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A composite follow-up of all previous assessments and investigations was undertaken. | [] | We received a boy 3-year-old boy with autistic disorder on hospital of pediatric ward A at university hospital Mohammed VI of Marrakesh. He has no family history of illness or autistic spectrum disorder. The history revealed that at 2 years of age. The child was diagnosed with a ‘‘severe communication disorder,'' with social interaction difficulties and sensory processing delay. A composite follow-up of all previous assessments and investigations was undertaken. Blood work was normal (thyroid-stimulating hormone (TSH), hemoglobin, mean corpuscular volume (MCV), and ferritin). Genetic testing was unremarkable (normal karyotype, negative for fragile X) and the magnetic resonance imaging (MRI) in search of a demyelinating attack of the white matter, electroencephalography (EEG), optometry assessment were also normal. At the conclusion of this composite assessment, the boy was given a primary diagnosis of autistic spectrum disorder. A plan was instituted including speech-language therapy, intensive individualized educational programming, and contact was encouraged with the Autism Society. For that purpose, the parents moved to morocco to be surrounded by family without any huge improvement. Upon direct questioning at initial presentation, the 3-year-old child was reported by the parents to have unexplained fatigue, Gastrointestinal symptoms included bloating, constipation and diarrhea. Psychiatric symptoms included a frequently depressed mood, disproportionate anger, and emotional lability. On exploring his history, the child was born from yemenite father and Moroccan mother in Germany at-term weighing 3500 g with an Apgar score of 9 at 1 minute and 10 at 5 minutes after an uneventful pregnancy and delivery. No concerns were present in the neonatal period. Development in the first 24 months of life appeared fine according to the parents-his motor skills seemed to progress normally and he achieved expected milestones. After 2 years of age, however, his language skills slowly began to regress. He also demonstrated a change in temperament as he started to whine repeatedly and to scream without provocation. Physical examination revealed a height and weight normal for age. He was uncommunicative, restless, and somewhat agitated. General examination was unremarkable other than dark rings around the eyes. There were no dysmorphic features evident. He had difficulty maintaining eye contact, and he appeared disinterested in what was taking place. No abnormality was found on abdominal assessment. Anti-tissue transglutaminase antibodies levels were 76 U (normal < 10). The patient underwent upper endoscopy as duodenal biopsy to confirm a celiac disease diagnosis. It shows a total villous atrophy corresponding to a stage 4 of Marsh classification. Given the positive screen for celiac disease (positive anti-tissue transglutaminase antibodies and results of duodenal biopsy), dietary intervention was immediately commenced. All gluten was eliminated from the boy's diet. Within 1 month, the boy's gastrointestinal symptoms were relieved and his behavior had changed. The mother reported that her boy became progressively more communicative. The child has continued on the gluten-free diet and has progressed well and remained healthy over the following 6 months.
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The Ecofit cup itself is associated with a reduced risk of dislocation. | [] | A 32-year-old female patient with Kniest syndrome presented at our department with a painful pseudarthrosis after femoral valgisation osteotomy 8 years ago. A Wagner cone stem and acetabular roof cup with a cemented Ecofit cup 2M (dual-articulation acetabular cup system) were implanted due to a dysplastic femur with a small medullary space. The Ecofit cup itself is associated with a reduced risk of dislocation. The patient was satisfied with the range of motion after hip endoprosthesis and reported a significant increase in quality of life. The patient is still comfortable with the hip prosthesis at the most recent follow-up 4 years after implantation. Follow-up radiographs over 4 years have not revealed any signs of loosening or migration, and no Trendelenburg sign was reported for the affected side.
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He was transferred to Medical Oncology department for subsequent management and was treated with chemotherapy. | [] | A 33-year man working in Gulf, presented with hemetemesis 15 days ago in the month of February this year. This was preceeded by low grade fever, bone pain, generalised bodyache, low back ache and generalised macular skin rashes. There was no history of abdominal pain, jaundice or features of chronic liver disease. He gave history of intake of analgesics for backache. After symptomatic treatment he returned back. He came to our OPD with Complete blood count which showed a haemoglobin of 8g/dl, total count 6700/mm3 platelet count 24000/mm3 and ESR 55mm/hr. On examination patient was pale with non pruritic macular rashes and mild splenomegaly. Residing in Middle East with background history of fever, backache, skin rash, splenomegaly and a low platelet count possibility of atypical infection like Brucellosis with NSAID induced gi bleed was considered. He was admitted for further evaluation and endoscopy done showed a nodular ulcerative lesions in the antrum of stomach and histopathologic examination confirmed adenocarcinoma of stomach. Liver function tests showed a high ALP (1536 U/l), elevated INR (2.15) though bilirubin, transaminases and serum proteins were normal. Brucella agglutination test was negative. Xray Chest was normal. Ultrasound of abdomen showed normal echotexture of liver, no SOL, normal portal vein and no IHBRD. CT scan of the abdomen revealed gross pylorus thickening with regional lymphadenopathy and skeletal metastasis with sclerotic foci involving vertebrae and pelvic bones and lytic areas in bilateral iliac blades with no liver metastasis or ascites. A skeletal survey was done which showed lytic lesions in the iliac and femoral bones. Bone marrow aspirate showed decreased red cells and platelets and marrow biopsy done was reported as metastasis from adenocarcinoma. Coagulation profile done was suggestive of DIC due to the raised levels of FDP (5,170 ng/dl) and a low fibrinogen \(152mg/dl). He was transferred to Medical Oncology department for subsequent management and was treated with chemotherapy. He succumbed to his illness within 10 months of diagnosis.
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Hysteroscