of laparoscopic approach to right btdr are still missing , we could show , that laparoscopy can be performed safely in right traumatic diaphragm rupture .
this is the 13th annual database issue of nucleic acids research , and the first one that goes entirely paperless . the list of molecular biology databases keeps getting bigger , and despite negative connotations sometimes connected to this growth , the databases are getting better and even more diverse . the current release of the nucleic acids research online molecular biology database collection ( supplementary table 1 ) includes 92 new databases , first described in this issue , and 49 additional new databases , featured in bioinformatics , bmc bioinformatics and other journals . these include first ever databases from ireland , portugal and united arab emirates ( 13 ) and a variety of other databases maintained all over the world . meanwhile , existing databases show remarkable resilience : out of 719 databases featured in the last year 's release ( 4 ) , only 2 were no longer maintained because their authors graduated , retired or changed focus , and one more has shifted to restricted access . in contrast , three databases , abcdb , eid and kdbi , that were considered dead last year and had been crossed off the list , have now been resurrected . in each case , their authors have moved to new work places and were able to resume maintenance of their databases . as promised last year , their accession numbers have not been re - used and these databases are now listed under the same entry numbers , 157 , 32 and 138 , respectively , that they had in previous releases . these numbers can be used to gain access to updated summaries of these databases on the nar website , e.g. . similarly , puma2 ( 5 ) , which replaced the wit2 database , has kept its number 118 in list . after 12 years of database issues and 8 years of the accompanying web supplement , it was interesting to check if they are really having an impact . in other words , how many people really care about them and use them ? to evaluate the impact of the nar database issues , i have used a tool that , despite all complaints and caveats , is commonly utilized for evaluating research productivity , namely the science citation index produced by the institute for scientific information . if databases are put on the web for the benefit of the research community , the frequency with which people use ( and cite ) a given database could serve as an indication of whether this database serves a useful purpose . an inspection of the citation figures for the 141 papers published 2 years ago in the 2004 nar database issue ( all citation data are as of october 15 , 2005 ) revealed a very encouraging trend . only five papers have not been cited at all and the same number of database descriptions five have been cited > 100 times , becoming , in isi parlance , instant citation classics. whatever the caveats , the fact that the paper describing the pfam domain database [ , nar collection entry no . ( 6 ) ] has been cited 375 times in < 2 years definitely indicates that this database is widely used by the research community . indeed , comparing a protein sequence against pfam it is probably no coincidence that the first author of the pfam paper also serves as the editor of the nar database issues . in the interest of full disclosure , the second best cited database , gene ontology ( go ) [ , nar collection entry no . ( 7 ) ] provides structured , controlled vocabularies and classifications that are also widely used in genome annotation , as well as for a variety of bioinformatics tasks . it is worth noting that each of these databases allows free downloading of its full content : they work by adding valuable expertise to the sequence data and have nothing to hide . the databases that form the international nucleotide sequence database collaboration , ncbi 's genbank , embl nucleotide database and japanese ddbj ( nar collection entries no . 13 ) , also attract a respectable number of citations , even though they are usually mentioned in the literature without a formal citation . the same is true for the protein data bank ( pdb ) ( nar collection entry no . more databases are probably headed the same way of becoming household names that are not considered to need a citation . on the other side of the spectrum are the databases that have never been cited in these 2 years , even by their own authors . this does not mean , of course , that these databases do not offer a useful content but one could always suggest a reason why nobody has used this or that database . usually these databases were too specific in scope and offered content that could be easily found elsewhere . ( 11 ) ] , maps single nucleotide polymorphisms onto known protein structures , allowing one to trace the location of the affected amino acid residues and correlate it with disease phenotypes . however , most of its data are extracted from omim ( , nar collection entry no . ( 12 ) ] is a database of complete genome sequences of plant and animal viruses . however , it often takes a while for the server to produce a response , which contains little information that would not be available in other databases , such as viperdb [ , nar collection entry no . ( 13 ) ] , viral bioinformatics resource center ( , nar collection entry no . 798 ) , vida ( , nar collection entry no . 602 ) . the ribosomal protein gene database ( rpg ) [ , nar collection entry no . ( 14 ) ] lists ribosomal proteins from just a handful of organisms , offering a tiny fraction of information that is available through pfam , uniprot , kegg orthology groups and a variety of other sources . the same problem plagues eyesite , a database of protein families in the eye [ , nar collection entry no . even the terrific graphics on its front page can not compensate for the fact that researchers interested in eye proteins can get their sequences from uniprot and other sequence databases and their structures from pdb . finally the signal transduction classification database ( stcdb ) [ , nar collection entry no . ( 16 ) ] offers an interesting approach to the hierarchical classification of eukaryotic signaling proteins . however , so many people use the go classification that it has become de facto standard and nobody is looking for alternative classification schemes . thus , the fact that this comment will most probably be the first time in 2 years that toposnp , virgen , rpg , eyesite or stcdb are mentioned in the literature could be a direct consequence of the overwhelming success of other databases . it is an open global marketplace of ideas , tools and approaches ; fortunately , nobody goes out of business . suggestions for the inclusion of additional databases in this collection should be directed to the author at galperin@ncbi.nlm.nih.gov .
the nar molecular biology database collection is a public online resource that contains links to all databases described in this issue of nucleic acids research . in addition , this collection lists databases that have been featured in previous issues of nar , as well as selected other databases that are freely available to the public and may be useful to the molecular biologist . the 2006 update includes 858 databases , 139 more than the previous one . the databases come with brief summaries , many of which have been updated recently . each database is assigned a stable accession number that does not change if the database moves to a new location and its url , authors ' names or the contact person address are updated . the complete database list and summaries are available online at the nucleic acids research website .
an 18-year - old female patient was referred from the department of prosthodontics regarding inadequate clinical crown heights for full mouth prosthetic rehabilitation . on intra - oral examination , the patient revealed short clinical crowns , occlusal wear with exposed dentin in the posterior teeth , and asymmetry of the gingival contours in the anterior teeth . the teeth were yellowish with the lack of contact points and revealed the absence of enamel in all the teeth [ figures 13 ] . preoperative photograph clinical presentation showing short clinical crowns orthopantomograph showing short crowns with taurodontism in consultation with the patient full maxillary and mandibular rehabilitation with porcelain fused metal ( pfm ) crowns extending to the second molars was considered to be the best therapeutic option . as the clinical crowns were inadequate for the placement of the pfm crowns , a full mouth surgical crown lengthening was planned . due to deficiency of sufficient tooth structure for prosthetic rehabilitation intentional endodontic therapy was done for 11 , 12 , 13 , 21 , 22 , 23 , 31 , 32 , 33 , 41 , 42 , 43 . coronal restorations carried out using glass ionomer cement . once the crown lengthening procedure has been decided the patient 's smile line gingival esthetic line , crown root ratio are evaluated , and the anticipated finish line is determined prior to the surgery . the biologic width determines the amount of bone to be removed . under the administration of 2% lignocaine sulcular and vertical releasing incisions were placed and a full mucoperiosteal flap was elevated in the labial and palatal / lingual aspects of the operated teeth . respective osseous procedures were performed using rotary instruments to achieve exposure of sufficient tooth structure coronal to the alveolar crest [ figure 4 ] . intraoperative : after ostectomy the flaps were sutured at the level of the alveolar crest with interrupted sutures . due to deficiency of sufficient tooth structure for prosthetic rehabilitation intentional endodontic therapy was done for 11 , 12 , 13 , 21 , 22 , 23 , 31 , 32 , 33 , 41 , 42 , 43 . coronal restorations carried out using glass ionomer cement . once the crown lengthening procedure has been decided the patient 's smile line gingival esthetic line , crown root ratio are evaluated , and the anticipated finish line is determined prior to the surgery . under the administration of 2% lignocaine sulcular and vertical releasing incisions were placed and a full mucoperiosteal flap was elevated in the labial and palatal / lingual aspects of the operated teeth . respective osseous procedures were performed using rotary instruments to achieve exposure of sufficient tooth structure coronal to the alveolar crest [ figure 4 ] . intraoperative : after ostectomy the flaps were sutured at the level of the alveolar crest with interrupted sutures . final tooth preparation was done after 6 months . full mouth porcelain fused to ceramic crowns placed , massive loss of tooth substance in ai results , in short , clinical crown heights that compromise the retention and resistance of the restorations necessitating crown lengthening procedures . the primary objective of the crown lengthening procedure is the restoration of the adequate biological width . biologic width is the physiologic dimension of the junctional epithelium and connective tissue attachment which is relatively constant at approximately0.07 mm of connective tissue attachment , 0.97 mm of junctional epithelium , and 0.69 mm of gingival sulcus . infringement on the biologic width by the placement of a restoration within its zone may result in gingival inflammation , pocket formation and alveolar bone loss . hence , it is recommended that there should be at least 3.0 mm between the restoration margins and bone crest . surgical crown lengthening includes the removal of soft tissue or both soft tissue and alveolar bone . reduction of soft tissue alone is indicated if there is adequate attached gingival and more than 3 mm of tissue coronal to the bone graft . this may be accomplished by either gingivectomy or flap technique , which allows for adequate biologic width when the restoration is placed 0.5 mm within the gingival sulcus . the time interval between the surgical procedure and final preparation and placement of restorations are much important . it has been reported that the marginal periodontal tissue show a tendency to grow coronally after surgery . the amount of tissue rebound seems related to the position of the flap relative to the alveolar crest at suturing . hence , it is critical that proper crown height be established during surgery without relying on flap placement at the osseous crest . it has been suggested that considering the postsurgical coronal rebound of the marginal periodontium , the final preparation and restorations be delayed for 12 months after the crown lengthening procedure and that any intermediate preparations be delayed for at least 6 weeks after surgery . in situations where the restorations can not be delayed , the restoration margins can be placed coronal to the gingival margin so that when the tissues grow coronally the restoration margins may shift to an acceptable subgingival position . early treatment of a patient with ai disorder can prevent progressive damage of dentition and the psychological impact of this condition . when crown lengthening procedure is required for prosthodontic rehabilitation , the biologic width needs to be considered . the understanding of the relationship between periodontal tissues and restorative dentistry is paramount to ensure adequate form , function , esthetics , and comfort of the dentition .
full mouth rehabilitation includes a promising treatment planning and execution thus fulfilling esthetic , occlusal , and functional parameters maintaining the harmony of the stomatognathic system . crown lengthening procedures have become an integral component of the esthetic armamentarium and are utilized with increasing frequency to enhance the appearance of restorations placed in the esthetic zone . crown lengthening plays a role to create healthy relationship of the gingiva and bone levels so as to gain access to more of the tooth which can be restored , if it is badly worn , decayed or fractured , below the gum line . this paper highlights the full mouth crown lengthening procedure performed on a patient with amelogenesis imperfecta .
serotonin syndrome refers to a disorder which results from excess serotonergic stimulation at central and peripheral serotonin receptors , usually caused by drugs or drug interactions . it was first defined in 1955 , but the precipitating drugs , signs , and symptoms became more common only after 1990s . despite its high prevalence ; serotonin syndrome is often under - diagnosed . this case report highlights a rare incidence of serotonin syndrome due to drug interactions of linezolid with rasagiline ; monoamine oxidase ( mao ) inhibitor . a 65-year - old female patient was admitted to the hospital with fever , chills and pain in left leg . she was prescribed levodopa / carbidopa , rasagiline , ropinirole , trihexyphenidyl , amantadine , metformin , and glipizide since 2 years . she also had multiple hospital and intensive care unit ( icu ) admissions for worsening parkinsonism . she was admitted to the ward for cellulitis and treated with intravenous clindamycin 600 mg thrice daily and intravenous benzylpenicillin 2 million units 4 times daily . on the 3 day of hospital admission , she had persistently high - temperature spikes ( above 102f ) , tachycardia and tachypnea . she became hypotensive and encephalopathic in the ward hence ; she was shifted to icu . considering sepsis with multi - organ dysfunction syndrome antibiotics was escalated to intravenous linezolid 600 mg twice daily and intravenous piperacillin with tazobactam 4.5 gm thrice daily . vancomycin was not considered taking into account her borderline renal parameters . on admission to the icu , her anti - parkinsonism drugs were continued ; oral hypoglycemic agents were stopped and switched to insulin on icu admission . later , the patient also had myoclonus , tremors , jerky movements , and there was no neck stiffness . computed tomography of the brain and cerebrospinal fluid analysis were not significant . her laboratory investigation revealed improving white blood cell counts from 14,200 cells / mm to 11,000 cells / mm ( normal range - 450012,500 cells / mm ) , better glycemic control and no other abnormalities . the second day of icu admission , her status remained the same with a high temperature of 103f , altered mental status , myoclonus , jerky movements and tremors . no other drugs were added , the same treatment was continued , and the medications she received were scrutinized . serotonin syndrome was suspected after ruling out other possibilities such as infection , drug abuse , cerebrovascular , and metabolic etiologies . causal association of serotonin syndrome as assessed by naranjo causality scale and world health organisation - uppsala monitoring centre causality assessment system with a naranjo score of six . the drug interaction probability scale assessment also revealed probable causality of serotonin syndrome due to rasagiline and linezolid . after 8 hours temperature settled , next day the patient 's heart rate was normal , her sensorium improved and tremors completely subsided . after 10 days , she started walking with support and was discharged from the hospital . linezolid is an oxazolidinone class of antibiotic and a weak , reversible mao inhibitor . in 2003 , records from postmarketing voluntary adverse event reporting system database identified 29 cases of serotonin syndrome associated with linezolid . in this case , a combination of serotonergic drugs ; linezolid and rasagiline were most likely to be responsible for serotonin syndrome . diagnosis of serotonin syndrome is purely on the basis of patient presentation as there are no laboratory investigations or blood serotonin levels for confirmation . it requires a strong clinical suspicion , proper medication history of serotonergic drugs and identification of specific signs and symptoms . clinical examination , laboratory investigations , imaging , and cultures ruled out the other possibilities that favour towards an infectious focus of fever in our case . the offending drugs in our case , linezolid and rasagiline were the only temporal cause for fever associated with serotonin syndrome . the serotonergic toxidrome is identified using hunter serotonin toxicity criteria , boyer 's and sternbach 's criteria . the clinical presentation is marked by a triad of cognitive changes ( confusion , visual hallucinations , elevated mood , agitation , insomnia , nervousness , delirium or coma ) , autonomic instability ( fever , hyperhidrosis , hypertension , palpitations , or tachycardia ) , and neuromuscular excitability ( myoclonus , tremors , chills , rigidity , hyperreflexia or akathisia ) . our case presented with fever , confusion , drowsiness , tremors , myoclonus , and jerky movements which were typical features to fulfill the diagnosis of serotonin syndrome . the onset of serotonin syndrome ranges from few hours to 3 weeks of increase in dose or initiation of drugs that may increase serotonin levels . in our case report , the patient was on rasagiline and presented with serotonin syndrome within 36 hours of initiation of linezolid . time to resolution of serotonin syndrome after discontinuation of the culprit drugs ranges from < 6 hours to 5 days . in our case , signs and symptoms resolved 8 hours after stopping both serotonergic drugs without any other treatment . an initial dose of 48 mg given orally , and maximum dose is 32 mg / day . if linezolid is to be administered , it is recommended that the serotonergic drug must be discontinued , or there must be a washout period of at least 2 weeks and observe the patient for serotonin syndrome . patients with unresolving fever , prescribers should think of the drugs and drug interactions causing the serotonergic syndrome . prescribers should know the medication history of the patient and washout period of serotonergic drugs . serotonin syndrome is self - limiting if identified early , culprit drugs are discontinued , and supportive care is initiated .
a 65-year - old female patient was admitted to the hospital for cellulitis . she had a history of diabetes mellitus and parkinsonism on levodopa / carbidopa , rasagiline , ropinirole , trihexyphenidyl , amantadine , metformin , and glipizide . we present here a case of rare incidence of serotonin syndrome associated with linezolid and rasagiline .
a 56-year - old male patient presented to our emergency department with chief complains of inability to void urine for 24 hours and vague abdominal pain . he was diagnosed as a case of acute urinary retention and was referred to the surgical team for further management . the surgical junior resident briefly examined him and attempted catheterization . after failing twice he decided to go for percutaneous suprapubic trocar catheterization . a residual volume of 2 l of coffee ground colored urine was noted on catheterization which was recorded as hematuria . later , he was reviewed next morning when the catheter bag was found empty without any drainage for 10 hours . on further questioning he denied any history of any symptoms of bladder outlet obstruction or urinary tract infection . he never had catheterization or urethral instrumentation before and denied any history suggestive of urethritis . on laparotomy the stomach was found to be dilated with the catheter tip along with the balloon in it [ figure 1 ] . it was a case of benign gastric outlet obstruction with a hugely distended ptotic stomach . but on the fourth postoperative day the patient had a massive upper gastrointestinal bleed from which he died in spite of all attempts for resuscitation . emergency suprapubic catheterization is a rewarding treatment for acute urinary retention due to urethral stricture , urethral trauma and complex prostatic disease . in most of the hospitals because of non availability of emergency urologist is not it is done by the relatively less experienced surgical trainees . the complications of this procedure are infection , inadvertent ureteral catheterization , extravasation of urine to intra or extraperitoneal space , clot obstruction of the catheter , extrusion of the catheter , hematuria , and bowel perforation . two cases have been reported in the literature where a delayed injury to ileum occurred while changing the catheter after a few months ( 2 months and 8 months ) of suprapubic cystostomy . a case has also been reported in the literature where occult transfixation of the sigmoid colon by a suprapubic catheter occurred . so blind suprapubic trocar cystostomy should be avoided in patients with previous history of midline laparotomy or lower abdominal operations . a small korean study by kim et al . showed that a preliminary ultrasound examination before suprapubic troar catheterization avoided bowel injury in comparison to 3.3% incidence of bowel injury without it . meticulous history taking and thorough and meticulous clinical examination are extremely important to reduce the risks associated with this procedure . the surgeon should carefully palpate the bladder and wait for adequate bladder distension . putting the patient in trendelenburg position also shifts the intraabdominal organs away from the puncture site and thus helps avoiding bowel injury . the availability of bladder scanners confirms the diagnosis and measure the bladder volume and thus making reduces the possibility of various complications . in our case we found that inadequate patient assessment , lack of experience of the junior surgical trainee , absent senior surgical input , nonavailability emergency urologist , and nonavailability of bladder scanner were the possible causes of this preventable complication . it has clearly demonstrated the importance of careful and meticulous history taking and physical examination . a good history and clinical examination it also reminds us the importance of taking all the necessary precautions to minimize the complications of blind suprapubic trocar cystostomy . whenever possible a quick bladder scan should be done before suprapubic cystostomy . this report will make a surgeon aware about the possibility of this unique but unlikely complication .
emergency percutaneous trocar suprapubic cystostomy is a common surgical procedure for acute urinary retention . although uncommon it can be associated with a few complications . the most dangerous complication is iatrogenic bowel injury . literature shows reported cases of small and large bowel injuries . we report a case of inadvertent placement of suprapubic catheter into a dilated and ptotic stomach . this is the first reported case of this complication of suprapubic cystostomy .
pneumatosis intestinalis ( pi ) is a physical or radiographic finding caused by a large variety of underlying conditions and ranges from benign to fatal . multiple mechanisms underlying the pathogenesis of pi have been proposed , but the pathogenesis is not yet fully understood . several chemotherapeutic agents are associated with pi , including cyclophosphamide , cytarabine , vincristine , doxorubicin , etoposide , docetaxel , irinotecan , and cisplatin [ 2 - 5 ] . abdominal radiography and computed tomography ( ct ) are the best techniques for diagnosing pi . we report a rare case of asymptomatic pi in an 18-yr - old man after induction of chemotherapy for b lymphoblastic leukemia with recurrent genetic abnormalities . an 18-yr - old man who had been diagnosed with b lymphoblastic leukemia with a t(1;19)(q23;p13 ) ; ( e2a / pbx1 ) complex karyotype was admitted for consolidation chemotherapy . he was treated with induction chemotherapy 2 mo prior to admission , and his bone marrow showed complete remission ( cr ) . the induction chemotherapy consisted of prednisolone , daunorubicin , vincristine , asparaginase , and intrathecal methotrexate . on admission chest radiography and upright abdominal radiography revealed gas collection along the wall of the colon and intraperitoneal free air ( fig . 1 , 2 ) . the vital signs were normal . on physical examination , the patient presented only with slight abdominal distention . laboratory results revealed a leukocyte count of 8.010/l with 87% neutrophils , a platelet count of 27810/l , and a hemoglobin level of 9.9 g / dl . other laboratory findings , including electrolytes , liver function tests , amylase , and crp , were within normal ranges . no acidosis was shown by atrial blood gas analysis . abdominal computed tomography ( ct ) was performed and revealed intramural gas along the colon as well as intraperitoneal and retroperitoneal free air ( fig . since there were no signs of secondary complications , such as peritonitis , conservative treatment was initiated . the patient was treated with high concentrations of oxygen ( 40% oxygen ) , broad - spectrum antibiotics ( ceftazidime , metronidazole , and teicoplanin ) , and parenteral alimentation ; oral intake was withheld . after 2 wk of therapy , abdominal radiography and ct showed almost complete resolution of pi . the patient progressed well , and abdominal ct taken after 4 wk of therapy showed no evidence of pi . however , a few days later , the patient developed fever , and blasts were seen on his peripheral blood smear . these findings were similar to his condition on the first diagnosis of acute lymphoblastic leukemia ( all ) . hyper - cvad ( cyclophosphamide , vincristine , doxorubicin , dexamethasone ) chemotherapy was initiated , and a follow - up bone marrow study revealed a second cr . the patient progressed well and received a haploidentical stem cell transplantation from his mother , because he did not have an hla - matched sibling or unrelated donor . pi is a rare condition characterized by multiple pneumocysts in the submucosa or subserosa of the bowel . several pathogenic mechanisms have been proposed to underlie pi : ( 1 ) increased intraluminal pressure , ( 2 ) defective mucosal integrity , and ( 3 ) bacterial gas production . mechanical obstruction , blunt trauma , or vomiting can increase intraluminal pressure and cause pi . enhanced gut permeability to gas can be induced by defects in the mucosa or the gut 's immune barrier ( intramural lymphoid tissue ) . attenuation of these barriers may account for pi in circumstances of normal intraluminal pressure , as seen in patients with pi due to immunosuppressive therapy and cytotoxic therapy . when the mucosal or immune barriers of the gi tract are compromised , bacterial invasion or gas diffusion into the wall becomes more likely . this theory is supported by reports that the gas disappears on treatment with antimicrobial drugs . based on an autopsy study , the overall incidence of pi in the general population is 0.03% . it has been reported that 15% of pi is idiopathic , and 85% is secondary to various conditions , including intestinal ischemia or infarction , inflammatory bowel diseases , infections , endoscopic procedures , surgeries , alpha - glucosidase inhibitor , and some chemotherapeutic agents . the differential diagnosis for acute abdomen and radiological images of the abdomen are important for diagnosis of pi . abdominal radiography and ct are the best techniques for diagnosing pi . on both radiographs and ct , pi usually appears as a low - density linear or bubbly pattern of gas in the bowel wall . ct is also more sensitive for detecting hepatic portal and portomesenteric venous gas [ 11 - 13 ] , the presence of which increases the possibility of pi due to life threatening causes . however , pi can be asymptomatic and resolve spontaneously in some cases . in mild cases , such as the present case , conservative treatment with antibiotics and nasogastric decompression the prognosis of pi ranges from benign to fatal and depends on underlying diseases and complications . many reports have indicated that pi is fatal in cases with intraportal gas , perforation , or severe sepsis . in contrast however , the presence of pneumoperitoneum and/or portal venous gas does not necessarily represent an abdominal crisis requiring operative intervention . therefore , the clinical history , physical examination , and laboratory tests are important for determining the management of pi . in the case of asymptomatic pi , if a patient with asymptomatic pi is treated with a scheduled chemotherapy regimen without perceiving pi , myelosupression by chemotherapy may exacerbate pi and cause life - threatening complications , such as severe sepsis or bowel perforation . , pi should be considered as a complication during or after chemotherapy for patients with hematologic malignancies .
pneumatosis intestinalis ( pi ) is a rare condition characterized by multiple pneumocysts in the submucosa or subserosa of the bowel . here , we report a rare case of asymptomatic pi after chemotherapy induction in an 18-yr - old man with b lymphoblastic leukemia with recurrent genetic abnormalities . the patient was treated conservatively and recovered without complications . the possibility of pi should be considered as a complication during or after chemotherapy for hematologic malignancies . conservative treatment should be considered unless there are complications , including peritonitis , bowel perforation , and severe sepsis .
the last couple of years have seen a marked growth of interest in the topic of crossmodal correspondences ( see marks , 2004 ; spence , 2011 , for reviews ) . crossmodal correspondences have been defined as a tendency for a sensory feature , or attribute , in one modality , either physically present or merely imagined , to be matched ( or associated ) with a sensory feature in another sensory modality ( see parise & spence , in press ; spence , 2012 ) . crossmodal correspondences have now been documented between pretty much every pair of sensory modalities ( see spence , 2012 ) . crossmodal correspondences affect behavior in a wide range of experimental paradigms everything from the redundant target paradigm through to the implicit association test ( see spence , 2011 ) . but how should such phenomena be explained at the computational / neural level ? at the computational level , bayesian coupling priors appear to provide a powerful tool to model the effects of crossmodal correspondences on multisensory integration ( ernst , 2007 ; parise & spence , 2009 ; spence , 2011 ) . the latest cognitive neuroscience research is now starting to provide answers regarding the time course and cerebral localization of these , often surprising , cross - sensory mappings ( bien , ten oever , goebel , & sack , 2012 ; sadaghiani , maier , & noppeney , 2009 ; seo , et al . one critical research question in this area concerns whether crossmodal correspondences are all of a kind or whether instead there are different kinds / mechanisms of correspondence at work ( spence , 2011 ) . one might , for example , want to know whether those correspondences that are based on the internalization of the correlations between stimuli / dimensions present in the environment are represented differently than the semantic correspondences associated with our tendency to use the same terms to describe , for example , certain sounds as having a high frequency while at the same time also using the same descriptor when talking about certain elements in a perfume or perhaps wine aroma ( so - called high notes ) . while discriminating between different kinds of crossmodal correspondence has sometimes been possible using behavioral techniques ( gallace & spence , 2006 ) , it is undoubtedly true that more robust insights into the shared vs. distinct mechanism(s ) underlying various examples of crossmodal correspondence are likely to emerge from a better understanding of the neural substrates underpinning these effects . in their latest research , 2012 ) utilized a simplified version of the psychophysical paradigm developed by parise and spence ( 2009 ) demonstrating enhanced multisensory integration for crossmodally corresponding pairs of auditory and visual stimuli ( small or large circles and low- vs. high - pitched sounds ; with integration being assessed by means of the magnitude of the spatial ventriloquism effect ) . using event - related potentials ( erps ) , bien et al . ( 2012 ) looked for any differences in the pattern of neural activation on those trials where crossmodally congruent vs. incongruent pairs of stimuli were presented . 2010 ) have also demonstrated similar effects when comparing the erps elicited by crossmodally congruent vs. incongruent combinations of abstract visual symbols and orthonasally presented odors ( see also kovic et al . , 2009 ) . interestingly , the magnitude of the p2 component at parietal recording sites correlated with the effects reported behaviorally in the study of bien et al . these researchers then used source localization to infer the likely source of this congruency effect as originating in right intraparietal sulcus , a site that was then temporarily lesioned by applying offline transcranial magnetic stimulation ( tms ) . crucially , this intervention led to the elimination of the effect of crossmodal congruency on participants ' behavioral performance ( not to mention on the erp signal ) . ( 2012 ) builds nicely on the earlier psychophysical research of parise and spence ( 2009 ) while also complementing the results of a study by sadaghiani et al . ( 2009 ) that highlighted differing neural substrates for natural , metaphorical , and linguistic mappings ( or crossmodal correspondences ) . that said , given that bien et al . ( 2012 ) did not fit psychometric curves to their data , it is still an open question whether the effects induced by tms are due to the reduced integration of crossmodally congruent stimulus pairs or else to a horizontal shift in perceptual space . in an earlier tms study by muggleton , tsakanikos , walsh , and ward ( 2007 ) , lesioning of right intraparietal cortex using tms knocked out stroop - like interference effects in a group of synesthetic participants . however , before jumping to the conclusion that the same neural substrates may underlie both crossmodal correspondences and synesthesia , it is worth noting that the cortical site targeted in the two studies was actually separated by a few centimeters . of course , the fact that tms was not applied over a control site in the study of bien et al . ( 2012 ) also means that it is not possible to know exactly how specific ( in neural terms ) their tms effects really were . the temporary lesioning approach to the study of crossmodal correspondences highlighted by bien et al . ( 2012 ) complements previous anecdotal reports that damage to other neighboring regions ( e.g. , the angular gyrus , located within the temporal parietal occipital , region ) results in the loss of the takete maluma effect ( see figure 1 ) . in the future , our understanding of the mechanism(s ) underlying the many forms of crossmodal correspondence that have been documented behaviorally in recent years ( see parise & spence , in press ; spence , 2011 , 2012 ) are likely to be greatly enriched by further cognitive neuroscience research using techniques such as tms . indeed , one might even go so far as to consider whether certain coupling priors underpinning crossmodal correspondences ( e.g. , between auditory and visual stimuli ) might not be encoded in the intraparietal sulcus itself ( cf . better fits with the angular shape shown on the left , whereas the word maluma fits better with the rounded shape displayed on the right . even those individuals from cultures without any written language have been shown to exhibit this effect ( see bremner et al . the only exceptions to this generalization appear to be those suffering from damage to the angular gyrus ( ramachandran & hubbard , 2003 ) and certain individuals suffering from autism spectrum disorder ( oberman & ramachadran , 2008 ; ramachandran & oberman , 2006 ) .
in a recent article , n. bien , s. ten oever , r. goebel , and a. t. sack ( 2012 ) used event - related potentials to investigate the consequences of crossmodal correspondences ( the natural mapping of features , or dimensions , of experience across sensory modalities ) on the time course of neural information processing . then , by selectively lesioning the right intraparietal cortex using transcranial magnetic stimulation , these researchers went on to demonstrate ( for the first time ) that it is possible to temporarily eliminate the effect of crossmodal congruency on multisensory integration ( specifically on the spatial ventriloquism effect ) . these results are especially exciting given the possibility that the cognitive neuroscience methodology utilized by bien et al . ( 2012 ) holds for dissociating between putatively different kinds of crossmodal correspondence in future research .
emergency presentations of post - operative perineal hernia are very rare and not well described in the literature . we present a case of small bowel obstruction with strangulation secondary to a perineal hernia and the surgical technique employed to repair the defect . an 81-year - old lady presented to the on call general surgeons with abdominal pain and vomiting for 24 hr . two years previously she had undergone a laparoscopic abdominoperineal resection for carcinoma of the rectum at another centre . , there were no signs of peritonism , observations were normal and markers of inflammation were not significantly raised . although not known to have a hernia , she described discomfort and swelling in her perineum over the previous year that sometimes made it difficult to sit down . direct examination of the perineum confirmed the presence of an irreducible but non - tender hernia ( fig . 1 ) . the following morning a ct of the abdomen and pelvis was performed which confirmed small bowel obstruction and revealed a cut off at the neck of a perineal hernia . figure 1:clinical examination of the perineum revealing perineal hernia . clinical examination of the perineum revealing perineal hernia . at operation , small bowel obstruction was found with volvulus of a loop of ileum around an adhesion at the neck of the perineal hernia ( fig . 2 ) . around 80 cm of gangrenous mid - ileum was resected and a primary end - to - end anastamosis performed . after thorough washout of the abdomen and pelvis , the wide pelvic floor defect was bridged with strattice tissue matrix , secured in place with 2.0 prolene sutures ( fig . one abdominal tube drain and two perineal suction drains were inserted and removed over the course of the next 7 days . figure 2:laparotomy findings of transition point of small bowel obstruction at neck of perineal hernia . the patient made an uncomplicated recovery and was discharged home . at 6 months follow - up , she remained well with no reported problems other than a slight increase in stoma activity . there was resolution of her previous perineal symptoms and no clinical recurrence of the hernia . perineal hernia is an uncommon but well - documented complication of abdominoperineal resection of the rectum . the reported incidence is between 0.34% and 7% , but there may be significant under reporting by patients who experience minimal symptoms or do not report them to their surgeon . a hernia may become evident up to 5 years following surgery and risk factors for development include neo - adjuvant chemo - radiotherapy and smoking . when recognized , many are treated conservatively but repair is indicated for symptoms such as pain , skin erosion and intermittent obstruction . surgical repair can be a challenge , and trans - abdominal , perineal and combined approaches have been described . reconstruction of the pelvic floor defect has been described with various types of synthetic and biological mesh as well as myocutaneous grafts such as a vertical rectus abdominis flap . synthetic meshes are contraindicated in the presence of contamination from non - viable bowel or bowel contents . reconstruction using myocutaneous flaps is a specialist , time consuming procedure with an appreciable morbidity and usually inappropriate in an acute setting . the use of a biological mesh , or acellular porcine dermal matrix , has the advantage of a low risk of infection when used in a potentially contaminated field . combined with an omental pedicle graft to fill the pelvic space , this produced a safe and effective repair . this has not been previously described in the emergency setting for a strangulated perineal hernia . in the era of extra - levator abdominoperineal resection it remains to be seen whether this practice will lead to a change in the reported incidence of perineal hernia . although rare , strangulation of a post - operative perineal hernia can occur and should be considered when assessing patients with a history of proctectomy and small bowel obstruction . where repair of a perineal defect in potentially contaminated circumstances is anticipated , an appropriate biological mesh can be selected and careful preoperative planning is required .
perineal hernia is a recognized but uncommon complication following proctectomy . emergency presentations of this hernia are very rare and are not well described in the literature . we present the case of an 81-year - old lady who presented with small bowel obstruction with strangulation secondary to a perineal hernia 2 years after abdominoperineal resection for carcinoma of the rectum . at laparotomy , a small bowel resection was required and a biological mesh was used to repair the perineal defect .
a coronary arterial fistula is a connection between one or more of the coronary arteries and a cardiac chamber or great vessel , having bypassed the myocardial capillary bed . the feeding artery of the fistula may drain from a main coronary artery or one of its branches and is usually a dilated and tortuous artery terminating in one of the cardiac chambers or great vessels . complications can arise from a coronary ' steal ' phenomenon occurring from the adjacent myocardium causing myocardial ischemia , thrombosis and embolism , heart failure , rupture , endocarditis , endarteritis and arrhythmias.1)2 ) there have been many reported cases of coronary artery fistulas,3 - 5 ) but only few reports of thebesian veins in korea.6)7 ) here , we presented a unique case of extensive persistent thebesian veins in an old patient presenting as acute coronary syndrome . she was an active smoker ( 1 pack per day during 50 years ) with a past medical history of depression requiring pharmacotherapy . the patient 's blood pressure was 145/48 mm hg with a pulse rate of 62 beats per minute . although her initial electrocardiography ( ecg ) showed sinus rhythm without st segment changes , after 2 hours of observation , t - wave inversions on lead v2 , v3 and v4 were recorded ( fig . the patient was treated for an acute coronary syndrome and her symptoms were relieved with intravenous nitroglycerin . coronary angiography demonstrated a marked capillary blush draining into the left ventricular ( lv ) cavity due to extensive multiple microfistulae arising from the left anterior descending artery ( lad ) , left circumflex artery and right coronary artery ( rca ) producing a left ventriculography ( fig . was severely dilated ( up to 10 mm ) and the lad was diffusely calcified with a 40% narrowing at the proximal lad . rca was also markedly dilated with a discrete 30 - 40% luminal narrowing at the posterior descending artery and posterior lateral artery bifurcation . echocardiography demonstrated concentric lv hypertrophy , normal lv chamber size and normal lv systolic function without the regional wall motion abnormality . she remained asymptomatic and was discharged on a beta - blocker and angiotensin converting enzyme ( ace ) inhibitor . two months later , the t - wave inversions at v2 , v3 and v4 were normalized . coronary artery fistula is a rare congenital anomaly with an incidence of 0.2% in patients undergoing diagnostic cardiac catheterization.8 ) drainage most frequently occurs into the right ventricle . thebesian sinusoids that supply nutrients in the embryonic heart are responsible for coronary artery to ventricular shunts . the pathogenesis of these fistulous communications has been attributed to a persistence of intertrabecular spaces in localized areas of the myocardium.9 ) embryonic sinusoids are formed from endothelial protrusions that extend into the epicardial surface joining the coronary arteries . a fistulous communication may be due to a failure of these intertrabecular spaces to fuse . in the present case , the shunt flow was so large that the lmca was severely dilated and coronary angiography was sufficient to display ventriculography . her wide pulse pressure and low diastolic pressure might be possibly due to the coronary fistula . approximately half of all patients with coronary artery fistulae remain asymptomatic ; the other half develop congestive heart failure , infectious endocarditis , myocardial ischemia induced by a coronary steal phenomenon , or rupture of an aneurysmal fistula.9 ) in coronary artery fistulae to the left ventricle , most common presenting symptoms are chest pain , exertional dyspnea and fatigability.10 ) treatment of a large single fistula involves surgical correction or coil embolization whereas small multiple fistulae are usually managed medically similar with the present case , although there are controversies on optimal medications . for example , there was a report that showed myocardial ischemia aggravation following nitrate therapy.11 ) nitrate therapy probably increases ischemia by both increasing leakage into the left ventricle and decreasing the overall coronary artery flow to the myocardium . however , chest pain was relieved after the intravenous nitrate administration in the present case which suggested that mechanism of ischemia from persistent thebesian veins remains to be elucidated . in addition to nitrate , we prescribed a beta - blocker and ace inhibitor to reduce cardiac workload and afterload and to increase systemic flow . further discussion and research are required to seek for the optimal medical therapy for this severe form of thebesian veins . because our patient showed typical symptoms of exertional chest pain and ecg findings consistent with myocardial ischemia and responded to medical treatment for acute coronary syndrome however , further examination including upper gastrointestinal endoscopy , computed tomography and ergonovine provocation test could be considered for persistent symptoms or signs suggestive of other causes of chest pain .
coronary artery fistula is a rare congenital anomaly . most patients with this anomaly are asymptomatic , but some may develop heart failure , myocardial ischemia or arrhythmias . we report a case of a patient who presented with myocardial ischemia secondary to persistent thebesian veins . coronary angiography demonstrated a marked capillary blush draining into the left ventricular cavity through multiple microfistulae from the left anterior descending artery , left circumflex artery and right coronary artery . the patient was discharged without chest pain and was medically maintained with a beta - blocker and angiotensin converting enzyme inhibitor .
a 79-year - old man was admitted due to severe chest pain and dyspnea for 3 days . the patient 's medical history showed that he had undergone treatment for sustained ventricular tachycardia six years ago . echocardiography showed an ejection fraction of 31% , severe aortic valve stenosis ( v max=5.26 m / sec , mean pressure gradient [ pg]=69.4 mmhg , aortic valve area by continuity equation=0.6 cm ) and mild to moderate mitral valve regurgitation ( proximal isovelocity surface area radius=0.7 cm ) . coronary angiography showed no significant coronary artery disease , while chest computed tomography showed calcification of the posterior wall of the ascending aorta ( fig . a vent cannula was inserted into the left ventricle via the right superior pulmonary vein . a root cannula was inserted into the ascending aorta . a thoracic endovascular aortic repair ( tevar ) balloon catheter ( 4030 mm ) for endo - aortic occlusion was inserted into the ascending aorta via the left superficial femoral artery using a c - arm ( ge , bayern , germany ) ( fig . blood cardioplegia solution was administered to arrest the heart via root cannula , and then this solution was administered via direct cardioplegia cannula every 20 minutes . follow - up echocardiography showed an ejection fraction of 33% , a well - functioning aortic prosthetic valve ( mean pg=22.4 mmhg ) and minimal mitral valve regurgitation . as an increasing number of older patients undergo cardiac operations , severe atherosclerosis of the ascending aorta has been appearing more frequently . as much as 13% of the patients undergoing cardiac surgery will have a significant associated atheromatous disease of the ascending aorta . in a patient with severe atherosclerosis of the ascending aorta , conventional aortic valve surgery can be technically demanding and is associated with high morbidity and mortality . to clamp an atherosclerotic aorta during a cardiac operation can increase the risk of cerebral embolism by atherosclerotic debris . therefore , aortic valve surgery in a patient with severe atherosclerosis of the ascending aorta requires modifications of techniques for cpb . several reports have described modifications of surgical techniques for managing aortic valve disease in limited numbers of patients with a severely calcified ascending aorta . . reported on 62 of 4,983 patients that had undergone an aortic valve replacement and were noted to have atherosclerosis of the ascending aorta . hypothermic circulatory arrest ( hca ) including ascending aortic endarterectomy , ascending aortic replacement , and balloon occlusion of the ascending aorta were used for the patients . have described a " no - touch " technique under deep hca for the porcelain aorta . svensson et al . reported successful aortic endarterectomy to facilitate aortic valve replacement with a calcified ascending aorta . cosgrove reported a forward - balloon occlusion of the ascending aorta after performing an aortotomy with the aid of hca . although various surgical strategies have been described for managing atherosclerotic aorta in patients requiring aortic valve replacement , the appropriate techniques are unknown . in addition , these strategies all need hca . have described hca as a useful method for replacing the aortic valve in a patient with an atherosclerotic aorta . however , it has a limitation in its safe duration without ischemic injury occurring to the brain , and longer cardiac arrest time is also associated with high mortality and morbidity . in our case , we performed aortic valve replacement using retrograde endoaortic balloon occlusion without hca . we maintained the patient 's body at a temperature of about 32 , unlike during hca . this procedure was performed successfully , and postoperatively , the patient recovered without any complications . although this technique still has a greater risk of dislodging emboli than the " no - touch " technique under hca , we believe that it was effective and could be a good option for high - risk patients who have a pathology of the ascending aorta while reducing the risk of cerebral embolism and without the burden of hca .
a 79-year - old man was admitted to samsung changwon hospital due to chest pain and dyspnea . the ejection fraction was 31% and mean pressure gradient between the left ventricle and aorta was 69.4 mmhg on echocardiography . chest computed tomography showed severe calcification of the ascending aorta . aortic valve replacement was successfully performed using a thoracic endovascular aortic repair balloon catheter without classic aortic cross clamping . the patient was discharged on the eleventh postoperative day .
complications involving the spleen have been reported with infarction , abscess , subcapsular hematoma , rupture , and hyper - reactive malarial splenomegaly ( hms ) or tropical splenomegaly syndrome . the latter is commonly understood as an immunological reaction from the infected host and may be confused with lymphoproliferative syndromes . hms is usually described in tropical residents as a result of chronic malarial infection , and is mostly recognized in young and middle - aged adults . a unique case of hms complicated by splenic infarct affecting a caucasian child is reported . a 4-year 11-month - old boy from the united states was staying with his family in a tribal region in indonesia in the preceding five months . he had recurrent febrile illnesses during this period and had been given chloroquine intermittently as prophylaxis , but his general health started to deteriorate about a month before admission . he was first admitted to a local hospital and was diagnosed to have plasmodium vivax infection . he was treated with chloroquine ( the exact treatment could not be verified ) but the condition did not improve . he was also found to have increasing splenomegaly and severe anemia ( lowest hemoglobin recorded , 3.6 g / dl ) . red cell transfusion was carried out twice before he was evacuated to singapore for further management . the liver and spleen were grossly enlarged at 6 cm and 8 cm below the costal margins , respectively . the first full blood counts showed hemoglobin 9.8 g / dl , white cells 5.48 10/l , platelet 123 10/l . immature trophozoites and gametocytes characteristic of plasmodium falciparum were also seen ( figure 1 ) , with a parasite load of 0.1% . treatment with atovaquone 500 mg - proguanil 200 mg for three days was commenced . figure 1photomicrograph of the peripheral blood film with giemsa stain ( 100 ) showing an immature trophozoite ( t ) and a gametocyte ( g ) characteristic of plasmodium falciparum infection . photomicrograph of the peripheral blood film with giemsa stain ( 100 ) showing an immature trophozoite ( t ) and a gametocyte ( g ) characteristic of plasmodium falciparum infection . computed tomography revealed enlarged liver and spleen with no signs of thrombosis in the portal venous system . an area of infarction was found in the anterior aspect of the lower pole of the spleen ( figure 2 ) . figure 2axial and coronal contrast - enhanced computed tomography showing an infarct in the anterior aspect of the lower pole in the enlarged spleen . axial and coronal contrast - enhanced computed tomography showing an infarct in the anterior aspect of the lower pole in the enlarged spleen . two days later , his hemoglobin dropped to 8.3 g / dl and another red cell transfusion was given . tests for glucose-6-phosphate dehydrogenase deficiency , direct coombs test , and occult blood in stool were negative . serum ferritin ( 664 mg / ml ) , bilirubin ( 15 mol / l ) , aspartate transferase ( 37 u / l ) , and alanine transferase ( 14 u / l ) were normal . serologic tests for hepatitis b , hepatitis c , human immunodeficiency virus , and parvovirus b19 were negative . lymphocyte subsets showed a mild increase in b cells ( 663/l , normal 65620 ) only . a week after completion of the anti - malarial treatment , the liver was no longer palpable and the spleen was 4 cm below the costal margin . repeat hemoglobin was 11.7 g / dl , identical to the post - transfusion measurement . parasite load was less than 0.01% , and no more parasites were seen on the blood film a week later . hyper - reactive malarial splenomegaly is believed to be an immunological complication of malarial infection . defective function of suppressive t cells leads to dysregulation of b cells and over - production of igm . the deposition of immune complexes in the reticuloendothelial system results in the enlargement of the liver and spleen even in the absence of significant parasitemia as illustrated in this case . differentiation from chronic lymphoproliferative disorders is important in the adult patient , but this is usually not an issue in childhood , though the condition has rarely been reported in pediatric ages . however , diagnostic confusion may happen as hms may be mistaken as other febrile illnesses in the young child , or when the child presents late after returning from an endemic area . splenic infarct is an unusual complication from malarial infection , but it is often innocuous and no treatment is required . splenic infarct has to be distinguished from abscess which has also been noted after malarial infection . spontaneous rupture is a recognized risk in patients with grossly enlarged spleens with an estimated risk of 2% in those affected by malaria . clinicians evaluating patients returning from the tropics should be aware that diagnostic and therapeutic details from remote healthcare facilities may be difficult to verify . failure to eradicate the parasite may be encountered because of inaccurate parasite identification , re - infection , inadequate drug dosing , drug resistance , or omission of the treatment for the hepatic stage of infection . it is not clear if the malaria parasite had been misidentified or a co - infection with two malarial species had not been found at the initial stage in the reported case . nevertheless , severe splenic complications following malaria should be looked out for in any child returning from the tropics , and they should be followed up for radical cure .
a 4-year - old boy from the united states had been staying in indonesia for five months when he presented with fever , severe lethargy , progressive weight loss , and abdominal distension . he was first diagnosed with plasmodium vivax infection in indonesia and received treatment with chloroquine . however , his condition continued to deteriorate and he required erythrocyte transfusion for severe anemia . three weeks into his illness , he was found to have low parasitemia with plasmodium falciparum with massive hepatosplenomegaly in singapore . a splenic infarct was also documented on computed tomography . treatment with atovaquone - proguanil resulted in stabilization of the hemoglobin level and rapid reduction in splenic size , with clearance of malarial parasites from the bloodstream . although reported typically in adult tropical residents , hyper - reactive malarial splenomegaly may occasionally be found in the pediatric traveler . clinicians receiving children returning from the tropical regions should be aware of this potentially life - threatening complication of partially treated malaria .
i used published data reporting possible incubation periods for 17 patients ( 6,7 ) plus data from two case - patients in an unpublished database maintained at the centers for disease control and prevention ( cdc ) . i built a simulation model in a standard computer spreadsheet ( excel 2000 , microsoft corp , redmond , wa ) ( see appendix ) . i first listed each possible incubation period for every patient for whom incubation period data were available ( table ) . then , for every patient , i assigned a random number generator ( function rand in excel software ) to each possible incubation period . this method is the equivalent of using a uniform distribution to select an incubation period from all possible choices . using a spreadsheet - based simulation software package ( @risk , palisade corp . , newfield , ny ) , i programmed the spreadsheet to run iterations of the model . during a single iteration , for each patient , the programmed model selects the incubation period with the highest random number for that iteration . after a single iteration , then , the program assigns another set of random numbers to each possible incubation period and selects and calculates the frequency distribution . after numerous iterations , the program combines all the frequency distributions from all iterations to provide a general frequency distribution . from this final frequency distribution , descriptive statistics can be obtained , such as the mean , median , 5th and 95th percentile values . i ran approximately 10,000 iterations , at which point each additional iteration caused the mean and the standard distribution for each possible day of incubation to change by < 1% . the three largest mean frequencies of incubation periods among the patients examined were 2 , 3 , and 6 days ( figure 1 ) . incubation periods of 1 , 4 , 5 , and 10 days were the second highest mean frequencies ( figure 1 ) . however , the confidence intervals ( 5th and 95th percentiles ) for most of the potential incubation periods clearly overlapped ( figure 1 ) . this finding indicates that with the given data set , an incubation period of 10 days is almost as likely to occur as an incubation period of 6 days . using the mean frequency of each incubation period , the 95th percentile is 12 days , with a median ( 50th percentile ) of approximately 4 days . data used for this simulation were obtained from canada ( 6 ) , hong kong ( 7 ) , and the united states , for a total sample size of 19 . many of the patients included in the database had multiple possible incubation periods ( see table ) , resulting in the confidence intervals displayed for each day . data are the mean frequencies of each individual incubation period , as shown in figure 1 . data used for this simulation were obtained from canada ( 6 ) , hong kong ( 7 ) , and the united states , for a sample size 19 . many of the patients included in the database had multiple possible incubation periods ( see table ) . the incubation period for sars is likely to be varied , with the frequency distribution being nonnormal ( figure 1 ) . thus , using mean incubation periods for activities such as mathematical modeling will probably result in a misrepresentation of sars transmission . the type of analysis presented here can help public health officials determine minimum quarantine periods for persons exposed to sars , who are not yet symptomatic . for example , public health authorities should be aware that in a small percentage of case - patients , the incubation period might be > 10 days ( figure 2 ) . given that data from only 19 patients were available for this analysis , some caution should be exercised when evaluating the results . adding or subtracting relatively small numbers of patients can cause estimates such as the 95th percentile of the cumulative frequency to change . the advantage of the method used here is that such data need not be specific . the method readily accepts data in which patients have multiple possible incubation periods . more data will likely reduce the confidence intervals for the frequencies of each incubation day ( figure 1 ) , giving a clearer picture of the actual frequency distribution of all incubation periods . the method can also be readily adapted to examine other aspects of sars epidemiology when unambiguous data are scarce . for example , with the appropriate data , this method can be used to examine the frequency distribution of when an infectious person infects other people . ( an excel workbook [ excel 2000 , microsoft , corp , redmond , wa ] containing the model used to calculate the results shown in figures 1 and 2 , and using the data shown in the table , is available in the appendix ) . also , distributions of incubation periods can be used to examine whether an association exists between incubation period and likelihood of hospitalization or death .
many severe acute respiratory syndrome ( sars ) patients have multiple possible incubation periods due to multiple contact dates . multiple contact dates can not be used in standard statistical analytic techniques , however . i present a simple spreadsheet - based method that uses multiple contact dates to calculate the possible incubation periods of sars .
the hernia sac passes through the obturator foramen , following the path of the obturator nerves and muscles . here we present a typical case of obturator hernia which was diagnosed by spiral ct preoperatively and the emergency operation was performed successfully . a 78-year - old woman was sent to our emergency department because of recurrent abdominal pain and constipation for 10 days . the colicky pain was getting worse in recent nearly six days , accompanied with nausea and vomiting . she also complained of intermittent right hip pain with radiation to the medial aspect of the thigh for several months , which worsened with extension , abduction , or internal rotation of right leg . the body weight was 38 kg . physical examination revealed a distended abdomen without tenderness or muscle guarding . the abdominal plain film revealed small intestine obstruction . enhanced ct scan demonstrated obvious intestinal obstruction with a low density mass in the right obturator canal area ( figure 1 ) the diagnosis of right obturaor hernia was confirmed and emergency laparotomy was performed . during the operation , a 10 cm loop of small intestine was revealed herniated into the right obturator canal ( figure 2 ) . the incidence is nearly 1% of all hernias 2 . with the nickname little old lady 's hernia , it usually occurs in multiparous and elderly emaciated women due to a wider pelvis and enlarged obturator canal . the patient had a positive howship - romberg sign , which was caused by the intermittent irritation of the obturator nerve . it was reported that 15~50% patients of obturator hernia may have positive howship - romberg sign 1 . in her early course the loss of the adductor reflex , named as hannington - kiff sign , was observed on the affected side , while the patellar tendon reflex was intact on the same side 3 . various imaging examinations have been applied to establish the diagnosis , including ultrasonography , herniography , ct scan and so on . among them , ct scan has superior sensitivity and accuracy 4 . in our case , preoperative diagnosis of obturator hernia was confirmed by spiral ct scan and emergent operation was performed . chang et al concluded that the duration of symptoms was one of the major factors affecting the rate of bowel resection . there are a variety of operative approaches including inguinal , retropubic and transperitoneal approach 6,7 . laparotomy via low midline incision was applied in this case because it had advantages of better exposure and facilitation of bowel resection when necessary . the advantages of laparoscopic surgery include less postoperative pain , shorter hospital stay and lower complications . however , it is usually reserved for the nonstrangulated hernia because of more challenging techniques and longer learning curve . methods of repair include simple suture closure , closure of the obturator with adjacent tissue , and mesh replacement during laparotomy 9 . many authors preferred a simple closure of the hernial defect with one or more interrupted sutures , in case of bowel resection 6 , 8 . in this case we did the simple suture and outcome was satisfying . in conclusion , it should be kept in mind that obturator hernia is a rare but significant cause of intestinal obstruction especially in emaciated elderly women .
obturator hernia is a rare pelvic hernia with incidence of 1% . it 's a significant cause of intestinal obstruction in emaciated elderly women . delayed diagnosis and surgical intervention contributed to its relatively high morbidity and mortality . we present a typical case of obturator hernia with positive howship - romberg sign and hannington - kiff sign . the diagnosis was confirmed by spiral ct preoperatively . during the emergency laparotomy , the incarcerated intestine was reduced and removed . obturator foramen was repaired by simple suture . the patient recovered uneventfully and no recurrence occurred during the follow - up . the obturator hernia should be included in the differential diagnosis if clinically suspected . early diagnosis and prompt surgical treatment are essential to reduce the morbidity and mortality associated with obturator hernia .
the abdominal cocoon is an infrequently encountered cause of intestinal obstruction that was was first observed by owtschinnikow in 1907 , and later , termed the cocoon by foo in 1978 . the exact aetiology of this entity ( also called scerosing encapsulating peritonitis ) is by and large unknown ( the idiopathic / primary variety of cocoon ) , but at times , cocoon formation can occur secondary to a variety of conditions ( secondary cocoon ) like drugs ( practolol , propranolol ) , chronic ambulatory peritoneal dialysis , ventriculoperitoneal shunts , diseases such as sarcoidosis , sle , liver cirrhosis , uterine leiomyomas , endometriotic cysts or tumours of the ovary , etc . ( 14 ) we report the unusual case of a young female patient with repeated episodes of intestinal obstruction due to further encasement of the distal ileum within a separate membrane that was wholly within the outer covering cocoon a cocoon within a cocoon. a 16 year old female patient presented to us repeatedly with episodes of small bowel obstruction that had previously responded to conservative management . this time , she had a vague mass in the right iliac fossa in addition to her obstructive features . although she responded to conservative management this time also , the mass did not disappear . a computerised tomogram ( ct ) scan was done to assess the mass , which revealed a fixed obstruction at the terminal ileum along with dilatation of the small bowel above . the whole of the stomach , small bowel and part of the ascending colon were encased by a thin membrane ( figure 1 ) . thin membrane that was encasing the stomach , small intestine and part of the ascending colon ( black arrows ) once this membrane was opened up , it was found that the terminal ileum was jumbled up and encased within another thick membrane ( figure 2 ) that was separate from the outer covering . jumbled up terminal ileum encased within another thick membrane ( black arrow ) the post operative period was uneventful . the histopathology report of the two membranes was non - specific , and did not reveal any evidence of tuberculosis . since it was first reported in 1907 , the abdominal cocoon still remains uncommon as a cause of intestinal obstruction . a search on pubmed revealed only about 92 articles on this topic ( abdominal cocoon ) to date . as mentioned earlier , the cocoon can arise as a primary disease when there is no obvious etiology , or it can be secondary , in the setting of various factors as have been listed . the primary form of the disease is much more common than the secondary form , and is seen usually in young females , especially from the tropical or subtropical countries . although the etiology is not known , it has been hypothesised that it may arise as a response to subclinical primary viral peritonitis , gynecological infections , or retrograde menstruation ( 3 ) . however , as the primary abdominal cocoon has been seen to occur in all ages , and even in males , casts some doubt on these factors solely being involved in the pathogenesis of the primary form of this unusual disease . the offending condition is more obvious in the secondary form of abdominal cocoon , where bowel encapsulation occurs in the presence of a variety of factors listed previously . it is uncommon for the diagnosis to be made with confidence preoperatively , since these patients present with non - specific signs and symptoms such as colicky abdominal pain , emesis and recurrent episodes of intestinal obstruction . there are no specific radiological features of the disease that can give the diagnosis with certainty . plain x - rays of the abdomen may reveal evidence of small bowel obstruction dilated bowel loops and air - fluid levels . cauliflower appearance in which the intestinal loops are arranged in a concertina shape with a narrow base . a ct scan may show dilated ileal loops towards the centre of the abdomen , or even , encasement by a membrane ( 5 ) . however , it is often only at laparotomy ( 5 ) or during laparoscopy that the actual diagnosis is made , when part or whole of the small bowel is seen encased within a sac . although the sac usually encases the small bowel , at times , it can extend to involve the colon , liver and even the stomach . lysis of this sac is sufficient to relieve obstruction in most cases , but in a tubercular cocoon , it has been our experience that there are further adhesions between bowel loops that require adhesiolysis ( 3 ) . however , in the absence of obvious evidence of tuberculosis within the abdomen , the diagnosis of tuberculosis as a cause of cocoon is only made with certainty following histological analysis . the presentation reported here is unusual in that there was encasement of the terminal ileum within a sac , and this itself , along with rest of the small bowel , stomach and ascending colon was encased within a larger sac . despite an extensive search of available literature , we could not come across any similar case in which bowel being encased within a cocoon , that itself was encased within a larger cocoon , as reported here an abdominal cocoon within a cocoon !
intestinal obstruction is a fairly common surgical emergency that usually occurs due to herniae , adhesions , or bands . rarely , uncommon causes such as an abdominal cocoon are encountered , where part or all of the small intestine is encased within a thin membrane . we report one such case of abdominal cocoon , in whom , further encasement of the distal ileum was seen within a separate membrane .
multiple studies advocate the use of ergonomic computer workstations that minimize tension in these muscles1 . in recent years , increasing numbers of workers have reported tension and pain in the muscles around their eyes along with headaches resulting from computer work2 . however , few studies have focused on methods to reduce pain and tension in the muscles around the eyes , perhaps because evaluating eye fatigue is difficult . electromyography ( emg ) is the most commonly used physiological measurement tool , and facial emg is sensitive enough to distinguish subtle changes in emotion3 . studies measuring the activity of the orbicularis oculi muscles have been conducted on diverse populations , and orbicularis oculi activity has become an important predictor of eye fatigue4 , 5 . although newer computers have large monitors on which multiple tasks can be performed , an increasing number of users link two monitors to create a large display . despite this trend , no studies have examined the effect of dual monitor use on the muscles surrounding the eyes . therefore , this study compared the orbicularis oculi muscle activity during computer work with single and dual monitors . ten computer workers who were 22 to 27 years of age ( mean sd , 25.42.0 years ) were included in this study . the participants were 173.2 5.2 cm tall and weighed 63.2 4.7 kg . subjects with a history of injury or neurological deficit in their upper extremities or trunk during the previous year were excluded from the study . this study was approved by the inje university faculty of health sciences human ethics committee . the activity of the right orbicularis oculi muscle was recorded with a mp150 system ( biopac systems , santa barbara , ca , usa ) using an el254s ( 4 mm , ag / agcl ) electrode . all emg signals were sampled at 1,000 hz and analyzed with the acqknowledge 3.9.1 software ( biopac systems , santa barbara , ca , usa ) . , a single monitor was used ( l - company , 20 in ) , and subjects typed text that was displayed in a pdf file on the right side of a divided display into a program on the left side of the divided display . under condition 2 , two identical monitors were used in a dual display ( l - company , 20-inch ) , and subjects typed text from a pdf file displayed on the right monitor into a program on the left monitor . under condition 3 , two different monitors were used to construct a dual display ( an l - company 20-inch monitor and an s - company 20-inch monitor ) , and the subjects typed text from a pdf file on the right monitor into a program on the left monitor . for each condition , the workstation included a keyboard and mouse on a table and a swivel chair with five wheels . the resolution of all computer monitors was set to 1,068 1,050 . to allow for lumbar motion analysis , the workstation table and chair could be adjusted for height and were initially set to ensure that the elbows , hips , and knees were flexed at 90. the keyboard and mouse were positioned 30 cm from the trunk , the monitor was reclined by 20 , and the top of the display was set at eye level . each study subject spent 15 minutes typing 300400 words using microsoft word ( microsoft , redmond , wa , usa ) at each of the three workstations . the statistical package for the social sciences ( spss , chicago , il , usa ) the muscle activity of the orbicularis oculi under condition 1 ( 9.8 4.0% ) was significantly decreased compared with that under condition 2 ( 12.6 5.7% ) or 3 ( 15.6 6.3% ) ( p < 0.05 ) . the muscle activity of the orbicularis oculi under condition 3 was significantly increased compared with that under condition 2 ( p < 0.05 ) . in the current study , emg was used to measure orbicularis oculi activity in computer users and evaluate tension in the muscles surrounding the eyes . activity in the muscles around the eyes was increased due to the increased movement of the head and eyes from one monitor to the other , which required constant refocusing and increased pupil action due to the larger screen area6 , 7 . eye blink , a component of the startle response , can be measured by recording the phasic emg activity in the orbicularis oculi . the blink reflex varies with emotional valence ; a greater response is induced by unpleasant stimuli , and the response is increased by stress8 . therefore , a consistent increase in the muscle activity of the orbicularis oculi is closely related to and likely a cause of eye fatigue , headache , and stress in those who use computers for long periods6,7,8 . additionally , the present study found that the activity of the orbicular oculi increased when a dual monitor setup that comprised monitors from different manufacturers was used , compared with that when both monitors were from the same manufacturer . despite using the same resolution , brightness , and contrast settings on the monitors from the different manufacturers , subtle differences in brightness and resolution required the eyes to readjust their focus , which increased the activity of the muscles surrounding the eyes6 , 7 . thus , dual monitors are not recommended , as they increase tension in the muscles surrounding the eye . if a dual monitor setup is necessary , the use of two identical models from the same manufacturer is recommended . a previous study also found that differences in computer workstations were associated with differences in fatigue10 . the present study found that the use of dual monitors increased orbicularis oculi activity ; therefore , to decrease eye fatigue in computer users , computer workstations that use either a single monitor or identical monitors from the same manufacturer in a dual setup , are recommended .
[ purpose ] this study compared the orbicularis oculi muscle activity during computer work with single and dual monitors . [ subjects ] ten computer workers 2227 years of age were included in this study . [ methods ] subjects performed computer work with single or dual monitors , and the activity of the right orbicularis oculi muscle was measured with a mp150 system . [ results ] the muscle activity of the orbicularis oculi under condition 1 was significantly decreased compared with that under conditions 2 or 3 . the muscle activity of the orbicularis oculi under condition 3 was significantly increased compared with that under condition 2 . [ conclusion ] the present study found that the use of dual monitors increased orbicularis oculi activity ; therefore , to decrease eye fatigue in computer users , computer workstations that use either a single monitor , or identical monitors from the same manufacturer in a dual setup , are recommended .
aneurysms of the left main coronary artery ( lmca ) are exceedingly rare clinical entities , encountered incidentally in approximately 0.1% of patients who undergo routine angiography . other causes include trauma , connective tissue disorders , kawasaki disease , vasculitis , congenital , mycotic , and idiopathic . we describe herein a 59-year - old male presenting with effort angina whose coronary angiogram revealed lmca aneurysm . a 43-year - old obese , hypertensive man , presented in emergency department with acute onset of chest pain at rest that radiated to his jaw and left arm associated with profuse sweating . on examination his pulse was 96/minute , blood pressure 190/100 mmhg , respiratory rate-28/minute and temperature -98.4 f. jugular venous pressure was normal . left ventricular apex and upper 1/3 of inter ventricular septum were hypokinetic on echocardiogram with left ventricular ejection fraction of 48% . coronary angiography revealed a fusiform lmca aneurysm originating at the distal segment 45.7 30.6 mm in size [ figures 1 and 2 ] . the patient was advised surgery but he refused and was conservatively managed with dual antiplatelets including aspirin 150 mg and clopidogrel 75 mg and anticoagulants . left coronary angiogram ( rao caudal view ) showing fusiform aneurysm of distal left main coronary artery left coronary angiogram ( lao caudal view ) showing aneurysm of distal segment left main coronary artery coronary artery aneurysm is an uncommon disease defined as coronary dilatation that exceeds the diameter of adjacent normal segments or the diameter of the patient 's largest coronary vessel by 1.5 times . the aneurysm occur most often in the right coronary artery followed in frequency by the circumflex and anterior descending artery . aneurysm of the lmca are extremely rare ; in a study by topaz et al . involving a series of 22,000 coronary angiograms , the etiology of these aneurysms is usually atherosclerotic in adults with a complex interplay between tissue metalloproteinases and the proteolysis of extracellular tissue proteins . increased levels of the 5a allele of mmp3 and mmp9 serve as additional markers promoting this pathogenesis . other causes include trauma , connective tissue disorder - polyarteritis nodosa , systemic lupus erythramatosus , ehler danlos syndrome , behcet disease , scleroderma , marfan 's syndrome ) , kawasaki disease , vasculitis ( takayasu arteritis ) , congenital , mycotic and idiopathic . a recent development is increased incidence of aneurysm in patients who have undergone coronary artery stenting both with drug eluting stents ( des ) and bare metal stents . in des it appears to be secondary to the hypersensitivity reaction to the des polymer predisposing to this condition once the effect of the antiproliferative drugs has worn off . the management of lmca aneurysm is not well established owing to the rarity and unpredictable natural history . however , these dilated sections of artery are not benign entities because they are subject to spasm , thrombosis and spontaneous dissection and can be a potential cause of myocardial infarction . surgical management is appropriate in symptomatic patients who have evidence of emboli from the aneurysm to the distal coronary bed , leading to myocardial ischemia . surgery is also indicated in cases of progressive lmca enlargement as documented by serial angiographic measurement . the preferred approach for surgical correction of lmca aneurysm entails proximal and distal end ligation of the aneurysm with bypass of the lad by means of the left internal mammary artery . placement of a saphenous vein graft to bypass the left circumflex may also be necessary to adequately perfuse the left ventricle . patients not managed surgically must nevertheless be monitored very closely and treated with antiplatelet and anticoagulation therapy to prevent thrombus formation within the aneurysm .
left main coronary artery aneurysm is an uncommon coronary anomaly . we describe herein a male whose coronary angiogram revealed left main coronary artery aneurysm . the purpose of the case report is to highlight the clinical picture , workup , and treatment options for such patients .
it presents as a neuropathic pain syndrome that according to the ihs classification criteria , persists or recurs 3 months after the onset of herpes zoster . it is commonly considered as a neural signal arising at some level within the auditory system . are reported by migraineurs , as are phonophobia and low - frequency hearing [ 36 ] . moreover , it is a well recognized though uncommon component of the ramsay - hunt syndrome . to our knowledge , there are no data in the literature on tinnitus in phn . we describe a 65-year - old woman who was referred to our outpatient unit because of severe head pain and atypical tinnitus . in november 2007 , the patient suffered an attack of high fever ( 39c ) associated with cough , myalgia , anorexia , headache , and gastrointestinal symptoms . the nature of this fever and the cause of the symptoms associated with it were not investigated and remained unknown . some days after recovering , she had an episode of herpes zoster that affected the skin areas innervated by the left ophthalmic division of the fifth cranial nerve , but did not involve her eye . the patient was promptly treated with antiviral therapy ( acyclovir 200 mg every 4 h for 7 days ) and the skin rash healed within 3 weeks . it manifested itself in a variety of ways , including a deep aching pain , sharp intermittent pain , lancinating pain and allodynia . it remained circumscribed to the skin areas innervated by the left ophthalmic division of the fifth cranial nerve . interestingly , this subject developed atypical tinnitus approximately 1 week after the rash resolved . the carotid echo - color - doppler sonography was normal in this subject , as were the cerebral mr and mr angiography . a diary , given to the patient to record both the pain and tinnitus ( scores from 0 to 10 ) , confirmed , as the patient had referred , that the intensity of the tinnitus increased in proportion to the intensity of the pain . two months later , during a follow - up visit , the patient referred that both the pain and tinnitus had progressively improved . in particular , the tinnitus had totally resolved 16 days after the start of therapy , while the pain persisted as a mild , inconstant sensation over the skin area of the left ophthalmic division of the fifth cranial nerve . one week before coming to us for the subsequent follow - up visit in september ( i.e. , 5 months after first coming to our attention ) , the patient decided to reduce the dosage of duloxetine to 30 mg daily . shortly after , however , she once again started taking 60 mg daily after both the pain and tinnitus recurred . in november , when still on the same therapy , the patient referred a further improvement in the pain and the total remission of the tinnitus . the treatment with duloxetine was discontinued in february 2009 , after which neither the pain nor tinnitus recurred . in the last visit , in july , we describe a 65-year - old woman who was referred to our outpatient unit because of severe head pain and atypical tinnitus . in november 2007 , the patient suffered an attack of high fever ( 39c ) associated with cough , myalgia , anorexia , headache , and gastrointestinal symptoms . the nature of this fever and the cause of the symptoms associated with it were not investigated and remained unknown . she had no history of any other significant past medical conditions . some days after recovering , she had an episode of herpes zoster that affected the skin areas innervated by the left ophthalmic division of the fifth cranial nerve , but did not involve her eye . the patient was promptly treated with antiviral therapy ( acyclovir 200 mg every 4 h for 7 days ) and the skin rash healed within 3 weeks . it manifested itself in a variety of ways , including a deep aching pain , sharp intermittent pain , lancinating pain and allodynia . it remained circumscribed to the skin areas innervated by the left ophthalmic division of the fifth cranial nerve . interestingly , this subject developed atypical tinnitus approximately 1 week after the rash resolved . the carotid echo - color - doppler sonography was normal in this subject , as were the cerebral mr and mr angiography . a diary , given to the patient to record both the pain and tinnitus ( scores from 0 to 10 ) , confirmed , as the patient had referred , that the intensity of the tinnitus increased in proportion to the intensity of the pain . two months later , during a follow - up visit , the patient referred that both the pain and tinnitus had progressively improved . in particular , the tinnitus had totally resolved 16 days after the start of therapy , while the pain persisted as a mild , inconstant sensation over the skin area of the left ophthalmic division of the fifth cranial nerve . one week before coming to us for the subsequent follow - up visit in september ( i.e. , 5 months after first coming to our attention ) , the patient decided to reduce the dosage of duloxetine to 30 mg daily . shortly after , however , she once again started taking 60 mg daily after both the pain and tinnitus recurred . in november , when still on the same therapy , the patient referred a further improvement in the pain and the total remission of the tinnitus . the treatment with duloxetine was discontinued in february 2009 , after which neither the pain nor tinnitus recurred . in the last visit , in july , both the pain and tinnitus were still absent . the patient we describe had herpes zoster ophthalmicus on the left side , but did not fortunately develop any ocular involvement , possibly because of the prompt administration of antiviral therapy . the patient , however , developed phn that persisted after the resolution of the rash and consisted of deep aching pain , sharp intermittent pain , lancinating pain , as well as allodynia ( pain induced by non - painful stimuli ) in the regions innervated by the left ophthalmic division of the fifth cranial nerve . the diary kept by the patient revealed that the pain and tinnitus were indeed related , with the intensity of the tinnitus increasing in proportion to the intensity of pain . tinnitus is commonly considered as a neural sign arising at some level within the auditory system . however , some observations point to connections between the somatosensory and auditory systems [ 911 ] . since tinnitus developed together with phn , increased in intensity in proportion to the intensity of the pain , was responsive to phn treatment and disappeared with phn recovery , it may be hypothesized that tinnitus was a symptom associated with the neuropathic pain in this patient . theoretically , tinnitus could be explained as an overexcitation of hearing structures in the same way as allodynia can be an overexcitation of neural structures in the somatosensory system . this interpretation may be supported by the observations of other authors , who described tinnitus in three migraine patients in whom tinnitus occurred during the course of a migraine attack and increased in intensity in proportion to the severity of pain .
we describe a woman who developed postherpetic neuralgia ( phn ) located on the skin areas of the left ophthalmic division of the fifth cranial nerve without ocular involvement . phn was associated with tinnitus , which was located ipsilaterally to the painful side and increased in proportion to the intensity of pain . tinnitus was responsive to treatment with duloxetine , 60 mg daily , and subsided when the phn resolved . this is the first description of tinnitus in phn .
a 58-year - old woman with an eight - year history of hypertension was referred to our institution due to progressive dyspnea . based on clinical symptoms , the new york heart association ( nyha ) class of the patient was class iv heart failure despite ten days of intensive medical management . an echocardiogram revealed a heterogeneous , round , echogenic mass attached to the left atrium and severe functional mitral stenosis , with a mitral diastolic pressure gradient of 10 mmhg , due to a mass protrusion into the left ventricle during diastole phase ( fig . the mass occupied most of the left atrial cavity and extended into the left ventricle through the mitral valve . the mass measuring 864 cm in size was well - circumscribed , friable , lobular , chondroid mass shaped like a bunch of grapes with a pinkish - white rugged surface , and it resembled the eggs of frog . the mass was attached to the left atrial septal wall and protruded into the left ventricle via the mitral valve . therefore , the mitral orifice was almost completely obstructed by this extremely large mass . since the friable , extremely large tumor was diffusely attached to the left atrial septal wall , it was removed in four pieces along with the atrial septal wall . after the mass was completely excised , the defect of the atrial septal wall ( 23 cm ) was closed with patch of dacron ( double velour ) . pathological examination revealed a malignant cartilaginous tumor showing hypercellularity , moderate nuclear atypia , and hyperchromasia . 3 ) . the mass did not demonstrate necrosis or malignant calcification , and its morphology suggested a diagnosis of chondrosarcoma . her postoperative course was unremarkable , and she was discharged 17 days after surgery . at the six - month follow - up , she did not have a residual tumor in the left heart or mitral valve dysfunction , the atrial septal wall defect was successfully repaired , and her cardiac function was classified as new york heart association class i. extra - skeletal chondrosarcomas are rare neoplasms , representing approximately 2% of all soft tissue sarcomas . only about ten reported cases in the english literature , primary cardiac chondrosarcoma in left heart is extremely rare [ 13 ] . primary chondrosarcoma in the left heart often originates from the myocardial tissue and mostly found in left atrium . it is presumed to arise from multipotent mesenchymal stem cells which undergo malignant cartilaginous differentiation . the tumor frequently originates from the endocardium , grows into the atrial or ventricular cavity , infiltration progress through the myocardial wall , extends into the pericardium and mediastinal structures [ 14 ] . systemic metastases are observed in 80% of patients during their initial diagnosis . the lungs and mediastinal lymph nodes are especially susceptible to metastatic deposits . it is suggested that metastatic lesion is often found in right heart or lung . in our case , imaging modalities , including a skeletal survey and gastrointestinal endoscopy did not reveal distant metastasis . the clinical presentation of cardiac chondrosarcomas includes intracavitary obstruction , cardiac tamponade , peripheral embolism , congestive heart failure , and various related symptoms . transthoracic echocardiography remains the diagnostic tool of the choice for the initial evaluation of cardiac masses which can clearly depicts the heart morphology in a variety of imaging planes . computed tomography reveal accurate imaging of the heart and the surrounding mediastinum , and provides better soft - tissue contrast than echocardiography [ 13 ] . however , computed tomography is still far inferior to echocardiography in the depiction of small moving structures such as the cardiac valves . magnetic resonance imaging is adequate for demonstrating the extension of tumors to the interatrial septum and pulmonary veins , as well as beyond the myocardium into the pericardial space with disruption of the pericardium . in most cases , however , histologic confirmation is the definitive diagnosis of primary cardiac malignancy [ 24 ] . in this case , the preliminary diagnosis was myxoma rather than chondrosarcoma , until we obtained the histopathological report after surgery , despite a pre - operative evaluation involving echocardiography and computed tomography . the prognosis of cardiac chondrosarcoma is poor , where survival is generally measured in weeks or months . in most patients with this condition , surgery remains the first line of treatment for cardiac chondrosarcoma , although long - term survival rates after excision are low because of early local recurrence and metastases . surgery is recommended for pathologic confirm but also to relieve the compression caused by the tumor , because partial or complete relief of symptoms may significantly improve the duration of survival and quality of life . chemotherapy and radiotherapy are indicated for malignant cardiac lesions that are not suitable for radical surgery ; however , these therapies have not been demonstrated to result in clinical benefits [ 25 ] . in our case , six months after radical excision , the patient was healthy without local recurrence or metastases . in conclusion , primary chondrosarcoma of the heart is an extremely rare malignancy , which can only be definitively diagnosed after a pathological examination .
chondrosarcoma is a rare entity of malignant tumor which arises from cartilaginous tissue , and the literatures on this disease are scarce . the first - line of treatment for cardiac chondrosarcoma is surgery . due to early local recurrence and distant metastasis , the prognosis is poor even after complete surgical excision . we present a case of chondrosarcoma in the left atrium causing functional mitral stenosis which required urgent surgical intervention , and the successful treatment outcome .
corpora cavernosa lymphomas only appear in the literature as a secondary involvement of a primary lymphoma of the penis . no evidence for any type of primary lymphoma to arise from the corpora cavernosa without penile involvement has been reported so far . because extranodal lymphomas tend to behave aggressively , treatment of diffuse b - cell lymphomas is usually rituximab - based chemotherapy with local excision when possible . clinical presentation of perineal masses are not characteristic ; therefore diagnosis is often performed too late . a 65-year - old man with pathological history of peripheral vasculopathy , leriche syndrome , and bearing a cardiac pacemaker , presented a 3-month history of dysuria , weak urinary flow , progressive voiding discomfort , and perineal pain , when a perineal swelling that was slowly growing was detected . physical examination showed normal external genitalia and a painful 4-cm perineoescrotal mass in contact with the bulbar urethra . voiding cistourethrography showed a slight narrowing of the diameter of the bulbar urethra , but not a significant urethral stricture , suggesting an external compression of the urethra . psa blood level was 0.56 ng / ml , erythrocyte sedimentation rate was 21 sec , and urinary culture was negative . the histopathological examination of a core biopsy showed a diffuse , dense infiltrate of atypical large lymphoid cells with frequent mitotic figures [ figure 1 ] . immunohistochemical studies showed the cells to be positive for b - cell marker ( cd20 ) and negative for t - cell markers ( cd3 and cd5 ) , cd10 , and bcl6 . the proliferative index ( ki 67 ) was 70% . based on these findings , a diagnosis of diffuse diffuse infiltration of large , atypical lymphoid cells ( h and e stain , 400 ) , strongly positive for cd20 ( inset , 400 ) a thoracic - abdomino - pelvic computer tomography ( ct ) was prescribed in order to search for a primary nodal origin of the lymphoma . findings of the ct were as follows : absence of mesenteric , retroperitoneal , or pelvic significant nodes , at the ct sagital section , the anatomy of corpora cavernosa is replaced by a heterogeneous 6 4.5 cm mass [ figure 2 ] . sagital tc view : the anatomy of corpora cavernosa is replaced by a heterogeneous 6 4.5 cm mass in conclusion , the tumor was classified as ie stage diffuse b - cell lymphoma ( single , extra - nodal , without b symptoms according to the ann arbor classification ) . a rituximab - combined chemotherapy r - chop ( cyclophosphamide , adryamicine , vincristine , and prednisolone ) was prescribed , to be administrated every 3 weeks for a total of six cycles . although the patient showed good clinical response , he died of cardiac toxicity after 8 months ( six cycles of chemotherapy ) . only one case of perineal reticulum cell sarcoma can be found in the literature , and it was described as a perineal mass with priapism . malignancies of the corpora cavernosa often appear as a primary involvement of a tumour of the shaft of the penis , frequently advanced squamous carcinoma , rarely lymphomas . the common presentation in those cases is a palpable nodular mass in the shaft of the penis . both advanced bladder and prostate cancer however , the clinical presentation in these cases is malignant priapism . as far as the lymphomas of the low urinary tract and genitalia are concerned , the testis is the most common site of lymphomatous involvement of the male genitalia . classical treatment of extranodal large b - cell lymphomas is rituximab - combined chemotherapy r - chop , and 40004400 rads radiation is added in selected cases . in extranodal primary affection , surgical excision should be performed in small lesions when these are reachable , but radical surgery and amputations should be avoided in low urinary tract lymphomas . in the case presented here , surgical excision was not possible . the location and extension of the tumor conditioned nonsurgical treatment . radiation therapy was envisaged , but the length of the mass invited us to reserve radiation for the residual tumor after chemotherapy . however , heart toxicity of chemotherapy led to the patient 's death 8 months after the diagnosis . the origin primary diffuse large b - cell lymphoma of the corpora cavernosa is uncertain . because there is no lymphoid tissue in the corpora cavernosa , it may be initially considered as a primary lymphoma . magnetic resonance is the best radiological exploration in those cases , because it provides optimum information about tumor stage and infiltration of neighbor organs . the absence of priapism in this case might be due to a severe peripheral vasculopathy and the leriche syndrome . other primary origin of this tumor could be considered , such as the prostate , the urethra , or the rectum . compression of bulbar urethra showed up as low urinary tract syndrome , simulating a prostatic obstruction . prostatic and rectal diseases were excluded by digital rectal examination , ultrasonography , and psa . to our knowledge , this is the first case of lymphoma of the corpora cavernosa without penile involvement described in the medical literature . the relevance of this case resides in its exceptionality , its clinical presentation simulating a urethral stricture , the need of biopsy to provide an accurate the diagnosis , and the potential toxicity of the aggressive chemotherapy treatment used in these cases of lymphomas in aged population .
primary male genital lymphomas may appear rarely in testis , and exceptionally in the penis and prostate , but there is not previous evidence of a lymphoma arising from the corpora cavernosa . we report the first case in the literature of a primary diffuse cell b lymphoma of the corpora cavernosa presented with low urinary tract symptoms , perineal pain and palpable mass . diagnosis was based on trucut biopsy , histopathological studies and computed tomographic images .
pseudotumor cerebri ( ptc ) or idiopathic intracranial hypertension ( iih ) is a disorder of elevated intracranial pressure ( icp ) without any evidence of infection , vascular abnormality , space occupying lesion , hydrocephalus or alteration of consciousness . all - trans - retinoic acid ( atra ) has been widely used in the treatment of acute promyelocytic leukemia ( apl ) . cases of pseudotumor cerebri associated with all - trans - retinoic acid treatment in acute promyelocytic leukemia ( apl ) have been frequently described in pediatric patients . a 25-year - old female patient presented to our clinic with fever and bleeding gums of three weeks duration . hematological investigations showed hemoglobin of 10.7 g / dl , platelet count 200000/mm ; white blood cell count of 2900/mm , differential count 30% blast cells , 6% neutrophils , 64% lymphocytes . serum biochemistry showed lactate dehydrogenase 566 u / l , peripheral smear showed normocytic normochromic anemia , reduced total count with predominant lymphocytes with atypical cells and adequate platelet count . bone marrow examination showed 84% blasts which were peroxidase positive with moderate cytoplasm indented to bilobed nuclei with immature chromatin . m3 with cd13 , cd33 , cd64 , cd117 , cd45 positivity and negative hla dr . real time pcr ( polymerase chain reaction ) qualitative analysis revealed the presence of typical fusion transcript pml - rara ( bcr1type ) in the cells . chromosomal analysis carried out using bone marrow showed 50% with 46xx ; t ( 15;17 ) and 50% with 46xx . detection of translocation t ( 15;17 ) by fish using bone marrow showed pml - rara fusion signal in 90% of interphase cells . ( 80 mg / day ) plus daunorubicin 100 mg / day for four days . after three weeks of induction chemotherapy , the patient started complaining of headache and diplopia . cerebro spinal fluid ( csf ) analysis was normal except for an elevated csf pressure . atra was stopped and the patient was started on anti cerebral edema measures and steroids . the patient achieved complete remission ( cr ) after induction chemotherapy without any neurological sequela . all - trans retinoic acid ( atra ) is used as differentiation therapy for acute promyelocytic leukemia ( apl ) . even though , atra is a well tolerated drug , some patients may develop complications like dry skin , retinoic acid syndrome , hypertriglyceridemia , sweet 's syndrome , hyperleukocytosis , and rarely myositis . the mechanism of neurotoxicity is thought to be similar to the pathogenesis of hypervitaminosis a. at higher doses of atra , retinoids enhances the production of csf and alters the lipid constituents of arachnoid villi , disrupting the normal transport system and impending the absorption of csf at arachnoid villi . warner et al . reported that csf retinol levels were higher in patients with idiopathic intracranial hypertension ( iih ) than in the subjects without iih . a progressive age - related reduction of rar expression in the central nervous system has also been postulated as a reason for less chance of ptc in the adult apl compare to the children . even though hallmark of ptc or iih is papilledema mr imaging of the optic nerves and pituitary gland may provide important clues like flattening of the posterior sclera , vertical tortuosity and elongation of the optic nerve , distension of the perioptic subarachnoid space , compressed pituitary gland or empty sella . table 1 shows the details of previously reported cases of ptc after atra for apml in the literature . most cases were reported during the early 2 - 3 weeks of initiation of atra . this case highlights the possibility of pseudotumor cerebri while on therapy with atra even in the adult apml . a strong clinical judgment is necessary to stop atra at the onset of neurotoxicity and reinstitute atra at the right time to attain clinical remission .
all - trans - retinoic acid is an integral part in the treatment strategy of acute promyelocytic leukemia ( apl ) . here we describe a case of pseudotumor cerebri associated with all - trans - retinoic acid ( atra ) during the induction therapy in an adult with acute promyelocytic leukemia ( apl ) .
serratia marcescens keratitis is commonly found in patients who have an abnormal ocular surface and wear contact lenses . the clinical presentation is characterized by diffuse haziness of the corneal epithelium and central or peripheral infiltrates with hypopyon . corneal ring infiltrate is an uncommon manifestation of s. marcescens keratitis but may be found in various circumstances such as pseudomonas , herpetic , acanthamoeba , or fungal keratitis . we report a human immunodeficiency virus-1 ( hiv-1)-infected patient who had a history of contact lens wear and developed ring infiltrate keratitis . a 29-year - old male presented at the cornea clinic , complaining of redness , pain , watering , and photophobia in the left eye for 3 days . he had been using disposable daily wear contact lenses for myopic correction for 2 years duration . the patient reported that 2 days ago he slept with his contact lenses overnight and woke up with purulent discharge in his left eye . his cd4 t lymphocyte count was 155 cells/l , currently being treated with highly active antiretroviral therapy . he had been using chloramphenicol eye drops , which he bought from a pharmacy for 1 day . slit - lamp examination showed a round diffuse area of subepithelial to midstromal infiltration with immune ring in the left cornea ( fig . the microscopic workup from corneal specimens revealed numerous gram - negative bacilli in gram stain ( fig . 2 ) and the culture result was s. marcescens sensitive to gentamicin , vancomycin , and amikacin . the treatment was started using fortified gentamicin ( 14 mg / ml ) eye drops to the left eye hourly . s. marcescens was first described by bizio in 1823 as a cause of red discoloration of cornmeal porridge ( bleeding polenta ) . this organism can cause purulent conjunctivitis , keratitis , scleritis , and endophthalmitis [ 5 , 6 ] . contact lens wear is a risk factor that predisposes patients to the development of infectious keratitis . in thailand overnight wear of a soft contact lens is a well - recognized risk factor for keratitis . s. marcescens is commonly found in the damp environment such as the bathroom where our patient kept the contact lens his decreased immune status with contact lens misuse had made him prone to the development of serratia keratitis . hiv patients have also been associated with abnormal ocular surface abnormality and dry eye ; therefore , they may be predisposed to ocular serratia infection . the corneal lesion seen in this patient was a stromal infiltration with an immune ring . this ring infiltrate , presumably the result of an immunologic response such as antigen - antibody complexes , complement , and polymorphonuclear leukocytes , can be seen in patients with severe corneal burns , corneal foreign bodies , pseudomonas , herpetic , acanthamoeba , or fungal keratitis , and abuse of topical anesthetic agents . nevertheless , we demonstrated that this immune ring is consistent with another presentation of serratia keratitis in the contact lens - wearing patient who had seropositive hiv . their appearance in this patient may have been modified by partial treatment or by his immunodeficiency status . however , the appearance of the corneal ring improved after treatment for 2 weeks . our findings showed that s. marcescens keratitis can present with ring infiltrate in a patient who wears contact lenses and suffers from hiv-1 infection .
purposeto describe corneal ring infiltrates caused by serratia marcescens in a patient with human immunodeficiency virus ( hiv-1 ) who wore contact lenses.methodsa case study of a patient with keratitis due to an infection caused by s. marcescens and exhibiting corneal ring infiltrates was reviewed for history , clinical manifestation , microscopic study , and management.resultsa 29-year - old man who had a history of contact lens wear and hiv-1 infection was admitted to hospital because of blurred vision , redness , and corneal infiltrates in the shape of a ring in the left eye . the visual acuity ( va ) in both eyes was hand movement ( uncorrected ) . corneal scrapings were performed . the culture results of the corneal specimens revealed s. marcescens . the culture results of the contact lens disclosed the same organism . the corneal ulcer responded well to treatment with topical gentamycin sulfate 14 mg / ml . the final va remained hand movement.conclusionss . marcescens can cause ring infiltrates in a hiv-1 patient who wears contact lenses . the treatment result for s. marcescens keratitis in a hiv-1 patient who wore contact lenses was favorable after intensive use of fortified topical antibiotics .
this 34-year - old male patient had an impacted mytilus coruscus shell in the esophagus . , the patients received a chest ct after contrast enhancement using a toshiba aquillion operating at 120 kvp and 62 mas , with a slice thickness of 3 mm ( fig . 1 ) . this 52-year - old male patient had a fish bone lodged in the espophagus . prior to fb removal , the patient received a chest ct after contrast enhancement using a siemens navigator operating at 120 kvp and 90 mas , with a slice thickness of 2 mm ( fig . the axial ct data were saved in dicom file format . to reconstruct the 3d images , the outer surfaces of the fb , skin , bones including vertebrae , inner surface of the trachea , and esophagus were drawn in all frames after detection of the outlines . the 3d images and photographs were compared with respect to the shape and location of each fb in the esophagus . after rotation of the 3d images , they looked similar in shape and orientation to the photographs of the fbs that had been removed . this 34-year - old male patient had an impacted mytilus coruscus shell in the esophagus . , the patients received a chest ct after contrast enhancement using a toshiba aquillion operating at 120 kvp and 62 mas , with a slice thickness of 3 mm ( fig . the fish bone was removed by open thoracotomy under general anesthesia . prior to fb removal , the patient received a chest ct after contrast enhancement using a siemens navigator operating at 120 kvp and 90 mas , with a slice thickness of 2 mm ( fig . the axial ct data were saved in dicom file format . to reconstruct the 3d images , the outer surfaces of the fb , skin , bones including vertebrae , inner surface of the trachea , and esophagus were drawn in all frames after detection of the outlines . the 3d images and photographs were compared with respect to the shape and location of each fb in the esophagus . after rotation of the 3d images , they looked similar in shape and orientation to the photographs of the fbs that had been removed . although the flexible fiberoptic endoscope is a very popular tool for fb extraction , the rigid esophagoscope is equally safe and represents a good tool in the hands of an experienced surgeon . if endoscopy fails , an open thoracotomy is the method of last resort . in asian countries , since fish bones and shells tend to be calcium - rich , they are visible upon routine x - ray examination , if sufficiently large in size . use of a flexible fiberoptic endoscope can often allow both a definitive diagnosis and extraction of the fb . in the present cases , prompt extraction of the rigid and sharp - edged fbs is necessary , since they can cause erosion and laceration of the esophageal mucosa if left untreated . endoscopic extraction is not risk - free , and problems that arise can demand emergency surgery . rapid knowledge of an esophageal fb , specifically , its composition ( i.e. , hard or soft ) , shape , size , location , and orientation , is vital for a surgeon in tailoring the safest and most effective treatment . our patients were successfully treated under endoscopy or by thoracotomy with full knowledge about the fb . at the time of the treatment the 3d reconstructions were made retrospectively , as was the comparison of these image reconstructions with actual photographs . the time required for making the reconstructed image was less than 30 minutes once the chest ct had been acquired , it took even less time when skilled personnel was involved . this shows another example of the practical value of ct reconstruction from the clinical point of view . therefore , while the present data was not instrumental in the treatment of the patients from which the data was drawn , the potential of ct reconstruction for future cases was confirmed . in the case of the shell fb , photographs of the shell that was removed were taken without inclusion of a ruler and the images were distorted due to endoscope angles , so measurements were not possible . for this fb , the 3d reconstructed images were helpful in determining the shape , size , and the relative location of the shell , and its orientation in the esophagus . rotation of the 3d images could be performed to mimic the endoscopic images and to be used in simulation of surgical procedures if needed . as a result , the patient was required to fast for 7 days until a second endoscopy confirmed healing . in this patient , real photographs and 3d images were available and were used for comparisons of fb size , location ( in the 3d image only ) , and orientation . the shape in the 3d images was very similar to that in the real fb photograph . the 3d image was rotated to match the endoscopic view of the fb ; the accuracy of the result indicates the potential of the approach as a simulation method . information concerning the distance from a target point to the fb is available with one mouse click . since ct is calibrated data , the 3d images can always be used for measuring distance . a 3d reconstructed image from ct data can be converted into a real touchable physical model through rapid prototype ( rp ) technology . before operations for cardiac anomaly or bone - destroying cancer surgery , the use of rp can generate a surgical simulation or allow a bone defect to be measured to make implants or flaps . patients with a vessel anomaly , vascular tumor , or stabbing injury can also benefit from the 3d reconstruction technique . organs with hollow spaces occupied by fluid or air are the best candidates for virtual endoscopy . the technique has been adapted in the screening for polyps or other space - occupying lesions of the large intestine . virtual endoscopy has not been attempted frequently for esophagus procedures , since flexible fiberoptic endoscopy is quick and convenient . in contrast , virtual esophagoscopy requires pretreatment with a gas - forming formula and motility - decreasing injections that distend the esophageal lumen to allow full examination of the esophagus . the gas - forming medicine might not be suitable for patients with an esophageal fb because of the danger of leakage of the medicine through damaged esophageal mucosa . until now , virtual endoscopy has not been tried in patients with esophageal fb . the reconstructions were able to accurately indicate the shape , size , location , and orientation of the fb in the esophagus in two patients . the type of material comprising an fb can not be determined in the absence of information concerning the ingested material . on the other hand , endoscopy is superior to 3d reconstruction in identifying the composition of an fb . the information in a 3d reconstructed image can be quickly converted into an rp , which can be used to simulate an extraction procedure such as flexible fiberoptic endoscopy or open surgery . finally , the present ct data was acquired in 2- or 3-mm - thick slices . use of slices of 1 mm thickness or less would provide finer detail , and the resulting 3d reconstruction would even more realistically mimic the fb .
this study was conducted to investigate the clinical application of three - dimensional ( 3d ) reconstructed computed tomography ( ct ) images in detecting and gaining information on esophageal foreign bodies ( fbs ) . two patients with esophageal fbs were enrolled for analysis . in both cases , 3d reconstructed images were compared with the fb that was removed according to the object shape , size , location , and orientation in the esophagus . the results indicate the usefulness of conversion of ct data to 3d images to help in diagnosis and treatment . use of 3d images prior to treatment allows for rapid prototyping and surgery simulation .
ventriculoperitoneal shunt is a commonly performed surgical procedure and offers good results in treatment of hydrocephalus . various complications have been reported , including shunt infection , migration , and cerebrospinal fluid pseudocyst . the migration of the peritoneal catheter can occur into the abdominal wall , git , bladder , vagina , scrotum , and mediastinum . . the rates of reported complications range from 24 to 47% and about 25% of these are abdominal complications . however , despite its easy installation , it may lead to few serious and rare complications which warrant early recognition to avoid morbidity and mortality . intestinal perforation by the distal end of ventriculoperitoneal shunt is a rare complication with incidence of 0.1 - 0.7% . young patients are commonly affected due to weak intestinal wall musculature which can be perforated easily by the hard tipped distal end of the tube . removal of the catheter by a less invasive method is advisable in the absence of infection . a 6-year - old male child came with a complaint of left focal seizures with 10 days history . the child was evaluated with a ct scan and mri and was diagnosed as right frontoparietal oligodendroglioma . the histopatholgical examination is suggestive of oligodendroglioma grade iii . in the same course of hospitalization , the child developed hydrocephalus and managed with ventriculoperitoneal shunt . the clinical and imageological conditions of the child improved after the shunt and he was discharged with advice of postoperative radiotherapy . after 4 months of surgery , the child presented with a complaint of protrusion of a shunt tube through anus with a history of 15 days on and off . on examination , the child was evaluated with an x - ray of the abdomen [ figure 2 ] and sigmoidoscopy which shows coiling of the tube in the descending colon and sigmoid colon with perforation [ figure 3 ] . the ct scan of the brain showed normal size of ventricles and no pneumocephalus and recurrence of tumor [ figure 4 ] . plain x - ray abdomen with coiling of shunt tube in descending and sigmoid colon clinical photograph of the patient with anal extrusion of a shunt tube sigmoidoscopy view showing perforation of colon ct scan of the brain showing a shunt tube in a normal sized ventricle we managed the case with separation of the proximal portion of the shunt tube from the distal portion and it was exteriorized and external ventricular drainage was connected and cerebrospinal fluid was sent for analysis . laboratory examination of blood showed that total leukocyte count was 9200 mm and in differential count neutrophils-62 , lymphocy tes-30 and eosinophils-8 . cerebrospinal fluid examination showed nil leukocyte count and sugar level was 40 mg /dl , proteins 219 mg / dl , and chloride 123 meq / dl . sigmoidoscopy found that ventriculoperitoneal shunt 's distal end was perforating and migrating into the descending and sigmoid colon and it was removed endoscopically . the patient was kept nil by mouth for 48 h being on ryles tube aspiration and intravenous supplementation . external ventricular drainage and ventricular end of the shunt tube was removed after 2 days and the patient was observed for 5 days for any development of peritonitis and raised intracranial pressure features . the patient was doing well without shunt , and ct scan of the brain suggested normal ventricular size , and patient was discharged . ventriculoperitoneal shunting is most commonly indicated surgical treatment of hydrocephalus ; however , the complication rate is relatively high . mostly due to infection , mechanical obstruction failure of csf flow there are a few reports of complications that include the perforation of visceral organs mostly gastrointestinal organs . the reported incidence of bowel perforation by ventriculoperitoneal shunt is 0.1 - 0.7% with mortality rate as high as 15% . complication like these often occur after a long period following surgery , suggesting that they resulted from a chronic inflammatory process rather than a traumatic event . it has been suggested that analyzed all reports related to these complications published in 84 articles and it has been also suggested that catheter length and the presence of fibrosis around the distal end of the catheter are important factors . an interaction between a silicon tube material of the shunt tube , csf contents ( particularly proteins and glucose ) and foremost immunomediators mediated in inflammatory response to infection in the peritoneum have also been proposed and published . nishijima et al . suggested two reasons for the frequent occurrence of intestinal perforation caused by the distal end of the shunt tube . firstly , the intestinal musculature is relatively weaker in children , and is , therefore , perforated easily by hard tip of the catheter . secondly , the children have stronger peristaltic activity than in older patients , which makes them vulnerable to this type of injury . it has been observed that intestinal perforation due to shunt cathetriazation can be related to a previously undergone abdominal surgery and prior abdominal infection . davidson et al . reported that malnutrition and a former abdominal surgery may increase the risk of migration due to adhesions arised from infection . however , the exact pathogenesis of shunt tube - related organ perforation is unclear , and various mechanisms have been suggested including foreign body reaction , pressure necrosis , poor general condition with weak bowel musculature , and previous inflammations to the bowel wall that may contribute to perforation.[410 ] in our case the predisposing cause can be malnutrition enhanced chronic irritation that might have weakened bowel wall that lead to perforation . in view of the high mortality associated with if the catheter left inside , then ascending infection can cause ventriculitis , meningitis , and finally sepsis by migrating intestinal flora through the catheter and its sheath , which are the possible developments . in view of this , paying attention in early diagnosis of this complication is advisable . prompt attention is advisable to recognize this complication and endoscopic removal of the shunt tube is advocated in the absence of infection to avoid major operative procedures and related morbidity and mortality .
we describe the extrusion of a ventriculo peritoneal shunt tube from the anus into a 6-year - old boy following the placement of a ventriculo peritoneal shunt for postoperative periventricular tumor - induced hydrocephalus . he was admitted with a complaint of extrusion of a tube through anus on and off during bowel evacuation . he was evaluated with an x - ray of the abdomen which was showing a coiled tube in descending and sigmoid colon , confirmed by sigmoidoscopy . the proximal end was exposed for external ventricular drainage and distal end was removed endoscopically . the patient was watched for peritonitis and managed conservatively . the proximal end of the tube was removed after 5 days of external ventricular drainage and after ruling out ventriculitis and meningitis . bowel perforation by a ventriculo peritoneal tube is a rare complication . diagnosis is often difficult and delayed . most of the bowel perforation is seen in young patients .
arachnoid cysts in the brain usually have an indolent course unless complicated by headache , seizures , increasing head circumference , behavioral disturbances , ocular , motor , speech disorders , and sudden cyst changes such as acute cyst expansion , sudden hemorrhage into the cyst , subdural hematoma , or subdural hygroma . rupture of arachnoid cyst causing subdural hygroma is very rare , with few case reports . we herein present a clinical case , radiology , and discussion of asymptomatic middle cranial fossa arachnoid cyst in a 15-year - old male child who presented with raised intracranial features following a trivial trauma . a 15-year - old male child presented with complaints of headache , visual blurring , and projectile vomiting for 20 days duration . the child had a history of trivial fall about 10 days prior to onset of headache , with no loss of consciousness . on examination , the child had bilateral florid papilledema and right lateral rectus palsy . there were no other focal deficits or signs of meningeal irritation . computed tomography ( ct ) scan of the brain showed a left middle fossa , galassi type 3 arachnoid cyst , with bilateral subdural hygroma / hematoma ( chronic ) , bilateral diffuse cerebral edema , and mass effect causing compression of both frontal horns [ figure 1 ] . magnetic resonance imaging ( mri ) of the brain showed bilateral collection in the subdural space , hypo on t1w [ figure 2 ] and hyper on t2w [ figure 3 ] images , matching with the intensities of cerebrospinal fluid ( csf ) with widened sylvian fissure on the left side and a compressed temporal lobe on the left side , suggestive of arachnoid cyst with subdural hygroma and mass effect . computed tomography scan plain , axial section showing hypodense region compressing the temporal horn with bilateral subdural hygroma magnetic resonance imaging brain t1w image showing subdural hypointensity in the temporal region and bilateral convexities compressing temporal and frontal lobes on the left side suggestive of arachnoid cyst with subdural hygroma and mass effect magnetic resonance imaging brain t2w image showing subdural hyperintensity in the temporal region and bilateral convexities compressing temporal and frontal lobes on the left side suggestive of arachnoid cyst with subdural hygroma and mass effect left pterional craniotomy , evacuation of hygroma , fenestration of cyst into suprasellar cistern , and marsupialisation of the cyst was performed . the patient developed pseudomeningocele , which was managed with lumbar csf drainage for 5 days and was discharged without any deficits . the postoperative imaging showed resolution of the subdural hygroma with small extradural and subgaleal collection of the csf [ figure 4 ] . the postoperative imaging showed resolution of subdural hygroma with small extradural and subgaleal collection of cerebrospinal fluid arachnoid cysts are considered intra - arachnoidal in location and account for 1% of the intracranial mass lesions . they can develop anywhere in the cerebrospinal axis but have a predilection toward the middle cranial base . they are usually asymptomatic , but may present with raised intracranial pressure , focal neurologic deficits , or seizures . being indolent and slowly growing , most of the arachnoid cysts can be managed conservatively , reserving surgical intervention for symptomatic lesions . intra - cystic hemorrhage and subdural rupture of the veins running over the surface of the cyst are well described . subdural rupture of the arachnoid cyst per se,[46 ] either traumatic or spontaneous , is sparingly reported with about 21 cases documented in literature . even a minor trauma can cause rupture of the cyst as seen in the present case , where the patient fell down while playing , without any loss of consciousness . the gradual seepage of the csf from the cyst into the subdural space , probably through a flap- valve effect , caused a gradual rise in the intracranial pressure . ruptures are usually asymptomatic in areas other than the cysts in the middle cranial fossa . however , immediate operative intervention was warranted in view of raised intracranial pressure and progressive neurologic deterioration .
intracranial arachnoid cysts developing in relation to the cerebral hemispheres and middle cranial fossa are usually incidental or asymptomatic . however , most of the clinically active cysts present with seizures because of chronic compression . presentation as raised intracranial pressure due to cyst rupture into the subdural space is a rare clinical entity . we herein present a case of an asymptomatic arachnoid cyst with rupture into the subdural space bilaterally and presenting as raised intracranial pressure .
tetanus is a disease characterized by hypertonia and muscular spasms due to the toxin tetanospasmin formed by clostridium tetani . tetanus continues to be widespread , especially in the developing world though incidences have come down due to immunization . we present a case of tetanus in a young girl which initially mimicked a striatal toe though the potential cause of inoculation was trauma to nose . a 16-year - old girl presented with a history of sudden onset of pain in left leg with difficulty in walking for 2 days . an upturned big toe without fanning of other toes was seen on the left [ figure 1 ] . a clinical diagnosis of a striatal toe was made and the patient was admitted for further evaluation [ figures 1 and 2 ] . contrast - enhanced magnetic resonance imaging of the brain revealed no abnormality of the basal ganglia . liver function test was normal and slit lamp examination did not show a kf ring . corrected serum calcium was 9.8 mg / dl . on the 3 day after admission , she developed stiffness of bilateral lower limbs followed by episodic spasms of the back and neck . there were no signs of autonomic hyperactivity and she did not have any seizures . on further probing , patient 's mother revealed that she had suffered a fall a day before onset of her symptoms and hurt her nose . her mother said that she had received her primary series of tt in infancy and received her last booster 6 years back at around 10 years of age . she was given tetanus immunoglobulin ( tig ) 5000 units intramuscularly stat and also started on intravenous metronidazole and diazepam infusion . on the 5 day after admission , she developed mild trismus and opisthotonus completing the clinical picture of generalized tetanus . she started showing signs of recovery by day 10 and made a complete recovery in 3 weeks from onset of symptoms . tetanus is caused by the toxin tetanospasmin released by the germinating spores of the anaerobic gram - positive bacilli c. tetani . as spores of the bacilli occur in the soil , tetanus frequently occurs following a contaminated wound , but cases have also been reported after dental procedures , surgeries , burns , intravenous drug abuse , and intramuscular injections . our patient presented with a localized form of tetanus affecting initially the left leg that mimicked a striatal toe even though there was no local injury in the leg . it is possible that our patient had another insignificant trauma to her leg that had gone unnoticed . a striatal toe is a finding in clinical neurology that reliably localized the lesion to the caudate nucleus and putamen . the absence of fanning of the rest of the toes differentiates the upturned big toe from the babinski extensor plantar response . tetanus presenting initially with the classic clinical picture of striatal toe has not been reported before . thus , it is important to keep localized tetanus in the differential diagnosis of extrapyramidal disorders presenting with such spasticity , especially in the absence of definite history of trauma . dystonias can be ruled out in such cases by checking for resolution after a dose of benztropine . severity of tetanus can be graded according to the ablett classification into four grades . according to the ablett classification , the world health organization recommends a 5 dose schedule for tetanus vaccination while in india additional doses are recommended in the universal immunization program ( uip ) . our patient had received three doses as dtp in infancy and another dose between 12 and 24 months of age . she also had received two booster doses of dtp and td at 5 years and 10 years according to her mother as per the uip . the advisory committee on immunization practices of the centers for disease control does not recommend either td vaccination or tig for patients who have received more than three doses of tt with the last dose within 5 years . td is recommended in clean wounds with last booster > 10 years back and other wounds with last booster > 5 years back . tig should be given in unclean wounds when vaccination status is unknown or patient has received <3 doses . tetanus prone wounds are > 6 h old , > 1 cm deep , stellate , ischemic , denervated , contaminated or infected . our patient had received tt immunization at the time of primary wound care as per the recommendations . she did not receive tig as she had received more than 3 doses of immunization in the past . identification of vulnerable patients by testing for protective tetanus antibodies is not viable in our setting . this may be due to a defect in the patients humoral immunity due to which she could nt mount an antibody response . rapid bedside tests to screen for tetanus immunization status have been found to be more effective than medical interview and wound assessment in some studies , but cost effectiveness remains an issue . wound care , antimicrobial therapy with metronidazole and human tig are cornerstones of therapy of established tetanus . a full course of active immunization with tt should be started early as neither clinical infection nor tig provide long - term immunity . cases of tetanus continue to come to medical attention in spite of widespread immunization in this age and time . we must be alert to the possibility and institute prompt treatment after a clinical diagnosis as early passive immunization reduces morbidity and mortality .
we report the case of a 15-year - old girl who was initially diagnosed to have a striatal toe . her condition progressed and she later developed clinical features consistent with tetanus . history of blunt trauma to nose was elicited retrospectively . antimicrobial therapy with metronidazole and both active and passive immunization was started immediately . the patient went on to make a complete recovery .
stridor due to vocal cord ( vc ) palsy is an alarming complication , albeit infrequent , of congenital neuraxial anomalies , particularly arnold chiari malformations ( acm ) ; meningomyelocele , encephalocele , and hydrocephalus may also coexist with the acm . timely neurosurgery can relieve the stridor completely , but not necessarily immediately , placing the affected children at risk of postextubation respiratory distress ; decision regarding the optimal timing and method of tracheal extubation in them is hence vital . however , unlike intubation , extubation issues in pediatric neuraxial anomalies are neglected and barely discussed . existing extubation guidelines for a known difficult airway largely address adults , and fewer airway - assist devices suitable for infants and neonates are available for routine use , especially in a resource - limited country like ours . airway management in such children is thus often individualized as is highlighted by the following case description . a 2-month - old male child presented with a 3-day - old history of breathing difficulty and high - pitched inspiratory stridor . he had a large head ( 42 cm circumference ) , occipital swelling ( 12 cm 15 cm size ) , tachycardia , tachypnea , nasal flaring , sternal and intercostal retraction , and 90% oxygen saturation ( spo2 ) . x - ray chest was normal , and magnetic resonance imaging brain revealed hydrocephalus , occipital encephalocele , acm type iii , and visible traction on the brainstem [ figure 1 ] . otolaryngology evaluation of vc using a flexible fiberoptic bronchoscope ( ffb ) revealed bilateral abductor vc paresis . the child was taken up for encephalocele repair and ventriculoperitoneal shunt insertion after administration of oxygen and steroids . anesthesia was induced in the lateral position with sevoflurane and oxygen , and tracheal intubation achieved during spontaneous breathing in two attempts . the surgery was uneventful , and at the end , tracheal extubation was done following adequate anesthetic recovery ; an infant ffb was not available to us . the stridor reappeared postoperatively causing strenuous breathing and hypoxia ( spo2 82% ) that eventually necessitated reintubation . direct laryngoscopy ( dl ) revealed impaired vc movements like before but no laryngeal edema . ffb - assisted tracheal extubation was attempted after 72 hours with the otolaryngologist present ; the endotracheal tube ( ett ) was pulled out over the ffb placed prior in the trachea and completely removed only after ensuring satisfactory breathing . magnetic resonance imaging brain ( sagittal section ) showing midline occipital encephalocele , tractional beaking of pontomedullary region of brainstem ( arrow ) and dysplastic cerebellum stridor develops in nearly one - third of children with coexistent acm type ii , lumbar meningomyelocele , and hydrocephalus ; bilateral abductor vc palsy is the cause in up to 12.5% of acm patients . the palsy is mostly secondary to brainstem herniation with traction on vagal fibers that innervate vc abductor muscles ; brainstem dysgenesis or ischemia and cranial nerve nuclei hypoplasia are infrequent causes . the brainstem traction can be completely relieved by early , simple correction of the encephalocele and hydrocephalus , thereby avoiding complicated craniovertebral decompression procedures for acm in small children . vc palsy may not resolve once irreversible brainstem damage occurs , necessitating prolonged airway support with tracheostomy , arytenoidectomy , or vc lateralization procedures . the latter may be problematic when a large occipital encephalocele and an over - sized hydrocephalic head accompany the stridor as in this case . intubation in the lateral position , intubation on a raised platform with foam cushions , and intubation with the head off the table edge are some of the intubation modalities described for such cases . the only available report focusing on airway management in acm - associated vc palsy emphasizes on maintaining spontaneous breathing during intubation and readiness with various intubation aids , emergency tracheostomy facilities , and trained personnel . tracheal extubation concerns , such as when and how to safely remove the ett , are also vital here . although delaying extubation is otherwise not unusual in a neurosurgical scenario , it is not routinely practiced in children with neuraxial defects where the airway difficulty is expected to resolve following surgery . on - table extubation is mentioned in two earlier reports , but a clear policy on this issue has not emerged due to dearth of reported experience . safe extubation in a difficult airway scenario is best achieved by adopting a strategic , step - wise approach , and various guidelines to this effect have been proposed . these however pertain only to adults and there are no specific guidelines to direct extubation in pediatric difficult airways . further , most airway aids that form an integral part of the extubation strategy in adults have limited routine use in children due to nonavailability of smaller sizes , inexperienced handling , and insufficient supporting literature . determining airway competence before extubation is ideal , but not easy . presence of the ett hinders clear vc visualization by dl or indirect laryngoscopy , while the cuff leak test is an insensitive predictor of extubation outcome in children . use of a ffb with a laryngeal mask airway ( lma ) is advocated for assessing preextubation vc status and also facilitating extubation and reintubation in adults ; however , lesser availability of smaller - sized ffbs and lmas and need for high operator expertise preclude the widespread use of this method in neonates and infants . laryngeal ultrasound is a noninvasive and easily reproducible adjunct to ffb and has proven useful for vc evaluation in intubated adults ; however , its application in small children needs further study . airway exchange catheter ( aec ) is a promising reintubation guiding tool that can be retained in the trachea for long and also provide supplemental oxygen . however , it can potentially exacerbate airway reactivity and obstruction in infants whose tracheas are smaller compared to the aec . in a study evaluating the utility of aecs in pediatric difficult airways , two infants required reintubation due to airway obstruction , possibly caused by the aec itself . in view of its yet uncertain risk thus , management of tracheal extubation in small children with stridor and neuraxial anomalies is yet unestablished and is largely based on individual preferences . a ffb - guided , on - table extubation trial appears to be a reasonable option at present .
stridor is a serious complication of congenital neuraxial anomalies , which though , can get completely resolved with early neurosurgical correction of the anomaly . however , stridor relief may or may not be achieved soon after surgery . persistent postoperative stridor can potentially cause extubation failure that may be difficult to handle in small children . there are no extubation guidelines for difficult pediatric airways as yet , and fewer appropriate airway - assist devices for routine use . management of an infant with occipital encephalocele , hydrocephalus and bilateral abductor vocal cord palsy , who developed post - extubation respiratory distress due to stridor is discussed , together with the relevant tracheal extubation issues in such cases .
malignant cells infiltrate the subarachnoid space and cerebral pia mater , causing meningeal irritation and cranial nerve symptoms . mc is frequently diagnosed in patients with breast cancer , lung cancer , lymphoma or leukemia as well as tumors of the central nervous system ( cns ) , mostly medulloblastoma . intrathecal chemotherapy is effective in patients with mc from lymphoma , leukemia and breast cancer . however , intrathecal treatment requires invasive procedures , preferably repeated lumber puncture , and limits the use of chemotherapy agents due to the cerebrospinal damage . a 60-year - old postmenopausal woman was referred to our hospital due to the tumor in the right breast , in april 2008 . she was diagnosed with stage iib breast cancer ( ct3n0m0 , invasive lobular carcinoma ) , and received 12 cycles of weekly paclitaxel 80 mg / m , with fec ( 5-fluorouracil 500 mg / m , epirubicin 100 mg / m , cyclophosphamide 500 mg / m ) as a neoadjuvant chemotherapy ( nac ) . pathological examination showed the breast cancer to be node - negative , estrogen receptor - positive ( 99% ) , progesterone receptor - negative , and human epidermal growth factor receptor type 2 ( her2)-negative , she developed a tongue deviation ; magnetic resonance imaging ( mri ) showed multiple leptomeningeal metastases ( fig . serum cancer antigen 15 - 3 ( ca15 - 3 ) and carcinoembryonic antigen ( cea ) levels increased from 7.0 iu / ml and 0.3 ng / ml to 96.0 iu / ml and 3.5 ng / ml , respectively . cerebrospinal fluid ( csf ) analysis was not performed ; mc was diagnosed through positive imaging and neurological symptoms . based on the diagnosis of mc , she received capecitabine monotherapy ( 2,400 mg / day ) . only 3 weeks later , her tongue deviation disappeared . since she had started receiving capecitabine , ca15 - 3 and cea levels gradually reduced ( fig . in america and european countries , breast cancer is most frequently associated with mc among extra - cns solid malignancies . about 25% of patients with breast cancer develop mc , usually late in the course of their metastatic disease . on the other hand , in japan , gastric cancer ( 55% ) and lung cancer ( 30% ) are highly associated with mc , but mc from breast cancer is extremely rare ( 1.7% ) . there are no distinct reasons for this difference , which may be attributed to the lower disease rate of breast cancer itself , or the lower incidence of invasive lobular carcinoma of all histologic subtypes of breast cancer in japan . gauthier et al . reported a high incidence ( 28% ) of invasive lobular carcinoma in mc from breast cancer [ 2 , 7 ] . by hematogenous spread or direct extension from bone metastasis or brain metastasis the most common sign is multiple cranial nerve palsies , but headache , backache , polyradiculopathies , incontinence , confusional state , lower motor neuron weakness and sensory abnormalities may be the principal manifestations [ 4 , 8 ] . although mc from breast cancer shows the best prognosis among mc from various malignancies , median overall survival is no more than 6 months following diagnosis of mc from breast cancer . treatment of mc relies mainly on intrathecally and/or systemically delivered chemotherapy , combined with other treatment modalities , such as irradiation or debulking surgery of meningeal metastases . there are no standard chemotherapy regimens for mc because neither prospective nor retrospective studies have shown any anticancer drugs to be highly effective against mc . there are reports of breast cancer - derived mc that responded to hormonal manipulation alone [ 2 , 8 ] , and some cases that demonstrate the efficacy of intrathecal trastuzumab against mc from her2-positive breast cancer [ 10 , 11 ] . the present case was hormone - resistant and her2-negative breast cancer , which limited options for chemotherapy . there is only 1 case report describing the efficacy of capecitabine monotherapy against mc from breast cancer . capecitabine is an oral prodrug of fluorouracil used in the palliative treatment of metastatic breast cancer that is resistant to taxane and anthracycline chemotherapy . animal studies demonstrate that capecitabine and its metabolites cross the blood - brain barrier in limited quantities . a study in humans using a fluoropyrimidine similar to capecitabine found that fluorouracil concentrations in csf were only 13% of plasma concentrations . we do not know the mechanism of response to capecitabine in our case , but a similar rapid and durable response to capecitabine was reported in a patient with refractory brain metastasis from breast cancer . it is possible that diffuse mc in our case disrupted the blood - csf barrier and allowed a higher fluorouracil concentration in the csf . since most patients with mc from breast cancer have various prior therapies and poor performance status , capecitabine monotherapy is a feasible approach in the treatment of mc from breast cancer .
a 62-year - old woman with breast cancer received neoadjuvant chemotherapy followed by breast - conserving surgery and sentinel node biopsy . during adjuvant endocrine therapy with aromatase inhibitor , she developed multiple bone metastases . thereafter , she received tamoxifen and zoledronate therapy . in may 2011 , she developed a tongue deviation and was diagnosed as having meningeal carcinomatosis . the tongue deviation disappeared 3 weeks after taking capecitabine ( 2,400 mg / day ) . magnetic resonance imaging of the brain showed regression of meningeal carcinomatosis . levels of tumor markers cea and ca15 - 3 changed from 96.0 iu / ml and 3.5 ng / ml to 47.0 iu / ml and 1.5 ng / ml , respectively . progression - free survival with capecitabine monotherapy was 5 months .
alopecia areata manifests as loss of hair on a hairy part of the skin , such as the scalp , beard , mustache , etc . , which may be patchy , confluent or diffuse in pattern . it may occur as a single , self - limiting episode or may recur at varying intervals over many years . the origin of the disease process is not fully understood ; however , there are indications for a t cell - mediated autoimmune process directed against an unknown autoantigen of the hair follicle . t lymphocytes that have been shown to be oligoclonal and autoreactive are predominantly present in the peribulbous inflammatory infiltrate . alopecia areata frequently occurs in association with other autoimmune disorders such as generalized vitiligo , lichen planus , morphea , lichen sclerosus et atrophicus , pemphigus foliaceus , atopic dermatitis , hashimoto 's thyroiditis , hypothyroidism , endemic goiter , addison 's disease , pernicious anemia , lupus erythematosus , diabetes mellitus , down 's syndrome and others [ 3 , 4 ] . she complained of loss of hair over the frontal area of the scalp and over the posterior aspect of the scalp above the neck region . she complained of depigmented lesions since 7 months on the right and left leg . on examination , loss of hair was seen over the frontal region of the scalp ( fig . 1 ) and over the posterior region of the scalp above the neck region . 2 ) . there was no evidence of atopic dermatitis , neurodermatitis , seborrheic dermatitis , verrucae , furunculosis , lichen planus and lichen sclerosus et atrophicus . systemic diseases like thyroid disorders , anemia , diabetes , allergic rhinitis and bronchial asthma , hypertension and ulcerative colitis were not observed in the patient . the association of alopecia areata with seemingly unrelated autoimmune disorders has led to an intense search for immune derangements . in vitiligo , an autoimmune hypothesis is suggested by its clinical association with a number of disorders considered to be autoimmune . vitiligo seems to occur about 4 times more often in patients with alopecia areata than in the general population . alopecia areata accounts for around 2% of new dermatological outpatient attendances in britain and the united states . among the factors that appear to be implicated in etiopathogenesis are the genetic constitution of the patient , atopic state , nonspecific immune and organ - specific autoimmune reactions and possibly emotional stress . alopecia areata has been considered as an autoimmune disease due to an aberrant t cell response against hair follicle self antigens . this autoimmune etiology has also been proposed on the basis of its association with autoimmune diseases , the presence of autoantibodies and various underlying immunologic abnormalities in the affected sites of these patients . lesions in alopecia areata do not form and resolve in the usual sense ; however , as evident by its chronicity and high rate of recurrence , the disease represents a continuous reactivity of the pilar units . the capacity of hair to react in clinically recognized patterns as alopecia areata is probably inherited , although the mode of inheritance , penetrance and expressivity of the genes must be highly variable . alopecia areata may begin as early as the first month of life [ 7 , 8 ] or as late as in the late seventies . atopy has been reported to occur with an increased frequency in patients with alopecia areata [ 4 , 9 ] . the atopic type in ikeda 's classification of alopecia areata comprised 10% of 1,989 patients . similarly , 11% of 736 patients with alopecia areata studied by muller and winkelmann had concomitant asthma or atopic dermatitis . in our patient the incidence of thyroid disease has varied from 8 - 28% in patients with alopecia areata . lewinski et al . also confirmed the frequent coexistence of alopecia areata and thyroid abnormalities . conversely , in 1994 , puavilai et al . estimated that the prevalence of thyroid disease is relatively low ( 7.2% ) , and patients with thyroid disease were not statistically different from patients with alopecia areata and the control group . , there is usually symmetric distribution of lesions and new patches may appear throughout the patient 's life vitiligo which is so extensive that few normally pigmented patches remain is known as vitiligo universalis . focal vitiligo may develop into generalized vitiligo or may follow an early - stabilizing clinical course . segmental vitiligo has important differences in etiology , prevalence of associated illnesses and therapy compared to other forms of vitiligo . segmental vitiligo is known for its early onset and rapid spread . without treatment , lesions are typically persistent throughout life , but stop developing within 2 years of onset . childhood vitiligo , in one survey , consisted primarily of typical generalized , nonsegmental vitiligo ( 78% ) , with focal vitiligo ( 14.4% ) and segmental vitiligo ( 4.6% ) being far less prevalent .
alopecia areata is a common cause of noncicatricial alopecia that occurs in a patchy , confluent or diffuse pattern . it may occur as a single , self - limiting episode or may recur at varying intervals over many years . the association of alopecia areata with localized vitiligo has not been reported . the association of alopecia areata with localized vitiligo in the same patient is documented here ; it is the first of its kind .
when a mega - trial comes up with the same findings as a meta - analysis , or vice versa , the evidence appears overwhelming . this was the case with the use of intravenous followed by oral beta - blockers in acute myocardial infarction in the international study of infarct survival ( isis)-1 study and the subsequent systematic review . it is not surprising , therefore , that perhaps the most prestigious of cardiological organisations came up with following the class 1 recommendation for deciding who should receive this therapy : " patients without a contraindication to beta - adrenoceptor blocker therapy who can be treated within 12 hours of onset of infarction , irrespective of administration of concomitant thrombolytic therapy or performance of primary angioplasty " . there is no suggestion in this guideline that some patients conforming with those criteria might benefit or be harmed more than others . this may well have been because the isis-1 investigators were pioneers in highlighting the potential dangers of subgroup analysis . this they did most vividly by pointing out how the results varied with a patient 's astrological sign . in many countries , notably the uk , the contemporary use of intravenous followed by oral beta - blockers is very low . it was reported that the british investigators used this therapy in only 2% of the patients in isis-4 . it is unlikely that it was ignorance because many of the hospitals participating in isis-4 also participated in isis-1 . this is unlikely because the use of aspirin and thrombolysis ( studied in isis-2 ) was very high , by international standards . there is a tendency to believe that somehow the designers of trials have been able to choose a basically homogeneous group of patients and , equally , that they have sound reasons for their exclusion criteria . beta - blockers are known to lower blood pressure , sometimes dangerously , in patients already hypotensive or in acute heart failure . it was entirely appropriate , therefore , that low blood pressure was an exclusion criterion in isis-1 . the investigators chose the figure of 100 mmhg , below which one should not give the drug . an unexpected finding in isis-1 was that the main reason for the lower mortality in the treated group was the prevention of cardiac rupture on the first day . hypertension is one of the factors in its genesis . as shown in table 1 , relationship between blood pressure and clinical outcome breslow - day test : = 5.269 , df = 2 , p = 0.072 . breslow - day ( low systolic blood pressure versus the other two blood pressure ranges ) : = 3.412 , df = 1 , p = 0.065 . testing for interaction of blood pressure and study , although not conventionally significant , the metoprolol in acute myocardial infarction ( miami ) trial leant support to the isis-1 trial and was the most important other contributor to the meta - analysis . would it be right to treat the more than 50% of patients with 0 - 2 risk factors with metoprolol ? , it would be brave to dismiss the findings in the high - risk subgroups . a proper statistical analysis ( as shown with the figures presented in the tables ) fifteen - day mortality in the miami trial breslow - day test : = 5.538 , df = 4 , p = 0.236 . breslow - day ( 2 rfs versus > 2 rfs ) : = 4.016 , df = 1 , p = 0.045 . testing for interaction of number of risk factors and study , p = 0.044 . the first meta - analysis of 27 536 patients , undertaken in 1986 , was significant at the 0.02 level , and demonstrated that intravenous followed by oral beta - blockade would save six lives in every 1000 patients treated . the second meta - analysis , of 29 260 patients , undertaken in 1999 , showed no significant benefit ( 95% confidence interval 0.85 - 1.08 ) . the first led authorities to recommend intravenous followed by oral beta - blockers for all patients . by the same logic , one should now treat no patients with this regimen . clearly , this is ridiculous . of course , it is desirable for the subgroups to be defined in advance and for them to be few in number . it is also customary to say that subgroup findings should be hypothesis - generating , but these hypotheses are seldom tested in adequately sized trials . it seems to me reasonable to look for evidence of benefit or harm in certain biologically plausible subgroups , even though the statistical basis for this evidence is not compelling . it is ludicrous and economically mad that we have to treat 1000 patients to benefit six because our trial methodology can not cope . certainly , it is quite wrong use a computer to look for statistically significant subgroups , as was done in the case of the astrological signs . but cardiologists have to make decisions on the basis of the very imperfect evidence which clinical trials usually provide . let them use their knowledge of the biology of the condition to interpret which subgroups are clinically relevant and look for ( albeit imperfect ) statistical support . the author would like to thank dr ralph d'agostino for providing a statistical analysis of the figures presented in this article .
clinical trialists and statisticians are very wary of subgroup analysis , for good reasons . clinicians have to deal with situations in which subgroups of patients differ widely from one another in their prognosis and response to treatment . few trials are large enough to demonstrate convincingly these differences in outcome , but often provide suggestive evidence . should we ignore this and treat all patients as the same , or should we allow dubious statistical evidence to buttress biological plausibility in making clinical decisions ?
dyskeratosis congenita ( dc ) is an inherited disorder with progressive multi - system involvement leading to bone marrow failure ( bmf ) , squamous cell carcinoma , pulmonary fibrosis ( pf ) , etc . liver transplantation ( lt ) for cirrhosis secondary to dc has not been reported previously and we describe here the perioperative management of the same . a patient with diagnosed dc at 25 years of age presented with esld to our institute in his 34 year . past history included haematopoietic stem cell transplant ( hsct ) for bmf secondary to dc . patient was diagnosed with cirrhosis post - hsct and was kept on medical management for 9 years . at the time of presentation he was not on any immunosuppressant . clinical examination revealed multiple dental caries , reticulate skin pigmentation , nail dystrophy , severe clubbing , epiphora , enlarged liver with irregular margins , splenomegaly and ascites . pre - operative liver function tests showed hypoalbuminemia ( 2.6 g / dl ) with slightly elevated transaminases . on arterial blood gas ( abg ) analysis , partial pressure of oxygen ( pao2 ) was 51.3 mm hg with widened alveolar arterial oxygen gradient ( pao2pao2 = 59.7 mm hg ) [ table 1 ] . abg with 100% o2 supplementation demonstrated an increased pao2 ( 158 mm hg ) and spo2 of 100% [ table 1 ] . arterial blood gas values at various time points high resolution computerized tomography ( ct ) demonstrated mild bilateral interstitial lung disease ( ild ) [ figure 1 ] . saline contrast echocardiography ( sce ) suggested severe right to left intrapulmonary shunting and macroaggregated albumin scan ( maa ) demonstrated a 21% shunt fraction . child - turcotte - pugh score was 10 ( child c ) and model for end stage liver disease score was 14 . patient was diagnosed with cirrhosis secondary to dc and posted for urgent living donor liver transplantation ( ldlt ) . high resolution computerized tomography thorax showing mild bilateral interstitial lung disease apart from standard anaesthesia monitoring , invasive haemodynamic monitoring ( central venous pressure , invasive arterial blood pressures and continuous cardiac output monitoring using flowtrac ev1000 edwards life sciences ) were used . anaesthesia was induced with propofol ( 1.5 mg / kg ) , fentanyl ( 1.5 gm / kg ) , and rocuronium ( 0.9 mg / kg ) with modified rapid sequence induction . maintenance of anaesthesia was with isoflurane in air ( minimum alveolar concentration 11.2 ) , with infusions of atracurium and fentanyl . baseline abg after induction of anaesthesia showed pao2 of 140 mm hg with fio2 of 0.5 . persistent svv > 13% in absence of sustained major vascular occlusion or arrhythmias was treated with 250 ml boluses of 5% albumin . noradrenaline and vasopressin infusion were titrated for a targeted mean blood pressure > 65 mm hg . after reperfusion , fio2 requirements increased from 0.5 to 0.7 to maintain the pao2 above 100 mm hg . estimated blood loss during the surgical procedure was 2100 ml . patient was gradually weaned from mechanical ventilation and vasopressors and extubated on post - operative day 2 in the intensive care unit . post - extubation intermittent non - invasive bilevel positive airway pressure ( bipap ) support was provided . immunosuppression was provided with steroids , tacrolimus and mycophenolate . on post - operative day 7 , patient developed tachypnoea , increased o2 requirement , fever and productive cough . chest auscultation revealed bilateral basal crepitations and basal lobe consolidation on chest x - ray . oxygen requirements were increased and were provided via high concentration o2 mask with reservoir bag to maintain acceptable levels of saturation . on availability of sputum culture reports ( culture demonstrated escherichia coli sensitive to carbapenems ) , patient was shifted from broad spectrum empirical antibiotics to culture specific antibiotics and patient gradually improved with decrease in oxygen requirements . patient was discharged from the hospital on post - operative day 29 with a pao2 of 70 mm of hg on room air , which at 2 months was 78 mm of hg . dyskeratosis congenita is a rare multisystem disorder characterized by the triad of abnormal skin pigmentation , nail dystrophy and mucosal leucoplakia . patients exhibit considerable clinical and genetic heterogeneity with x - linked recessive , autosomal dominant and recessive forms . bone marrow failure and pf are the most common causes of mortality(6070% for bmf/15% for pf ) in these patients . hsct is the only curative option for bmf but increases the risk of pulmonary complications . hepatic involvements including hepatomegaly , hemosiderosis , fibrosis , and cirrhosis have been reported in 10% of cases . presence of severe hypoxemia and orthodeoxia and absence of ild led to a probable diagnosis of hepatopulmonary syndrome ( hps ) . imbalance between vasoconstrictors and vasodilators leading to pulmonary vascular dilatation at the pre - capillary and capillary level in patients with liver disease results in hps . subsequent intrapulmonary shunting and v - q mismatch causes progressive hypoxia , dyspnoea , and cyanosis . the patient reported here was fulfilling the criteria for hps : ( 1 ) chronic liver disease ( cld ) , ( 2 ) alveolar - arterial oxygen gradient > 15 mm of hg and intrapulmonary shunt ( demonstrated by sce and maa ) . severe intrapulmonary shunting ~21% and pao2 of 51.5 mm of hg on room air placed him in almost the very severe category of type i hps . lt being a definitive treatment for hps and severity of hypoxaemia limiting time for medical therapy trial , lt was offered to the patient on an urgent basis . the prevalence of hps in patients with advanced liver disease is approximately 1633% . without lt the 5 years survival rate for cirrhotic patients with hps however , more recently gupta et al . in their study have reported a mortality rate of 9% in patients with severe hps in peri - transplant period . it has also been demonstrated that severity of pre - operative hypoxaemia correlates with the peri - transplant mortality . rapid progression of pre - operative hypoxaemia in these patients mandates minimal transplant waiting time . ldlt reduces transplant waiting time and prevent functional status deterioration and thus provide good results in patients with severe hps . the low acceptable thresholds of pao2 values were kept keeping in mind pre - operative severe hps and hypoxaemia . restrictive fluid therapy using svv along with lung protective ventilation strategies ( low tidal volume ) were used to minimize pulmonary complications . other supportive therapies like o2 supplementation , incentive spirometry , intermittent non - invasive ventilatory support , adequate attention to asepsis and antibiotics were provided as per institute 's protocol . hps can be an important cause of hypoxemia in patients of dc presenting with cld . directed pre - operative evaluation to determine the primary cause of hypoxaemia can help in identifying patients who may benefit from lt .
dyskeratosis congenita ( dc ) is an inherited disorder with progressive multisystem involvement . end stage liver disease ( esld ) in patients with dc is rare . we describe the perioperative management of a patient with dc induced esld and severe hepatopulmonary syndrome for living donor liver transplantation .
nutrient profiling classifies individual food products according to their nutrient content . in the 1924/2006 european regulation , nutrient profiling is proposed as a tool for deciding whether a given food might be eligible or not for bearing nutrition or health claims . one core aim of the regulation is to avoid a situation where nutrition or health claims mask the overall nutritional status of a food product , which could mislead consumers when trying to make healthy choices in the context of a balanced diet ' . report of a who ( world health organization)/iaso ( international association for the study of obesity ) joint technical meeting recently highlighted the importance of using validated nutrient profiling systems and listed a number of possible validation methods . these include assessment of construct validity , that is , testing whether healthy foods ( identified as eligible by nutrient profile system ) make healthy diets , and unhealthy foods ( that is , non - eligible ) make unhealthy diets . the aim of the present study was to assess the construct validity of five nutrient profiling systems , using diet modeling with linear programming for designing healthy and unhealthy diets . the dietary data and the food database were derived from the inca french dietary survey . each food of the food database ( n=597 ) was allocated to one of two classes , eligible and non - eligible , according to each of the five european nutrient profile systems tested . two systems are currently used for food labeling , namely choices v2.2 and green keyhole , two have been proposed to assess food products ' eligibility to claim but that are not currently in use , namely the french afssa system ( agence franaise de scurit sanitaire des aliments ) and the ec system ( european commission draft ) , and foodprofiler , a system in use for self - limiting advertisement to children . using a previously described diet modeling approach , the feasibility of designing healthy or unhealthy diets with eligible foods only , or with non - eligible foods only , was tested for each nutrient profiling system . healthy diets were defined by the fulfillment of a set of a forty nutrient constraints , and unhealthy diets by the nonfulfillment of the same set of nutrient constraints . to ensure realism to the modeled diets , constraints on foods and food groups were included and the possibility to design a 2000-kcal diet was tested . four feasibility tests were used to assess the construct validity of each system . tests a , eating healthily with eligible foods only , and d , eating unhealthily with non - eligible foods only , were used to assess whether the systems respected the nutritional common sense . tests b , eating unhealthily with eligible foods only , and c , eating healthily with non - eligible foods only , assessed more finely the level of permissiveness . the following terminology is used : strict unfeasibility ' means that no mathematical solution can be found at all . when solutions can be found , the range between the minimum and the maximum energy achievable is called the energy range ' . a system is considered as valid when tests a and d are feasible , and tests b and c are unfeasible . the percentage of eligible foods varied between systems : choices : 31.8% green keyhole : 35.6% afssa : 35.8% ec system : 46.5% and foodprofiler : 49.5% . figure 1 shows that all tested systems allowed 2000 kcal healthy diets with eligible foods ( test a ) , and 2000 kcal unhealthy diets with non - eligible foods ( test d ) . regarding test b , all tested systems exclude 2000 kcal from their energy range and were , therefore , considered as unfeasible : strict unfeasibility was observed for choices and green keyhole systems . unfeasibility was observed also for the foodprofiler and ec systems , the maximum energy achievable being 1772 kcal and 1801 kcal , respectively , and for the afssa system , with a narrow and unrealistic energy range ( 587907 kcal ) . regarding test c , test c was feasible for choices and green keyhole ( 2000 kcal healthy diets allowed with non - eligible foods ) and it was unfeasible for afssa and ec systems ( the energy range for test c excluded 2000 kcal but the minimum energy achievable was close to 2000 kcal : 2018 kcal and 2162 kcal , respectively ) . one important result is that , with all tested systems , it was possible to design healthy diets with eligible products and unhealthy diets with non - eligible products ( tests a&d ) , demonstrating that all systems satisfied the nutritional common sense . in line with the latter results , choices was previously presented as valid based on its beneficial impact on dietary quality in modeling studies simulating the replacement of foods not complying with the choices criteria by existing choices - compliant products . nevertheless , choices and green keyhole allowed to design 2000 kcal healthy diets with non - eligible products , and were , therefore , identified as invalid according to this construct validity assessment . in contrast , foodprofiler , afssa and ec systems were identified as valid because they answered as expected to the four tests . the three valid systems showed differences in their level of permissiveness . with the afssa system , it was almost feasible to design a 2000-kcal healthy diet with non - eligible foods ( test c ) , which suggests that some healthy foods are misclassified as non - eligible . therefore , this system rather display a keep the best ' approach when classifying the unhealthy food products . in the opposite , with the foodprofiler and the ec system , it was almost feasible to design a 2000-kcal unhealthy diet with eligible foods ( test b ) , which shows that these systems rather display a stop the worst ' approach by misclassifying some unhealthy foods as eligible . analyzing the foods selected by linear programming in these tests could help identify the misclassified foods , and then fine - tune the systems . the validation method used in the present study is perfectible , for instance by improving the realism of diets using more sophisticated diet modeling techniques . nevertheless , this study revealed that some nutrient profiling systems , among those currently used , show weaknesses by failing this construct validity assessment . among the three systems identified as valid in the present study , the ec system is the less demanding in terms of nutritional information , it would , therefore , be the easiest to implement for regulating nutrition and health claims in europe .
nutrient profiling classifies individual food products according to their nutrient content . according to the who ( world health organization ) , validation is a key step in the development of a nutrient profiling system . the aim was to assess the construct validity of five european nutrient profiling systems ( choices , keyhole , ( afssa ) , european commission ( ec ) system and foodprofiler ) . construct validity was assessed for each of the five - selected nutrient profiling systems by testing whether healthy foods ( that is , identified as eligible by the system ) make healthy diets , and unhealthy foods ( that is , non - eligible ) make unhealthy diets , using diet modeling . the afssa , ec and foodprofiler systems were identified as valid , but differences in their levels of permissiveness suggested some misclassified food products . the two other systems failed the construct validity assessment . among these three systems , the ec system is the less demanding in terms of nutritional information , it would , therefore , be the easiest to implement for regulating nutrition and health claims in europe .
it classically occurs in the second decade of life , has its onset during puberty , and affects both sex and all ethnicities . the estimated prevalence in the general population is 54 per 100,000 . using a combination of four indices , ie , central k ( descriptive of central steepening ) , the i - s value ( inferior - superior dioptic asymmetry ) , sim k , and the srax index ( skewed radial axes ) , rabinowitz was able to distinguish 98% of keratoconus videokeratographs from a group of normal controls,1 and in marked keratoconus , simple observation of the presence of keratometry over 47.20 d can make the diagnosis.2 sensory strabismus occurs due to temporary or permanent loss of vision in one or both eyes . we present the case of a 17-year - old boy with bilateral keratoconus , exotropia , and a nodular lesion of the bulbar conjunctiva in the left eye . a prism cover test revealed a 45 prism d exodeviation ( figure 1 ) . the aligned eye was the right eye and the keratoconus in this eye was of late onset . vision in the left eye was poor and the keratoconus was advanced in this eye . the patient has a corrected visual acuity of 6/9 in his better and aligned eye and an acuity of 1/60 in the affected eye . keratometric measures were 42.75 d at an axis of 28 and 49.0 d at an axis of 118 in the right eye , and 52.0 d at an axis of 154 and 58.75 d at an axis of 64 in the left eye . simk1 was 45.24 d at an axis of 113 and sim k2 was 42.75 d at an axis of 25 in the right eye . sim k of the left eye could not be obtained because of a high steep keratometry value . refraction in the right eye was + 1.5 , 7.5 25 and refraction in the left eye could not be obtained ( figure 2 ) . a search of pubmed yielded only two studies reporting development of sensory strabismus after keratoconus . in these reports , binocular function in patients with longstanding asymmetric keratoconus was investigated by the authors . the first of these reports was by sherafat et al , who investigated a database of approximately 350 patients with keratoconus.4 only 20 of these patients were identified as having abnormal binocular function.4 the abnormalities described in their study were central suppression of the deviating eye , reduction or loss of stereopsis with a microtropia when wearing a scleral lens , and a manifest exotropia with suppression when the poorer eye was uncorrected . sherafat et al accepted the age of diagnosis as being the age of onset of keratoconus , and suggested that the adult visual system in these cases had been affected by prolonged visual deprivation . however , it has generally been assumed that the sensitive period ends at about 7 years of age and that the adult human visual system is not affected by visual deprivation.59 it is possible that the age of onset in the patients reported on by sherafat et al may have been earlier than puberty and therefore their visual deprivation may have settled . the second report was by khan and al - shamsi , who investigated a database of 103 patients with keratoconus and identified abnormal binocular function due to longstanding keratoconus in only seven patients.10 in their series , six patients had persistent exotropia . after intervention to improve visual acuity , six patients complained of constant binocular diplopia that resolved after successful surgical alignment . the authors explained that resolution of diplopia by realignment of the eyes enabled suppression of scotoma and freedom from diplopia . the major limitation of their study was the lack of information provided on age at onset of keratoconus and a lack of explanation regarding the etiology of suppression of keratoconus . in our study , neither the patient nor his parents knew when the keratoconus had started , but did give two clues , ie , a history of childhood - onset strabismus and vernal conjunctivitis with eye rubbing . this lesion had invaded the cornea , was surrounded by injected blood vessels , and looked like pseudoepitheliomatous hyperplasia or a sequel of keratoconjunctivitis in childhood ( figure 3 ) . pseudoepitheliomatous hyperplasia is an inflammatory lesion that usually occurs on the eyelid , but rarely in the bulbar conjunctiva . however , there was no active inflammation of the conjunctiva in our patient , so this lesion may have been a sequel of keratoconjunctivitis in childhood . the causes of poor eyesight are varied , with chorioretinal atrophy , congenital cataract , optic atrophy , retinal disease , complicated cataract , leucoma , coloboma , high myopia , congenital glaucoma , penetrating trauma , contusional eye trauma , and traumatic cataract being the more common causes.11 sensory strabismus also appears to have various causes , but is not a consequence of keratoconus,1,12 which typically commences at puberty and commonly affects both eyes . however , when one eye is more severely affected than the other , visual loss can be prevented by glasses or contact lenses . the visual loss is gradual and occurs primarily as a result of irregular astigmatism and myopia.13 the sensitive period for development of suppression ends when the keratoconus has settled . the presence of a nodular lesion of the bulbar conjunctiva and a history of vernal conjunctivitis and eye rubbing in this patient confirms vernal keratoconjunctivitis in childhood . rabinowitz1 and sugar12 consider that atopic conjunctivitis and eye rubbing are a cause of keratoconus . the probable scenario in this patient is that vernal keratoconjunctivitis caused the childhood keratoconus , which became advanced in the left eye , and the prolonged visual loss was not corrected before the age of 7 years . we believe that the abnormal binocular function due to keratoconus in our patient and in the cases reported by other authors occurred before the age of 7 years . review of the relevant literature suggests that keratoconus seldom begins in the sensitive period during which abnormal visual experience affects visual development and may induce sensory exotropia . in our patient , sensory exotropia had developed because of prolonged visual loss based on the keratoconus in his poorer eye . if keratoconus occurs before the age of 7 years and prolonged visual loss is not corrected , sensory strabismus can develop , as in our patient .
this case report describes a 17-year - old boy with sensory strabismus due to keratoconus and an ipsilateral nodular lesion of the bulbar conjunctiva . the aligned eye was the right eye and keratoconus in this eye was of late onset . vision in the left eye was poor and keratoconus was advanced in this eye . due to the longstanding nature of the keratoconus and its occurrence in a developmentally sensitive period , sensory exotropia had developed in the left eye . there was a nodular lesion of the bulbar conjunctiva in the ipsilateral eye . if keratoconus occurs before the age of 7 years and the prolonged visual loss is not corrected , sensory strabismus can develop , as in this patient .
the sad fact is that road traffic accidents constitute the number one killer in libya.1 not a day goes by in libya without the deaths of families , young men , women and children in horrific car accidents.1 it is estimated that more than one million people lose their lives as a result of road accidents.2 a number of factors , including human error , the vehicle , safety measures , environment and place of accident influence the seriousness of the accident . the department of transport and license in benghazi determines the relationship between safety and liability in accidents.3 this had promoted the understanding of the elements of the environment and ideas of safety that greatly influence the driver 's attitude . there will therefore , be practical recommendations , and an enforcement of the road and traffic regulations to reduce the number of road traffic accidents.4 male drivers are responsible for highest number of fatalities in major accidents in the world.5 the purpose of the study was to identify all major factors , responsible for road accidents in benghazi . the department of transport and license in benghazi collects data on the incidence of road traffic accidents . it identifies , any relationship between the development of the culture of safety and the likelihood of accidents . the result is an understanding of the elements of the environment and the culture of safety that tend to have greatest influence on the driver 's attitude . consequently , practical recommendations will emanate on how the ideas of safety and road traffic rules can reduce the road traffic accidents . data was collected from traffic and license department and analyzed statistically according to different parameters such as age , sex , time , environmental factors , type of roads , vehicles running out of control , non - adherence to traffic rules , drunk driving , lack of concentration while driving , change of lanes , etc . a total of 1265 accidents occurred during the years 2006 - 2007 within benghazi city limits , 11.14% were fatal , 67.35% caused severe injuries and 21.51% escaped with minor injuries . this is followed by 13.47% who died on the fly - over , and 2.12% on minor roads connected to main roads within the city . the mean and its standard deviation of accidents were 16.66 25.67 and variance of fatality was 1.54 . fatalities in road traffic accidents according to zones table 2 shows accidents resulting in severe injuries . the major accidents occurred within city limits ( 87.67% ) followed by 5.98% on the coastal highway and least number of accidents was 2.11% on minor roads linking main road . the mean of the severely injured persons in total number of accidents is 16.66 31.82 . severe injuries in road traffic accidents by zones table 3 shows accidents and deaths that occurred within 24 hours : 39.23% occurred between 13:00 - 18:00 hours , followed by 33.84 % from 18:00 to 24:00 hours and the lowest number of accidents 5.38% 0 - 6 hours with a mean of 24.99 13.01 and a variance of 0.52 . road traffic accidents according to time interval and death rate table 4 shows hit and run victims ( both sexes ) . males were 85.93% of the total hit and run victims , while female were 14.06% . one - hundred - thirty people lost their lives between 2006 and 2007 , while 850 people were severely injured ( table 1 and 2 ) . the accidents and deaths were not due to a single cause , but were the result of combination of failures , including driver error , problems with the vehicle , surface of the road and lay - out , weather conditions and the time of day.78 more road accidents occurred here late at night than occurred in developed countries.9 the results , ( table 3 ) , revealed that 33.84% of the accidents occurring between 2006 and 2007 , may have been due to environmental factors such as fog or winter rains . in addition , unlit streets , lack of concentration by drivers , aggression , faulty car tyres , failure of the brake system and vehicle lights and age of the vehicle were contributing factors . collision occurred as a result of obstruction of the roads by broken down vehicles and reduced visibility resulting from bad car lights , which is a typical feature of accidents in benghazi . the study confirmed that reduced visibility as a result of ghibli ( sirocco ) , winter rains also led to accidents.1011 one of the causes of accidents in benghazi city is suburban expansion of benghazi city in the last few years . this has led to a phenomenal increase in the use of motor vehicles . road accidents are inevitable consequences of the increased distances traveled since the tendency is to drive at high speeds to reach destinations more quickly . conflicting desires of drivers and their lack of courtesy , multiple access points and exits on both sides of the road , advertisements that distract drivers attention , signs , visibility , and parked cars may be some of the causes of collisions . it is evident from the results that the highest number of fatalities was recorded near the city ( 73.04% ) compared to 0% on the city 's main roads and rough roads . the accidents and high velocity impact collisions on the highway and main road were due to high speeds and the sudden change of lanes by drivers without due care or regard for other drivers or oncoming vehicles.10 accidents involving pedestrians increase with urbanization . this increased risk - involving pedestrians as a result of the lack of crosswalks and sidewalks and inadequate lighting on small roads . together with other factors such as alcohol abuse and pedestrian carelessness , the number of accidents increase . it is also possible that a large number of these accidents have been noted as having been caused by the distraction of the driver or other observational failures . studies910 have shown that most accidents occur in the early hours of the morning whereas , the study in benghazi showed that most accidents occurred between 13:00 and 24:00 hours . it is concluded from the studies that major road traffic accidents occur as a result of environmental factors and stress . in addition , fatalities and the seriousness of the accidents depend on number of factors such as the age of the vehicle , safety measures , human error and time and place of accident .
objectives : the aim of the study was to evaluate the factors responsible for road traffic accidents in benghazi.material and methods : retrospective and descriptive studies were done in the years 2006 - 2007 . the data was collected from traffic and license department , benghazi . the data were analyzed , based on fatalities , the severely handicapped , hit and run victims and were correlated with age , sex , time , environmental factors , type of roads , etc.results:one-thousand-two-hundred-sixty-five accidents occurred between the years 2006 - 2007 within the benghazi city limits ; 11.14% of the injuries were fatal ; 67.35% of the victims had severe injuries and 21.51% escaped with minor injuries . table 1 shows that 73.04% lost their lives within the city limits , 13.47% on the fly - over , and 2.12% on minor roads connected to main roads within the city limits . the mean of the accidents and its standard deviation were 16.66 25.67 with a variance of fatality of 1.54.conclusion:it is concluded from the studies that major road traffic accidents occur because of environmental stress factors . in addition , fatalities and the seriousness of the accidents depend on a number of factors such as the age of the vehicle , safety measures , human error and time and place of accident .
enema administration has been a common practice worldwide for several centuries for the treatment of chronic constipation and for preparation of patient for a diagnostic test or a surgery . it seldom causes so prolonged lower gastrointestinal bleed that requires massive blood transfusion and rarely requires colectomy . we report an unusual complication of herbal enema in the form of severe ileo - colitis with persistent massive lower gastrointestinal bleed immediately after administration of enema for the treatment of chronic constipation . the bleeding was refractory to conservative treatment and was managed with emergency total laparoscopic colectomy . a 57-year - old male patient presented with persistent massive bleeding per rectum for one month that developed immediately after administration of herbal enema by a religious quack for the treatment of chronic constipation . he was admitted at some other hospital for the same complaint and was managed conservatively with oral glucocorticoid , 5-aminosalicylate preparation , metronidazole , entofoam ( hydrocortisone acetate ) and sucralfate enema . he had history of 48 units of blood transfusions during the course of conservative management to raise the haemoglobin above 8 mg / dl , but the condition did not improve and the patient was referred to our centre for further management . on clinical examination , the patient was pale , had tachycardia ( pulse , 136/min . ) and hypotension ( systolic blood pressure , 70 mmhg ) . colonoscopy revealed extensive ulcerations and friability of entire colon , more on left colon and ileal intubation also showed multiple ulcerations [ figure 1 ] . cect of abdomen showed distended small and large bowel with no air - fluid level . endoscopic view : ulcerations and friability of colonic mucosa we gave the patient a trial of conservative management because the condition was not permissible to withstand a major abdominal surgery . conservative management did not work and once again patient developed massive lower gastrointestinal bleed bringing haemoglobin down to 4.9 g% . patient was planned for emergency laparoscopic total colectomy . on laparoscopy , there was evidence of mild ascites , thick oedematous inflamed and friable large bowel and distal 30 cm of ileum . large bowel was very friable and developed 3 small inadvertent perforations by bowel holding grasper during dissection and manipulation of colon [ figure 2 ] . the patient developed hyponatremia and paralytic ileus in early postoperative period that were managed conservatively . oral liquids were started on postoperative day ( pod ) 3 and after that stoma started functioning . drains were removed on pod5 and patient was discharged on pod 7 with advice of restoration of bowel continuity after 8 - 10 weeks . majority of the population of india is seemingly unaware of the potential hazards associated with herbal medications and of the limited knowledge and diagnostic skill of those who are prescribing such type of treatments . contrary to the widespread belief that because it is natural it is safe , herbal therapy probably carries major risks and produces more serious side - effects than any other form of alternative medicine . the common natural ingredients used in enemas are aloe , coffee , garlic and milk thistle . other types of enemas include the ones made with mineral water , epsom salt , glycerin , vinegar , bark of the marula tree , fruit of the cucumis africanus , various wild herbs , industrial thinner , turpentine , undiluted dettol , ginger , pepper or soap . the injurious side - effects of herbal enema vary from mild abdominal discomfort and self - resolving haemorrhagic proctocolitis to severe colitis . most of the time , colitis responds conservatively and rarely require blood transfusion and colectomy . herbal enema induced massive lower gastrointestinal bleed is a life threatening clinical condition usually refractory to conservative management as in our case . nowadays , the indication for surgery is mainly limited to acute , uncontrollable , and recurrent forms of lower gastrointestinal bleed . there are few studies in literature that address the feasibility of laparoscopic colectomy in emergency conditions . marcello et al reported in their case - control study comparing laparoscopic total colectomy for acute colitis with a matched open colectomy group that laparoscopic total colectomy is feasible and leads to a faster recovery . the mortality rate from a subtotal colectomy in emergency setting is approximately 20% in most collected series , because of the reluctance of surgeons to perform a subtotal colectomy early and defer it till desperate circumstances arise . on the basis of surgical outcomes of this patient and literature support , we need for spreading awareness regarding the potentially disastrous adverse effects of herbal medications prescribed by quacks and advocate early intervention in such type of severe ileocolitis with massive rectal bleed .
various colonic side - effects of herbal enema have been reported in literature ranging from mild abdominal discomfort to self - limiting haemorrhagic colitis . it rarely requires blood transfusion or subtotal colectomy . we report a 57-year - old male patient developing severe ileo - colitis with persistent massive rectal bleeding immediately after herbal enema administration for the treatment of chronic constipation and was resistant to conservative management . patient was managed successfully with emergency total laparoscopic colectomy . post - operative recovery of the patient was excellent .
spindle - cell hemangioendothelioma ( sche ) is a rare vascular tumor that was first described in 1986 by sharon weiss . sche commonly starts at childhood or young adult though the tumor may affect all the age group . clinically , the tumor presents as a superficially located firm red to brown nodules on the distal extremities . most of the reports found that the most common site of occurrence of the tumor is the lower extremities . histologically , multiple cavernous blood vessels alternating with cellular areas consist of collapsed vascular spaces separated by spindle shaped fibroblastic cells constitute the bulk of the tumor . here , we present a young adult who presented to us with multiple spindle cell hemangioendotheliomas . the case a 25-year - old male patient presented to outpatient department of tertiary care hospital with multiple painful elevated swellings of both left upper and lower extremities for last 15 years . fifteen years back , he first noticed a small pea - shaped mildly painful swelling on the center of his chest . subsequently the number of the lesions increased in number as well as in size and new lesions appeared on left foot , thigh , hand and forearm . these lesions initially were firm , but with progression of the disease some of the lesions became soft . no history of gastrointestinal bleeding or pathological fracture was present . with above complaints , the patient attended to local doctor and applied some ayurvedic medications over the lesions and developed some oozing and crusting and blister formation with serosangunous discharge from the dorsal aspect of the left foot . examination of the lesions revealed multiple nodules and tumors of 26 cm in size distributed over planter and dorsal aspect of left foot [ figure 1 ] , dorsal aspect of left hand and fingers [ figure 2 ] and front and back of the left leg . most of the lesions were oval to globular in shape , skin colored and with a well - defined margin . some of the lesion showed crusting , oozing and post - inflammatory hyper - pigmentations . multiple skin - colored well - defined nodules and tumors 26 cm on planter and dorsal aspect of left foot multiple skin - colored well - defined nodules and tumors 26 cm on dorsal aspect of left hand and fingers lesions were non - tender , soft to firm in consistency and partially compressible . complete hemogram , sugar , urea , creatinine urine analyses were within the normal limits . however , liver function tests revealed elevation of transaminase level ( sgot-71 , sgpt-114 and alp-181 ) . x - ray of the hand and feet showed soft tissue swelling with calcification without any bony abnormality . we carried out color doppler study of the patient which revealed dilated veins with phleboleiths . most interestingly , histopathology of the patient revealed multiple vascular spaces ; some were dilated and other were collapsed with slit - like openings . the cellular elements of the tumor were mainly composed of multiple spindle - shaped cells with vesicular nucleus [ figure 3 ] . based on clinical , radiological and histopathological findings , diagnosis of sche was made and the patient was referred to plastic surgeon and excision followed by full thickness skin grafting was done of the leg lesions . however , the result was unacceptable to the patient . h and e staining showing multiple cavernous blood vessels alternating with cellular areas consisting of collapsed vascular spaces separated by spindle - shaped fibroblastic cells previously , it was thought that sche is a low - grade malignant tumor with a high tendency for local recurrence and minimal potential for distant metastasis . however , further studies prove that it is a benign multifocal process and the present study indicates that it is a reactive vascular proliferation . often the lesions of sche affect children and young adult though any age group can be affected by this tumor . commonly the tumor affects the distal limbs though the occurrence of sche in neck and oral mucosa has also been reported . clinically , sche presents as multiple cutaneous or subcutaneous bluish or red nodules , which occasionally may be painful . the natural course of the disease is persisting lesions and appearance of newer lesions along with the passage of time . histological character of the tumor is multifocal , totally or partially intravascular , irregularly dilated thin - walled cavernous spaces with thrombi and phleboliths intermixed . commonly , solid focal area of aggregates of spindle - shaped cells is found in between the cell . when we first show the case , the differential diagnosis was other vascular tumor like maffucii syndrome , gorham 's disease , kaposi 's sarcoma , cystic lymphatic malformation , masson 's hemangioendotheliome vegetant intravasculaire and spindle - cell hemangioma . maffucii syndrome also occurs in puberty ; however , in addition to vascular tumor it also affects bone . in this case , the absence of the bone lesions helped us to exclude this diagnosis . the absence of osteolytic bony lesions and lymphatic malformations exclude the possibility of gorham 's disease also . blue rubber bleb nevus syndrome usually starts at birth or early childhood , predominantly affecting the trunks and extremities and associated with gastrointestinal bleeding . the profile of our case did not match with blue rubber bleb syndrome in the absence of any gastrointestinal involvement . this patient presented to us after a decade long persistence of painful nodules , which first appeared in chest rather than starting from lower extremity . the patient relied more on traditional medicines than modern medicine and option of cosmetic surgery was unacceptable to him
spindle - cell hemangioendothelioma ( sche ) comprise a rare subset of vascular tumors , and here , we describe such a case and review the clinical presentation , patho - physiology , differential diagnosis of these tumors to promote early identification and discussion guidance . a 25-years - old male patient presented with multiple painful elevated swellings of both left upper and lower extremities for last 15 years without any systemic involvement . after excluding close differential diagnosis by relevant investigations an excisional biopsy was performed . based on clinical , radiological and histopathological findings , diagnosis of sche was made and full thickness excision and skin grafting were performed . the case is reported due to its rarity and adds our knowledge to the existing literature .
fiberoptic endoscopes are the most commonly used instruments to evaluate colonic pathology in modern day practice . the procedure is considered to be safe but complications such as bleeding and perforation , although rare may occur and may even be lethal especially in cases of late detection . intraperitoneal perforations are relatively common and their incidence may be quoted anywhere between 0.1% and 3% of therapeutic colonoscopies ( 1 - 4 ) . we present the case of combined pneumoperitoneum , pneumoretroperitoneum , pneumomediastinum , pneumopericardium and subcutaneous emphysema after sub mucosal tear of the ascending colon , without evidence of intraperitoneal sepsis . a 70 year old female patient , who underwent routine colonoscopy for high grade dysplastic colonic polyps , presented to our emergency department six hours after the procedure , complaining of bubbling neck and general abdominal discomfort . there was no respiratory compromise and abdominal examination showed only minimal tenderness in the lower abdomen . full blood count was normal and a chest radiograph on admission showed subcutaneous emphysema , pneumo - mediastinum and pneumopericardium but no other abnormality ( fig . 1 ) . computerised tomography of the abdomen showed in addition , pneumoperitoneum and pneumoretroperitoneum ( fig . 2 ) . a water - soluble contrast examination of the colon failed to reveal evidence of a colonic perforation . cxr showing subcutaneous emphysema , pneumomediastinum and pneumopericardium ct showing pneumoperitoneum and pneumoretroperitoneum the patient was treated conservatively and on the fifth post admission day she was discharged home . the rate of colonic perforation with regards to colonoscopy ranges from 0.1% to 3.0% ( 1 - 4 ) . previous studies reported that perforation is more common with therapeutic colonoscopies compared to the diagnostic colonoscopy 0.44% vs. 0.16% ( 1 ) . the commonest sites of colonic perforation are sigmoid and hepatic flexure , these being the most difficult areas to negotiate with the scope . the cause of perforation is described by three different mechanisms ( 5 ) : pneumaticmechanicalassociated with therapeutic colonoscopy associated with therapeutic colonoscopy immediate perforation is caused by direct trauma from excessive pressure or electrocautery ; delayed perforation is usually due to necrosis . serosal tears occurred at mean pressure of 202 + 15mmhg , and mucosal rupture occurred at mean pressure of 226 + 14mmhg . they concluded that sigmoid perforations occurred more commonly as a result of instrument trauma rather than excessive insufflation ( 6 ) . in our case minor subserosal tear was noticed after difficult hepatic flexure negotiation and the procedure was abandoned . forcible herniation of the colonic mucosa becomes permeable to air without visible perforation and then the air may pass into the peritoneal cavity ( complete perforation ) or may lead to retroperitoneal gas collection and extension to the mediastinum and neck ( incomplete perforation ) ( 7 ) . the reason is anatomical spread through the retroperitoneal fascial compartment , which extends through the posterior mediastinum to the neck . the air may enter the retroperitoneum via a mesenteric route , via direct perforation or even via pneumatosis cystoides coli ; then it may dissect along structures passing into the periphery including the great vessels and psoas muscle as in our case . mansfield and colleagues histologically demonstrated the existence of potential communication between the mediastinum and the pericardium at the ostia of the pulmonary veins ( 8) and this may explain the pneumopericardium in our patient . the investigation of choice is computerized tomography with which the exact type of perforation can be identified . in conclusion , we have presented a rare case of extra peritoneal micro perforation during diagnostic colonoscopy , with subsequent air distribution into all body compartments . to our knowledge the traditional treatment of colonic perforation consists of laparotomy and over suturing if the colon is clean , or exteriorization of the colonic ends if soiled ; however non- operative management may be appropriate in patients with no evidence of peritonitis . it is important to be aware that impressive radiological findings without the signs of general peritonitis should not preclude a conservative approach to management . any deterioration in general condition or clinical signs will naturally warrant surgical intervention by open or minimal access approach .
a 70 year old lady presented to the emergency department complaining of bubbling neck and abdominal discomfort . she underwent diagnostic colonoscopy six hours before admission . clinical examination showed a haemodynamically stable patient and imaging revealed free air in all body compartments . we report a rare case of micro perforation during diagnostic colonoscopy with massive distribution of air in all body compartments , which was successfully treated conservatively .
cholecystitis is a common condition that is treated with intravenous antibiotics and often eventually requires the patient to undergo a cholecystectomy . however , if treatment is delayed or incomplete , chronic cholecystitis results in complications that include acute pancreatitis , gallbladder empyema , perforation and cholecystoenteric fistula . mirizzi s syndrome is one rare phenomenon in which fistula forms between the gallbladder and large bowel , facilitating entry of the stone into gastrointestinal tract . the fistulous connection usually forms after a preceding episode acute cholecystitis leads to inflammation and adhesions forming between the gallbladder and colon , usually at the hepatic flexure . it is important to be aware of mirizzi s syndrome as it is a cause of bowel obstruction.(1 ) bouveret syndrome is another rare complication of cholelithiasis with an incidence of 1 - 3% of those with gallstone ileus.(2 ) it occurs when a gallstone passes through a cholecystoduodenal fistula and lodges in the pylorus or proximal duodenum causing a gastric outlet obstruction.(3 ) we report the first incidence of chronic cholecystitis resulting in a fistula between the gallbladder and a duplication cyst overlying the stomach . a 71 year old obese patient presented with a one day history of epigastric pain that had become generalised , constant and reported as 10/10 severity at its worst . further past medical history revealed angina , type 2 diabetes mellitus , asthma , transient ischaemic attacks and atrial fibrillation . on examination her abdomen was distended , bowel sounds were reduced , she had generalised peritonism that was worst in the epigastric area . biochemical and haematological investigations revealed a c - reactive protein of 7.4mg / l and a white cell count of 17.6 10/l . liver function tests ( bilirubin 13 , alkaline phosphatase 78 , alanine aminotransferase 20 ) and serum amylase were entirely normal . a ct scan of the abdomen and pelvis was requested which revealed inflammation surrounding the anterior of the stomach , a large irregular thick - walled collection arising from the fundus of the gallbladder , free fluid around the liver , spleen and paracolic gutter . ct scan demonstrating a thick walled gallbladder with free fluid in the paracolic gutter and a collection in the porta hepatis ct scan demonstrating a gastric duplication cyst it is unclear from the ct images whether the cyst is contained within the gastric cavity . an emergency laparotomy was undertaken due to the radiological findings and clinical deterioration of the patient . during the operation the gallbladder was shrunken , thick - walled and fistulated into gastric duplication cyst which contained several one centimetre gallstones and pus . these findings resulted in the patient undergoing a cholecystectomy , partial gastrectomy and a roux - en - y reconstruction . the pathology report confirmed that the gallbladder had features of chronic cholecystitis and the gastric mucosa was consistent with a duplication cyst that did not communicate with the gastric cavity . histological specimen demonstrating the cyst overlying the stomach specimen demonstrating a gastric duplication cyst in the gastric lining the patient recovered from the operation in intensive care and then underwent rehabilitation by a multi - disciplinary team on the ward . gallstone is a common disease with a prevalence of 10% in the united states and western europe . however , it is only symptomatic in 2030% of patients , with biliary pain colic being the most common symptom.(4 ) an accurate diagnosis is essential to the management and prevention of further complications . an abdominal ultrasound scan is a non - invasive tool to detect gallstones , which in addition to blood tests and the clinical impression of the patient can help determine the underlying pathology . intravenous antibiotics are an important initial treatment , endoscopic retrograde cholepancreatography can then be utilised to remove gallstones , insert stents to aid drainage of the gallbladder and perform sphincterotomy . if the diagnosis is unclear or more complex ct scans have proved to show better visualisation and are therefore considered to be the gold standard.(5 ) appropriate treatment should be implemented after considering the diagnosis and health of the patient . the patient was due to have an elective cholecystectomy after reducing a certain amount of weight . the complication that resulted from this ongoing inflammation was a perforated , necrotic gallbladder fistulating with a gastric duplication cyst , which was found to contain multiple gallstones . prognosis is frequently related to early recognition , management of any co - morbid conditions , and careful selection of treatment modalities.(4 ) in conclusion , cholecystitis should be appropriately treated to prevent patients suffering with ongoing symptoms and eventually developing complications that cause them to require urgent higher risk surgery .
cholecystitis can result in complications if not completely treated . these include gallbladder empyema , perforation and cholecystoenteric fistula.we report the first incidence of cholecystitis resulting in a gallstone fistula with a gastric duplication cyst . a 71 year old patient presented with generalised peritonism that was worst in the epigastric area . computer tomography ( ct ) revealed a perforated necrotic gallbladder . emergency laparotomy , cholecystectomy , partial gastrectomy and roux - en - y reconstruction was required . the patient made a slow but full recovery.pathology results revealed that chronic cholecystitis had resulted in a fistula with a duplication cyst overlying the greater curve of the stomach . several one centimeter gallstones were found within the cyst cavity .
to prevent the side effects of excessive accumulation of iron in the body , chelation therapy is recommended in transfusion - dependent patients ( ceci et al 2003 ; marx 2003 ) . pharmacologically , the tight binding of chelators to iron blocks the iron s ability to catalyze redox reactions ( ceci et al 2003 ) . consequently , a chelator that binds to all binding sites of the iron completely inactivates the free iron . the two common iron - chelating agents available for the treatment of iron overload are deferoxamine and deferiprone ( ceci et al 2003 ) . deferiprone is the only orally active iron - chelating drug to be used therapeutically in conditions of transfusional iron overload ( nagarajan et al 2005 ) . it is indicated as a second - line treatment in patients with thalassaemia major , for whom deferoxamine therapy is contraindicated , or in patients with serious toxicity to deferoxamine therapy ( ceci et al 2003 ) . the reaction between deferiprone and iron to form a complex red substance can be described as three molecules of the chelator , deferiprone , reacting with one molecule of iron . however , the actual mechanism of the deferiprone iron binding reaction is not well described . deferiprone is a bidentate chelator : a single molecule can interact with only two of the coordination sites on iron ( figure 1 ) . this study focused on the reaction between 1 molecule of deferiprone and 1 molecule of iron . the two main alternative pathways for the deferiprone iron binding reaction are c - c cleavage and c - o cleavage . the quantum chemical analysis for bonding energy of deferiprone ( c7h9no2 ) was performed according to classical bonding theory ( goldberg 1989 ) . the resulting complexes between deferiprone and iron from each alternative reaction pathway were analyzed , and the required energy for complex formation by each pathway was compared . deferiprone is a bidentate chelator : a single molecule can interact with only two of the coordination sites on iron ( figure 1 ) . this study focused on the reaction between 1 molecule of deferiprone and 1 molecule of iron . the two main alternative pathways for the deferiprone iron binding reaction are c - c cleavage and c - o cleavage . the quantum chemical analysis for bonding energy of deferiprone ( c7h9no2 ) was performed according to classical bonding theory ( goldberg 1989 ) . the resulting complexes between deferiprone and iron from each alternative reaction pathway were analyzed , and the required energy for complex formation by each pathway was compared . the details and the required energy for complex formation in c - c cleavage and c - o cleavage pathways are presented in table 1 . the required energy for complex formation in c - c cleavage was less than for c - o cleavage . details and required energy for complex formation in c - c cleavage and c - o cleavage pathways bond - breaking accumulated energy bond - forming released energy required energy = accumulated energy released energy abbreviations : ev , electron volt the recommended treatment for many congenital hematological disorders , especially for thalassaemia major , is regular blood transfusions . these transfusions lead to the harmful accumulation of iron in the body and subsequent hemochromatosis ( ceci 2003 ) . deferiprone is a new oral iron - chelating agent which is effective in removing iron from the heart , which is the target organ of iron toxicity and mortality in iron - loaded thalassaemia patients ( kontoghiorghes et al 2003 ) . because a single molecule can interact with only 2 of the coordination sites on iron , 3 molecules are required for complete binding ( merson and oliver 2002 ; kontoghiorghes et al 2003 ) . the crystal structure of deferiprone is orthorhombic ( chan et al 1992 ) . in each molecule , the oh group and the co oxygen are insignificantly intramolecularly hydrogen - bonded ( chan et al 1992 ) . the fundamental intermolecular and insignificant intramolecular hydrogen - bonded dimer structure of deferiprone is maintained , but is distorted and supplemented by hydrogen bonds between the co oxygen of each deferiprone molecule and the oh group of one formic acid molecule ( chan et al 1992 ) . tam et al ( 2003 ) noted that future chelator research would focus on the application of chelators for other diseases and the development of new effective chelators . evidence on the differences in the mode of action of chelators , and molecular structure activity correlations , is valuable for future metallopharmacological studies ( kontoghiorghes et al 2004 ) . therefore , research on the biochemical reaction in deferiprone iron complex formation can provide useful information for further bio - iron research . in the present study , the energy required for c - c cleavage was much less than for c - o cleavage . in addition , the total energy requirement for c - c cleavage was negative , implying that this reaction can occur without any external energy source . the resulting complex fits the reported tertiary structure model for the deferiprone iron complex ( wiwanitkit 2005 ) .
to prevent side effects of excessive accumulation of iron in the body , chelation therapy is recommended in transfusion - dependent patients . the reaction between deferiprone and iron to form a complex red substance can be described as 3 molecules of the chelator , deferiprone , reacting with a molecule of iron . however , the actual mechanism of the deferiprone iron binding reaction is not well understood . a quantum chemical analysis of the deferiprone iron binding reaction was performed , focusing on the reaction between 1 molecule of deferiprone and 1 molecule of iron . the two main alternative pathways for the deferiprone iron binding reaction were shown to be c - c cleavage and c - o cleavage . the required energy for complex formation in c - c cleavage was less than for c - o cleavage . the total energy requirement for c - c cleavage was negative , implying that this reaction can occur without any external energy source . the resulting complex fits the reported tertiary structure model for the deferiprone iron complex
lichen sclerosus ( ls ) is a chronic inflammatory dermatosis that is characterized by the involvement of vulval and perianal region . extragenital ls has been considered uncommon ; however , the involvement of face is extremely rare . lichen sclerosus ( ls ) is a chronic inflammatory disease that primarily causes vulval and perianal lesion in prepubertal , peri- and post - menopausal women , and the presence of white papules and atrophic patches . the pathogenesis is unknown , but it seems to be related to genetic susceptibility and autoimmune mechanisms . ls is more common in women than men , and the ratio of female to male ranging from 6:1 to 10:1 . it is most common on the neck , shoulders , axillae , trunk , buttocks , thighs , and wrists . a 25-year - old girl , without any history of trauma or any other significant medical history or any family medical history , presented with white patch over her nose for 3 years . it started as erythema , expanding gradually , and finally turned out to be a white patch without any symptom . examination revealed a well - demarcated , mild atrophic porcelain - white patch with a violaceous periphery over the left side of her nose . close inspection of the affected area revealed the presence of the comedo - like openings on the surface of the lesion [ figure 1a ] . histopathologic examination of the lesion showed atrophy of the epidermis , follicular plugging , focal basal cell vacuolization , and homogenization of dermal collagen along with a moderate lymphomononuclear cell infiltrate [ figure 1b and c ] . after the diagnosis was made , oral triamcinolone was prescribed with 8 mg / day combined with topical 0.1% tacrolimus twice a day , the lesion does not expand anymore with partial remission . ( a ) the lesion on the nose ( b and c ) pathological feature of ls ( h and e , 100 ) histologic features of biopsy in each case confirmed the diagnosis of ls . according to the review [ table 1 ] , we found that as the lesion of vulvar is always itching , ls of face is always asymptomatic and appears as ivory- or porcelain - white macules , papules , and plaques . it prefers to affect the infraorbital ( 3/7 ) , forehead ( 2/7 ) , and nose ( 2/7 ) . the age of onset is also around prepubertal , perimenopausal , and postmenopausal , which is correspondence with ls . the sex ratio is almost equal ( female to male is 4:3 ) , which is a little different from ls of other sites . interestingly , almost all the cases affected the right side except ours , so it still needs more cases to prove and further exploration . treatment may be effective with oral or intralesional steroids , and topical use of steroids can also lead to remission ; however , topical use of tacrolimus only may have minimal benefit . summary of reported cases of lichen sclerosus of face though ls of face is rare on the face , it still need us pay attention to this rarely reported manifestation of ls . attili and attili reviewed the lip ls , which reminds us that it can occur in any sites . atypical locations would be the palmar and plantar regions , nipples , scalp , vaccination sites , and the face . since patients with ls of face always complain the whitish plaque affecting the appearance , early diagnosis shows extremely important . however , disease of early stages is usually difficult to diagnose . based on both of clinical and histologic features , the diagnosis of ls is made . nowadays , dermoscopy and confocal microscopy are good choices for patients with face involvement without biopsy ; they greatly help the diagnosis of ls if the patient refuses biopsy concerning the cosmetic impairment . the dermoscopy shows a whitish plaque with comedo - like openings on the surface of the lesion , while the confocal microscopy reveals hyperkeratosis , atrophy of the epidermis , dark and round structures containing bright amorphous material , and scattered inflammatory cells , as well as coarse collagen in bundles . all of these presences are correspond to the histopathologic features of epidermis atrophy , fibrosis of the upper dermis , and follicular plugging . however , it should also differentiate from morphea , vitiligo , atrophic lichen planus , and discoid lupus erythematosus . to date , there are numerous therapies including oral and topical corticosteroids , topical calcineurin inhibitors ( tcis ) , estrogen , retinoids , various vitamins , phototherapy , surgical excision , and emollients are also recommended as a supportive treatment . however , it is not easy to make it complete resolution of clinical signs and return to normal skin texture and color , but therapy can help alleviate symptoms and prevent further progression . oral corticosteroids combined with topical steroids or tcis might be helpful to control the progress of the lesion . hence , early diagnosis with dermoscopy or confocal microscopy and early treatment with oral corticosteroids combined with topical steroids or tcis might cure it without hypopigmentation and atrophy . what 's more , patients with classic ls of vulvar have about 0.3 - 4.9% risk developing squamous cell carcinoma ( scc ) . while extragenital ls is not associated with the development of scc , few publications referring to the long - term follow - up of ls patients . our review of the patients with ls involving face did not show any relationship with scc and other cutaneous tumors . compared with ls of vulva and mucosa , it may have less malignant potential . though we still need to follow - up our patients , this study is limited by the number of cases , for reasons that some may under reporting , misdiagnosis as other diseases and asymptomatic presentations . however , dermatologists should also pay attention to this rarely reported manifestation of ls and consider it as a differential diagnosis . early diagnosis with dermoscopy or confocal microscopy and early treatment might cure it without hypopigmentation and atrophy . it may have less malignant potential when compared with ls of vulva and mucosa , but we still need to offer long - term monitoring . our case demonstrated that though rare , face is also one presentation of ls , which we should not ignore . what 's more , dermoscopy or confocal microscopy may help us early diagnosis without invasiveness , and it may have less malignant potential . our case demonstrated that though rare , face is also one presentation of ls , which we should not ignore . what 's more , dermoscopy or confocal microscopy may help us early diagnosis without invasiveness , and it may have less malignant potential . our case demonstrated that though rare , face is also one presentation of ls , which we should not ignore . what 's more , dermoscopy or confocal microscopy may help us early diagnosis without invasiveness , and it may have less malignant potential .
we present a case of lichen sclerosus ( ls ) involving face and review of the relevant literature since 1970 . this article highlights the rarely reported manifestation of ls . early diagnosis with dermoscopy or confocal microscopy and early treatment might help cure it , and it may have less malignant potential .
secondary cardiac tumors are 20 to 40 times more frequent than primary cardiac malignancies.1 ) the most common secondary tumors of the heart originate from leukemia , melanoma , lung cancer , breast cancer , and lymphoma.2 ) cardiac involvement may arise from hematogenous metastases , direct invasion from the mediastinum , or tumor growth into the vena cava and extension into the right atrium ( ra ) . however , cardiac metastases from renal cell carcinoma ( rcc ) are rare , and in the absence of either direct vena caval or right cardiac extension , involvement of the left heart is extremely rare , with only a few known reports in the medical literature.3 ) a 77-year - old man , who was diagnosed with rcc with peritoneal seeding 2 months previously ( fig . he had been treated with sunitinib malate ( sutent , pfizer , korea ) for only 3 days because of severe general weakness and poor oral intake . at the time of admission , his vital signs were unstable ; his blood pressure was 78/46 mmhg , heart rate 136 beats per minute , and body temperature 36.7. laboratory tests showed a normal leukocyte count ( white blood cell , 7,450/l ) , and po of 72 mmhg and pco of 33 mmhg on arterial blood gas analysis . a 12-lead electrocardiogram revealed sinus tachycardia , and chest radiography findings included right hilar enlargement and reticular opacities in both lungs , in addition to cardiomegaly ( fig . chest computed tomography ( ct ) revealed a large left ventricular mass involving the lateral wall ; however , evidence of right ventricular or inferior venal caval involvement was not found ( fig . two - dimensional transthoracic echocardiography showed a very large , lobulated , oscillating mass attached to the lateral wall and occupying the cavity of the left ventricle ( lv ) ( fig . there was no evidence of metastasis to the inferior vena cava or ra ( fig . cardiac metastases from rcc , are extremely rare , and usually occur by either of two mechanisms . the first employs a venous hematogenous pathway through the renal vein to the right heart . in patients with isolated disease and delayed disease progression to the right heart , with no involvement of the inferior vena cava , microdissemination through a venous hematogenous pathway remains the most probable mode of metastasis.4 - 6 ) the second pattern is through the lymphatic vessels of the thorax , involving the carinal lymph nodes that collect the drainage from the posterior wall of the heart , and then , through reversed lymphatic flow caused by metastasis to the nodes , metastatic rcc can spread to the pericardium and the left myocardium . lymphatic drainage from the anterior and inferior walls of the heart passes through the parasternal and diaphragmatic lymph vessels . this hypothetical second pattern of rcc spread to the heart is supported by the observations that disseminated disease progression generally involves the supradiaphragmatic lymph nodes , lung , and pericardium , and the side of the heart more frequently involved is the left.6 - 8 ) the second pattern of spread is more compatible with our patient , because there was no involvement of the vena cava or ra , and at the time of diagnosis , a left ventricular mass causing lvot obstruction was identified . other possible causes of lvot obstruction include metastasis of unknown origin , myxoma , rhabdomyoma , and rhabomyosarcoma . although a biopsy of the left ventricular mass was not performed because of patient refusal , a secondary metastatic cardiac tumor of rcc is suspected , considering that there were multiple metastases in both lungs and the peritoneum . about one - third of patients diagnosed with rcc develop metastatic disease , and metastatic rcc can be diagnosed as a solitary metastatic lesion or as widespread systemic disease . a solitary metastatic lesion eventually develops into a systemic pattern of recurrent disease.9 ) although there have been advances in chemotherapy , the median survival time of patients with metastatic rcc is 6 to 12 months , and the 5-year survival rate is only 9%.10 ) metastatic rcc is highly resistant to chemotherapy , hormones , and radiotherapy , and the treatment of metastatic rcc remains ineffective . in the palliation of isolated , metastatic disease , surgical resection can play an important role . currently , combination therapy that includes surgery and chemotherapy is the best chance of palliation and cure . cardiac metastases from rcc in the absence of vena caval extension often grow slowly , tending to present many years after curative treatment . every attempt should be made to resect these slow - growing metastatic lesions when they involve the heart . coronary occlusion or compression from tumor masses can lead to myocardial infarction , eventual heart failure , and death . in our case , the patient refused surgical resection and had severe general weakness and cachexia ; therefore the aggressive surgical removal of the left ventricular mass was impossible . it is clear that although rare , renal cell cancer has the potential to metastasize to both the left and right ventricles , and cardiac metastasis should be considered in the clinical setting of renal cell cancer when the patient complains of sudden dyspnea of unknown origin and presents with sudden heart failure caused by lvot obstruction .
most cases of cardiac metastasis from renal cell carcinoma ( rcc ) involve the vena cava or right atrium . left ventricular metastases from rcc without involving the vena cava or right atrium are extremely rare . herein we report a case of rcc with left ventricular metastasis causing left ventricular outflow obstruction ( lvot ) .
the mission of icrh is to contribute to sexual and reproductive health and to strive for the recognition of sexual and reproductive health rights for every man and woman in every stage of their lives . icrh has divided this field into four clusters : hiv and sexually transmitted infections ( sti ) including human papilloma virus ( hpv ) with a particular focus on prevention ; maternal health including mother & child health , with specific attention for safe motherhood and family planning ; sexual and gender based violence ( sgbv ) , harmful traditional practices such as female genital mutilation ( fgm ) and forced / child marriage : integration of sexual and reproductive health and rights within health systems . in each of these four clusters , icrh performs a diversity of projects , most of them involving both scientific research and interventions . this combination allows for a cross - fertilization between science and action and contributes both to the societal relevance of the scientific research and the other way around- to the scientific underpinning of policy measures . reproductive health problems most often transcend the field of pure medicine , since they are deeply rooted in the fabric of communities and families , and hugely affected by societal organization , cultural values and economic circumstances . reproductive health research and action are therefore , if they aim to be effective , pre - eminently multidisciplinary ventures , and this is also how icrh s projects are devised . the research staff of icrh consists of a variety of experts , ranging from physicians , obstetricians and epidemiologists to anthropologists , lawyers and woman s health activists . the beneficial impact of this interdisciplinary way of working is shown by the multitude of field tools , policy advices and sensitization instruments that have resulted from the projects , as well as by the broad gamut of scientific magazines in which the research findings of icrh have been published . another key characteristic of icrh is its emphasis on international and cross - sectoral cooperation and networking . in the course of its 15 years of existence , icrh has built sustainable cooperation platforms with numerous organizations in all parts of the world and in all sectors : universities , ngos , public authorities , thematic networks , international institutions , private sector ? most projects are conducted in cooperation with several other groups , raising the expertise , know - how and creativity to a higher level than would be the case if only one single organization took forward the project . explaining how icrh works can best be done by giving some examples of its projects . we list below just a few examples , but a full list of references can be obtained on the website . momi ( missed opportunities in maternal and infant health ) : this project , funded by the european commission , started in 2011 , aiming at developing a package of interventions targeting the health of newborns and women in the early postpartum period and throughout the first year after childbirth . this package will be delivered through a combined facility- and community - based approach designed to integrate services and strengthen health systems . it is implemented in four african countries ( burkina faso , kenya , malawi and mozambique ) by a consortium consisting of several african and european partners . example 2 : kesho bora : a better future : this large multicentre study including a nested randomized controlled trial was implemented in five research sites in east , west and south africa . the overall aim of the study was to optimize the use of antiretroviral drugs during pregnancy , delivery and breastfeeding both to prevent mother - to - child transmission of hiv-1 ( pmtct ) and preserve the health of the hiv-1-infected mother in settings where the majority of women breastfeed . the findings from the study have been important for informing international and national pmtct guidelines in developing countries where hiv-1 infected women have little or no option other than to breastfeed their babies . the who guidelines on antiretroviral drugs for treating pregnant women and preventing hiv infection in infants : recommendations for a public health approach were importantly influenced by findings from the kesho bora study . example 3 : post abortion family planning : an estimated 8 million induced abortions are performed annually in china . however , the delivery of post abortion family planning ( pafp ) services is almost inexistent in china . in 2005 , icrh took the initiative , in collaboration with 5 other institutions to conduct the first study in china on the impact of post - abortion family planning services on contraceptive use and abortion rate . the main purpose of the study was to define optimal ways to introduce pafp services in urban areas in china in order to reduce the rate of unwanted pregnancies and induced abortions . desafio is an institutional university cooperation programme financed by the university development cooperation of the flemish university council ( vlir - uos ) and coordinated by icrh . the programme aims to strengthen the university eduardo mondlane ( uem ) as a developmental actor in mozambican society in the area of sexual and reproductive health ( srh ) and hiv / aids . the innovation of the programme lies primarily in the multi - disciplinary approach , combining medical , social , cultural and legal aspects of reproductive health , in the linkage between research and community outreach , and in the long - term approach gradually developing the local capacity and creating a sustainable environment . in terms of academic output , more than 300 peer reviewed scientific articles have been ( co- ) authored by the centre s researchers , more than 20 phd dissertations and more than 100 master dissertations have been promoted . in terms of organizational structure , icrh has followed a remarkable and quite innovative track . it has been established as an academic research institute within the structure of the department of gynecology , faculty of medicine and health sciences of ghent university , but through the nature of its activities it developed immediately strong links with communities and organizations in developing countries , which have been consolidated in a multitude of forms of cooperation , but also in the establishment of daughter ngos . currently , such organizations exist in mozambique and in kenya , and probably more will follow in other parts of the world . the daughter icrhs are autonomous local organizations established according to the legislation of their countries and governed by representatives of local stakeholder groups . in order to ensure close ties between all icrhs , an additional not - for - profit organization icrh global serves as a coordination platform between icrhs and in the future it will also develop networking and sensitization activities in the field of sexual and reproductive health . through its unusual structure , icrh as a group has placed itself on the crossroad of four key levels in sustainable development work : academic research , field action , global connectivity and local empowerment . in terms of funding , this challenging situation creates a rather insecure financial working environment , but on the other hand it forces icrh not only to apply a prudent and scrupulous financial management , but also to remain creative and innovative in its project development , and to stay in the vanguard of the scientific and societal thinking and acting in the field of reproductive health . icrh has proven in the last few years that it has been very successful in coping with this situation , as is shown by the fast growing number of projects . currently , icrh is coordinator of dozens of projects and programmes , some of them with budgets of several millions of dollars .
the international centre for reproductive health ( icrh ) was established by prof . dr . marleen temmerman in the aftermath of the un conference on population and development in cairo in 1994 . this conference called for world - wide action to improve the sexual and reproductive health situation of the global population in general and for vulnerable groups in particular , and this is exactly what icrh is striving for and has been working on for the last 15 years . icrh is a multidisciplinary centre of excellence , in research , capacity building and fieldwork in sexual and reproductive health and hiv prevention , and an advocate for sexual and reproductive health and rights . right from the start , icrh has opted for a global approach , which has resulted in a broad geographical spread of activities , with projects in africa , latin america , asia and europe . since its inception , icrh has participated in more than 120 projects , often as the coordinator , and through this work it has contributed considerably not only to scientific knowledge , improvement of health systems and increased accessibility of health services for vulnerable groups , but also to the quality of live of numerous individuals . since 2004 , icrh has been recognized as a who collaborating centre for research on sexual and reproductive health .
it is well - known that herpes simplex virus type 1 ( hsv-1 ) is the primary infectious cause of blindness in the united states . while hsv-1 infects corneal epithelial cells , disease presentation as herpetic stromal keratitis ( hsk ) is predominantly driven by immune responses to the replicating virus , particularly those responses mediated by cd4 t cells . these responses include th1 and th17 types and are mediated by interleukin-2 ( il-2 ) , gamma interferon , il-17 , and other cytokines . these effects may linger and persist long after the virus is no longer detected . that hsv-1 is capable of undergoing a subsequent latent infection , only to reactivate at the initial site of infection , repeated chronic leukocytic infiltration ultimately leads to irrevocable tissue damage . while recognition of this immunological component of the corneal pathogenesis might infer an anti - inflammatory intervention , the virulent nature of this infectious agent demands caution . accordingly , the recommended treatment for hsk involves frequent initial topical corticosteroid doses in combination with a modified nucleoside antiviral agent . the rationale behind this treatment is that the corticosteroid suppresses inflammation and the antiviral slows down the viral replication so that the patient s immune cells can clear the infection . the paradox here is that systemic immunosuppression is not an option in this situation , due to the potential for a life - threatening viral infection . it is this exact conundrum that jiang , yin , stuart , and lieb investigated in their recent mbio article ( 1 ) . jiang and colleagues reasoned that an alternative approach might be to limit signaling upstream of the cd4 t cell response . dcs are professional antigen - presenting cells required for optimal innate and adaptive immune responses ( fig . 1 ) . the team utilized an existing mouse strain with dcs knocked out for the atg5 locus , whose product plays a major role in membrane formation of the autophagosome . in control studies , the authors demonstrated that the absence of dc autophagy had limited effects on innate immunity , consistent with previous studies . importantly , cd4 t cell activation was indeed reduced in the dc autophagy - null animals . activation involves production of inflammatory cytokines and ultimately leads to an increase in b and t cells . jiang et al . showed that hsv-1-infected animals with autophagy - deficient dcs are reduced for cd4 t cell activation and cytokine production . while hsv-1 replicates slightly better in the corneal epithelia of these animals , there is less overall disease . these effects are noted with red arrows . adapted using data from references 2 and 3 . the key finding of this body of work is that following productive primary viral replication , corneal disease was significantly decreased when dc autophagy was absent . the authors therefore appear to have succeeded in threading the needle between dampening the cd4 t cell response to limit corneal disease without losing control of the viral infection . these results clearly dictate a need to more fully understand the mechanism through which dc autophagy regulates dc biology . it is conceivable that drug targets exist within the dc autophagy regulatory pathway that could be exploited to modulate the dc response . an intriguing implication of such finidngs is that future attempts at viral vaccine development might also utilize dc autophagy modulation as a mechanism to manipulate the immune response . jiang , yin , stuart , and lieb have provided us with some interesting observations to consider . additional biochemical and molecular genetic analyses will be required to define the specific details of the mechanism through which dc autophagy is regulated , so that these findings can potentially be leveraged in an attempt to reduce hsk disease .
abstractin a recent mbio article , y. jiang , x. yin , p. m. stuart , and d. a. lieb [ mbio 6(6):e01426 - 15 , 2015 , doi:10.1128/mbio.01426 - 15 ] presented an elegant set of experiments that utilized a transgenic , knockout strain of mice whose dendritic cells ( dcs ) are incapable of undergoing autophagy , to dissect out the aspects of the chronic inflammatory response following viral infection of corneal epithelial cells . the authors results provide a potential proof of concept that the dc autophagy pathway may be a valid target for therapeutic drug design in certain inflammatory pathologies .
aging is associated with adipose tissue increase , lean mass decrease and water content1 ( table 1 ) . angiotensin decrease and altered baroreceptorial function cause a minor and delayed response to brain thirst perception.2 other age - related changes are the inability of the kidneys to retain sodium and concentrate urine . this is due to a decrease in nephron numbers and a reduced sensitivity of renal receptors to antidiuretic hormone ( adh).3 furthermore , comorbidities , malnutrition , and poly - pharmacy cause a decrease in human homeostatic functions ; together , these factors cause a high morbidity and electrolytic imbalances in the elderly . an 87-year - old woman was hospitalized for the onset of confusion , tremors , an inability to walk , and a fever that she had been experiencing for 36 hours . she had a clinical history of arterial hypertension , cerebrovascular disease , colon diverticulosis , depression , and anxiety . she lived at home and was being treated with valsartan / hydrochlorothiazide 160/12.5 mg / day , furosemide 25 mg three times a week , aspirin 100 mg / day , bromazepam 2.5 mg / ml 12 drops at bedtime . she usually took high dosages of lactulose and sorbitol syrup as a laxative each day ( about 70 g / day ) . during her physical examination , the patient was confused and drowsy . she showed hyposthenia in her upper and lower limbs , symmetric and diffuse moderate hyporeflexia , her temperature was 37.8c . her heart rate was 94 beats per minute , rhythmic , arterial pressure was 90/60 mmhg in clinostatism ; a hypophonesis in her left lung basis was present , abdomen was meteoric , tender , painless , and no peripheral edema was present . laboratory tests revealed severe hyponatremia with marked hypokalemia , hypocalcemia , mild hypochloremia , and metabolic alkalosis . renal function was decreased ; high levels of transaminase , mild hypoalbuminemia , and neutrophil leukocytosis were present . cholinesterase , t - troponin , autoantibodies , aldosterone , basal cortisol , parathytoid hormone ( pth ) , and thyreostimulating hormone ( tsh ) were normal ( table 2 ) . potassium in 24-hour urine was inferior to 20 meq / l and sodium in 24-hour urine was inferior to 10 meq / l ; both of these results suggested a nonrenal cause of electrolyte loss . electrocardiogram revealed sinus rhytm , with sporadic supraventricular and ventricular extrasystoles , presence of u waves . a chest x - ray showed a circumscribed left basal effusion , a brain computed tomography scan showed chronic vasculopathy . the correction of hydroelectrolytic imbalances was the first treatment ; intravenous saline 2000 ml / day , potassium chlorure ( kcl ) 60 meq / day , sodium chlorure ( nacl ) 40 meq / day , calcium gluconate 10% 10 ml / day . ceftriaxone 2 g / day and levofloxacine 500 mg / day intravenously , human albumin 20% 50 ml every other day intravenously , enoxaparine 4000 international units ( iu)/day subcutaneously , were administered . during hospitalization the patient presented acute delirium and was treated with haloperidol 5 mg and prometazine chloridrate 25 mg intramuscularly . she was discharged after 12 days after resolution of symptoms and normalized laboratory tests ( table 2 ) . diagnosis on discharge was delirium in patient with abnormal use of laxative and severe electrolytic imbalance . the naranjo probability scale indicated a probable relationship between laxative use , electrolytic imbalances , and the onset of rhabdomyolysis ( score = 7).4 over - the - counter drugs such as laxatives are usually not considered to be dangerous . on the other hand , nearly one in every 25 individuals taking over - the - counter drugs are potentially at risk for a major drug drug interaction , as shown by studies in the literature.5 laxatives have an high osmotic power , resulting in the movement of great quantities of liquid and mineral salts in intestinal lumen.6 this very simple case report seems to be representative of the issues mentioned above . it reveals the importance of elderly care provided by dedicated operators , since inadequate treatments may cause unpleasant consequences on frail and very old people . rhabdomyolysis is a life - threatening event caused by crush injuries , natural disasters , ischemia , infections , stress exercise , but also by alcohol and drugs . a number of drugs may play a key role in determining rhabdomyolysis , either through a direct or indirect insult to sarcolemma . rhabdomyolysis is known to be a cause of hyperkalemia ; it may sometimes be associated to hypokalemia such as in our case report , with consequent cell wall alterations in ion transport and hyperpolarization.7 extracellular decrease in potassium potentiates negativity in membrane muscle cell fiber potential , normally 90 mv . the sodium potassium atpase pump has to be reset ; this is a metabolically active process which causes atp consumption . the alterations may derive from discrepancy between atp need and its availability . our patient s lack of compliance did not allow us to perform an electromyography in order to exclude wallerian degeneration . however , the absence of pain , caudocranial progression in the limbs , motor deficits , and regression of symptoms after appropriate treatment influenced our diagnosis.8 delirium , which is common in severely compromised elderly people , may be due to fever but especially to hyponatremia,3 which may cause intracellular cerebral edema . the velocity of deficit onset may be much more important than its severity.9 over - the - counter drugs such as laxatives may cause rhabdomyolysis through electrolytic imbalances . a careful monitoring of all the drugs taken by the elderly is one of the most important duties of a physician since drug interactions and their secondary effects may be fatal .
the present study describes a case of laxative - induced rhabdomyolysis in an elderly patient . an 87-year - old woman was hospitalized for the onset of confusion , tremors , an inability to walk , and a fever that she had been experiencing for 36 hours . she often took high dosages of lactulose and sorbitol syrup as a laxative ( about 70 g / day ) . during her physical examination , the patient was confused , drowsy , and she presented hyposthenia in her upper and lower limbs , symmetric and diffuse moderate hyporeflexia , and her temperature was 37.8c . laboratory tests revealed severe hyponatremia with hypokalemia , hypocalcemia , hypochloremia , and metabolic alkalosis . moreover , rhabdomyolysis markers were found . the correction of hydroelectrolytic imbalances with saline , potassium and sodium chlorure , calcium gluconate was the first treatment . during her hospitalization the patient presented acute delirium , treated with haloperidol and prometazine chloridrate intramuscularly . she was discharged 12 days later , after resolution of symptoms , and normalized laboratory tests . over - the - counter drugs such as laxatives are usually not considered dangerous ; on the other hand , they may cause serum electrolytic imbalance and rhabdomyolysis . a careful monitoring of all the drugs taken by the elderly is one of the most important duties of a physician since drug interactions and their secondary effects may be fatal .
every patient with acute respiratory distress syndrome ( ards ) is hypoxemic by definition . in these patients , mechanical ventilation ( mv ) is often life - saving . repetitive cyclic stretch however , results in regional overdistension or / and derecruitment which is associated with a number of severe complications termed ventilator - induced / associated lung injury ( vi / ali ) . the attributable mortality of vi / ali has been estimated to be at least 9% , and despite evidence that high concentrations of oxygen ( fractional inhaled concentrations of oxygen [ fio2 ] greater than 50% ) , can lead to hyperoxic acute lung injury ( hali ) , oxygen therapy remains a cornerstone of management . little is known about " permissive hypoxemia " and for the most part , clinicians will optimize positive end expiratory pressure ( peep ) to enable reductions in fio2 , accepting oxygen saturations in the mid to high 80 's . in the first three icu days , most ards patients are ventilated with average fio2 > 59% ( mean fio2 delivered on day 1 = 70% ) , but it is not uncommon for the most severely ill to require much higher fio2 concentrations ( 100% ) for prolonged periods or frequent intervals . in a previous issue of critical care , li and colleagues elucidate the potential mechanisms regulating interactions between injury cascades resulting from hyperoxia and high tidal volume ventilation . using gene - deficient models and specific inhibitors of intracellular signaling pathways , this author group demonstrate that the combination of hyperoxia and high tidal volume ventilation results in augmented lung injury , evidenced by indices of increased lung inflammation , microvascular permeability , and lung epithelial apoptotic cell death . the combined detrimental effect of oxygen and repetitive cyclic stretch was shown to result in the activation of specific intracellular signaling pathways . the paper by li and colleagues is part of a growing body of literature suggesting that the response of the mechanically ventilated lung to biochemical or biomolecular stimuli is profoundly altered by the coexistence of injurious stimuli that synergize at the cellular level as well as at the tissue level . more importantly , the findings suggest that interference or cooperation of signals may have critical physiological consequences such as activation of death pathways . studies on various model systems have shown that a relatively small number of transcription factors can set up strikingly complex spatial and temporal patterns of gene expression . this pattern creation is achieved mainly by means of combinatorial or differential gene regulation ; that is , regulation of a gene by two or more transcription factors simultaneously or under different conditions . li and colleagues offer insight into the specific molecular details of the mechanisms of combinatorial regulation of hyperoxia and high tidal volume . in their model , mitogen - activated protein kinase erk1/2 , c - jun nh2-terminal kinases , and downstream binding of the transcription factor ap-1 were responsible for orchestrating the molecular response and cellular physiological consequence of hali plus vi / ali whilst lung stretch alone is dependent on activation of the jnk pathway , high volume plus hyperoxia mediated its detrimental effect via jnk and erk 1/2 activation . despite a historical emphasis on nf-b - dependent inflammation - related genes as mediators of injury , li and colleagues ' paper suggests that the augmented response seen when high volume and hyperoxia coexist appears to be nf-b independent . the molecular implication from their paper is that individual stimuli exert intracellular effects via independent signaling pathways that may converge or diverge at specific molecular ' nodes ' or ' hubs ' critical control points and potential targets for therapy . moreover , molecules that were previously perceived as reflecting redundancy in the response represent a sophisticated system that probably depends on the ' message ' carried rather than the messenger . the clinical implications of deciphering injury specific intra - cellular signaling is that it provides novel insight into the potential for future molecular treatment of injury - specific stimuli . exposure to hyperoxia is a well - established model of lung injury characterized by the development of pulmonary edema and inflammation . the development of hyperoxic lung injury was until recently thought to require the generation of reactive oxygen species , which leads to alveolar epithelial and endothelial cell death by both apoptosis and necrosis . disturbance of cell - death pathways , either local ( pulmonary ) or distal ( kidney and intestines ) , has also been implicated in the pathogenesis of vi / ali and ensuing mof , and this disturbance is a key feature of the alveolar remodeling process during recovery from injury . in contrast to the literature on vi / ali , where increased expression of cytokines and chemokines has been thought to be related to increase epithelial cell apoptosis , in studies of hali , overexpression of cytokines or chemokines ( for example , tnf- , il-1 , il-6 , cxc - chemokine receptor 2 , and il-11 ) , growth factors ( for example , insulin growth factor and keratinocyte growth factor [ kgf ] ) , or the subunit of the na , k - atpase have been shown to protect animals from hyperoxia by attenuating death signals . moreover , this effect appears to be independent of potential antioxidant effects , since antioxidants by themselves do not reverse or prevent all of the manifestations of hali and since the ability of certain cytokines to inhibit hyperoxia - induced cell death is independent of major alterations in lung antioxidants . this evidence raises crucial questions about how different injury signal components are integrated ; what are the morbidity and mortality defining manifestations of vi / ali and/or hali and which , where and when should " outcome - defining " pathways be blocked . fio2 = fractional inhaled concentration of oxygen ; hali = hyperoxic acute lung injury ; il = interleukin ; nf = nuclear factor ; tnf = tumor necrosis factor ; vi / ali = ventilator - induced / associated lung injury .
in patients with acute respiratory distress syndrome ( ards ) , supportive therapy with mechanical ventilation and oxygen is often life saving . further acute lung injury however , is an unfortunate consequence of oxygen therapy as well as mechanical injury secondary to ventilator induced / associated lung injury ( vi / ali ) . in this issue of critical care , li et al . expand on the intra - cellular signaling pathways regulating interactions between injury cascades resulting from hyperoxia and high tidal volume ventilation . the findings , suggest that interference or cooperation of different signals may have critical consequences as evidenced by indices of increased lung inflammation , microvascular permeability , and lung epithelial apoptotic cell death .
multiple endocrine neoplasia type 1 ( men 1 ) is an autosomal dominant tumor syndrome arising from mutations of the men 1 tumor suppressor gene on chromosome 11q13 . furthermore , majority of these thymic epithelial tumors are thymic carcinoids and thymoma patients in men 1 is rare . only four cases of thymoma with men 1 were found when we performed a literature search using pubmed [ 36 ] . although thymic carcinomas often contain tumor cells having a neuroendocrine feature , thymomas with neuroendocrine differentiation are extremely rare [ 7 , 8 ] . herein , we report a case of thymoma ( world health organization [ who ] type b3 ) with neuroendocrine differentiation in men 1 . a 68-year - old man , who was already diagnosed as men 1 previously , was admitted to our hospital with gradually progressive muscle weakness . enhanced computed tomography ( ct ) and magnetic resonance imaging ( mri ) of the chest revealed an anterior mediastinal tumor ( figs . 1a and b ) and no other tumorous lesions were found . figure 1:enhanced ct ( a ) and mri ( b ) of the chest revealed an anterior mediastinal tumor . enhanced ct ( a ) and mri ( b ) of the chest revealed an anterior mediastinal tumor . histologically , the tumor cells show round to oval , hyperchromatic nuclei and the nucleoli of the tumor cells are inconspicuous . they proliferate with a solid nest - like pattern , and hassall 's bodies , vague individual keratinization and psammoma bodies and coarse calcification are seen in the center portion of the tumor nests ( fig . 3c ) . taken together , pathologists diagnosed of thymoma ( who type b3 , masaoka stage i ) with neuroendocrine differentiation combined with men 1 . hematoxylin and eosin staining.figure 3:immunohistochemical findings of the tumor for synaptophysin ( a ) , chromogranin ( b ) and cd56 ( c ) . immunohistochemical findings of the tumor for synaptophysin ( a ) , chromogranin ( b ) and cd56 ( c ) . after surgery , his symptom ( progressive muscle weakness ) unchanged and he was diagnosed as amyotrophic lateral sclerosis . at six post - operative months , there was no evidence of local recurrence or distant metastasis . first , majority of thymic epithelial tumors in men 1 are thymic carcinoids and patients with thymoma in men 1 is rare [ 36 ] . second , thymomas with neuroendocrine differentiation are extremely rare [ 7 , 8 ] . to the best of our knowledge , miller et al . reported a case of synchronous thymoma ( type b3 ) and thymic carcinoid in a woman with men 1 . kojima et al . reported a mixed - type invasive thymoma ( who histological type was not available ) with men 1 . de toma et al . reported a thymoma ( type b1 ) in men 1 . reported a case of myasthenia gravis and thymoma ( who histological type was not available ) in men 1 . discussed that the association of tumor of the thymus and parathyroid gland is not surprising as these structures have a common embryonic origin from the third pharyngeal pouch . this discussion is helpful to understand the association between thymic epithelial tumor and men 1 . examined possible genotype phenotype correlation in thymic carcinoid from men 1 and reported a high prevalence of truncating men 1 mutation in patients with thymic carcinoid . this might be a pathways of tumorigenesis of thymic carcinoids in men 1 at least in part . none of previously reported four thymomas in men 1 [ 36 ] showed neuroendocrine differentiation . however , reports of thymomas with neuroendocrine differentiation have been extremely rare [ 7 , 8 ] . the present case is a first case of thymoma with neuroendocrine differentiation in men 1 . park et al . also reported an atypical thymoma ( type b3 ) with neuroendocrine differentiation combined with hyperparathyroidism . although their case did not fulfill the diagnostic criteria of men 1 , their case might have a similar genetic background with the present case . it has been reported that men 1-related thymic carcinoids carry an ominous prognosis due to their aggressive nature and potential for metastasis , and they are associated with increased mortality in patients with men 1 . to date , it is unclear whether neuroendocrine differentiation itself is associated with increased malignancy in thymic epithelial tumors . further , long - term follow - up of thymomas with neuroendocrine differentiation will be necessary .
abstractthymic epithelial tumors occur in 15% of patients with multiple endocrine neoplasia type 1 ( men 1 ) . majority of these thymic epithelial tumors are thymic carcinoids and patients with thymoma in men 1 is rare . furthermore , thymoma with neuroendocrine differentiation was also rarely reported . herein , we report a 68-year - old man having type b3 thymoma with neuroendocrine differentiation in men 1 and to the best of our knowledge this is the first such case ever reported .
although the incidence of tracheoinnominate artery fistula ( tif ) has decreased , it is still one of the most dangerous complications after tracheostomy . almost 75% of patients die from a tif , even after surgical repair has been performed . therefore , it is doubtful whether surgical repair is an effective method for treating tif ; however , it is still considered to be the optimal treatment option for tif . recently , the endovascular intervention has become the first - line treatment modality for vascular diseases and has replaced the classical surgical approach in many areas . in some cases of tif , endovascular stent graft repair ( esgr ) was successfully used as the first - line treatment.1 as the long - term outcomes and complications of tif endovascular intervention have been rarely reported , it is still wondered that the endovascular intervention can be treated as a better modality than surgery . here , we present a rare case of recurrent tif due to stent graft fracture after esgr . a 14-year - old male patient who had been followed - up for cerebral palsy and epilepsy from birth was admitted to the emergency room with fever , increase in the cough and sputum , and fresh bloody secretion through the tracheostomy . the patient had history of tif developed in 10 days after tracheostomy and was treated with endovascular stent graft ( jo stent graft , jomed gmbh , rangendmgen , germany ; 12 mm/58 mm ) insertion of the innominate artery . on the initial chest x - ray , migrated stent fragments could be seen and stent fragmentation was presumed to be caused by stent graft fracture ( fig . hence , chest computed tomography was performed , and it revealed separate stent fragments in the innominate artery that was filled by thrombus . as the bloody secretion through the tracheostomy increased , an emergent operation was planned . ( a ) initial chest x - ray after endovascular stent graft repair ( arrow ) of the innominate artery . ( b ) chest x - ray shows the migrated stent fragments ( arrow ) that developed due to stent graft fracture under general anesthesia , the sternotomy was performed . initially , the proximal and distal portions of the innominate artery were dissected and ligated with sutures , and then the inflammatory and necrotic tissues around the tif were resected . the inflammatory lesion in the tracheal defect was excised and the end - to - end anastomosis was performed . the patient was given ventilatory support for 4 days , and he was transferred to the general ward on the 7th postoperative day ( pod ) and discharged on the 33th pod . ( a ) the endovascular stent fragments ( arrow ) could be seen through a tear in the innominate artery . ( b ) after placing the innominate artery laterally , the endotracheal tube ( arrow ) could be seen through the fistula in trachea . since deguch et al successfully performed esgr in a patient with tif , 14 tif cases , including our case , that were treated by endovascular intervention have been reported until 2012 in the world literature.1 2 3 4 5 6 7 most endovascular interventions were performed as the first - line treatment , except one case , in which endovascular intervention was performed as a bridge to surgical treatment.8 considering the fact that bleeding could be controlled in most of cases , esgr seems to be an optimal treatment option for a tif.2 3 4 5 however , based on different individual situations , esgr can not be considered to be superior to surgical repair and should be prudently considered depending on the case . to date , three cases immediately developed endoleak after stent graft insertion , and three cases were recurrent tif 's which were stent - related complications . of the three cases of endoleak , one case was recovered after operation and the other case was recovered after an additional esgr . however , the third patient died from hemorrhage even though an additional esgr was performed . of the three recurrent tif 's , two cases were developed due to aggravation of fistula by continuous pressure of the inserted stent , and one case due to stent graft fracture as mentioned in our case . first two patients died from rebleeding even though the operation was performed.4 5 6 7 8 although there were several reports of successful management in tif patient who underwent esgr,1 2 7 it is still difficult to decide whether esgr for tif is a permanent treatment of option due to critical postinterventional complications . as the long - term outcome of esgr in tif has been unclear , esgr should be prudently performed in a child or an adolescent who has a long life expectancy . in patient with life - threatening bleeding from tif , esgr could be a good treatment of option as a bridge to surgical treatment . besides , the planed surgical correction is the most ideal approach for dealing with postinterventional complications .
tracheoinnominate artery fistula ( tif ) is one of the most dangerous complications after tracheostomy , and fetal even after surgical repair . recently , endovascular stent has been introduced as an option for the treatment of tif . unfortunately , endovascular stent repair could not replace the surgery due to rare clinical reports about the long - term follow - up and complications of stent graft . more collection of clinical data are necessary for the evaluation of long - term results . we report a rare case of recurrent tif due to stent graft fracture after endovascular stent graft insertion in the treatment of tif , and reviewed the world literature .
it is the third most common extracranial childhood solid tumor with an annual incidence of 4 - 7/million . common sites of involvement are head and neck areas , genitourinary tract , extremities , trunk , retroperitoneum , and biliary tract . less than 15 cases of primary rms of the breast have been reported to the best of our knowledge . here , we report two cases of which one is a primary rms of the breast , whereas the other is metastatic rms from an orbital primary . a 16-year - old postmenarchal girl at posttreatment follow - up for rms presented with complaint of lump in the right breast . she was treated with chemotherapy based on international rhabdomyosarcoma study 4 ( irs 4 ) protocol and radiotherapy . after a disease - free interval of 4 months , a well - defined firm nodule of 2 1 cm was palpable in the upper outer quadrant of right breast with no palpable axillary or supraclavicular lymphadenopathy . ultrasonogram ( usg ) showed an ill - defined hypoechoic lesion [ figure 1 ] . complete metastatic workup including bone marrow study , bone scan , and computerized tomogram ( ct ) scan of the thorax was negative for metastasis . it has postacoustic enhancement with edge shadowing the patient was given six cycles of second - line chemotherapy with vincristine , topotecan , and cyclophosphamide followed by rt ( 41.4 gy/23 # ) to the breast . the patient continues to be disease free at the follow - up of 72 months from completion of the treatment . a 12-year - old premenarchal girl presented with a lump in the right breast of 5 months duration . on examination , the breast was enlarged and had a 9 cm 9 cm mass with the involvement of the nipple areolar complex [ figure 2 ] . multiple enlarged ipsilateral axillary nodes , the largest measuring 3 cm 3 cm , were present . metastatic workup including bone marrow study and positron emission tomography ( pet ) scan were negative . histopathology showed a viable residual tumor of size 3.5 cm 3.5 cm 2 cm . the patient received rt , 41.4 gy/23 # to the chest wall followed by boost of 10 gy/5 # to the tumor site . the patient is disease free at the follow - up of 35 months from the completion of treatment . enlarged right breast with the destruction of nipple areolar complex in a case of primary rms of the breast a 16-year - old postmenarchal girl at posttreatment follow - up for rms presented with complaint of lump in the right breast . she was treated with chemotherapy based on international rhabdomyosarcoma study 4 ( irs 4 ) protocol and radiotherapy . after a disease - free interval of 4 months , a well - defined firm nodule of 2 1 cm was palpable in the upper outer quadrant of right breast with no palpable axillary or supraclavicular lymphadenopathy . ultrasonogram ( usg ) showed an ill - defined hypoechoic lesion [ figure 1 ] . complete metastatic workup including bone marrow study , bone scan , and computerized tomogram ( ct ) scan of the thorax was negative for metastasis . it has postacoustic enhancement with edge shadowing the patient was given six cycles of second - line chemotherapy with vincristine , topotecan , and cyclophosphamide followed by rt ( 41.4 gy/23 # ) to the breast . the patient continues to be disease free at the follow - up of 72 months from completion of the treatment . a 12-year - old premenarchal girl presented with a lump in the right breast of 5 months duration . on examination , the breast was enlarged and had a 9 cm 9 cm mass with the involvement of the nipple areolar complex [ figure 2 ] . multiple enlarged ipsilateral axillary nodes , the largest measuring 3 cm 3 cm , were present . metastatic workup including bone marrow study and positron emission tomography ( pet ) scan were negative . histopathology showed a viable residual tumor of size 3.5 cm 3.5 cm 2 cm . the patient received rt , 41.4 gy/23 # to the chest wall followed by boost of 10 gy/5 # to the tumor site . the patient is disease free at the follow - up of 35 months from the completion of treatment . enlarged right breast with the destruction of nipple areolar complex in a case of primary rms of the breast the involvement of breast is rare , the primary involvement being extremely rare . in a series reported from an intergroup rms study , of the total 26 patients described , 19 had metastatic disease and the remaining 7 had primary disease in the breast . alveolar histology was common and was seen in 24 patients and embryonal variant was seen in 1 patient . rarely , it is reported in adults with the oldest patient known being 60 years old . among patients with metastatic rms , synchronous metastasis to the breast the commonest primary site for metastatic rms to the breast are extremities and head and neck areas . the tendency of rms to metastasize to breast in adolescent girls is thought to be related to the expression of insulin - like growth factor ( igf ) receptors in the tumor cells . the breast epithelium and stroma are known to express growth factors igf - i and igf - ii . the metastatic rms patient in our study had the primary in the orbit and presented with a very short disease - free interval of 4 months . there has been no previously reported case of metastasis to the breast from an orbital primary . after excluding metastatic disease elsewhere breast conserving surgery benign breast diseases are more common in this age group the diagnosis of primary rms is often delayed . our patient also experienced a delay in referral because the initial diagnosis by the primary physician was fibroadenoma . hence , mrm was performed after induction chemotherapy with the option of delayed breast reconstruction . rms of the breast is an aggressive malignancy , and long - term survival is approximately 50% in primary cases and around 20% among metastatic cases .
rhabdomyosarcoma ( rms ) is a common soft - tissue neoplasm in the pediatric age group . common locations are head and neck , genitourinary areas , trunk , and extremities . two pathologic variants of this malignancy are embryonal and alveolar . the involvement of breast is rare . herein , we report two cases of alveolar rms of the breast of which one is an isolated breast metastasis from an orbital primary , whereas the other is a primary rms of the breast . both the patients were treated with surgery followed by adjuvant chemotherapy and radiotherapy and are currently disease free at six and three year respectively , following completion of treatment .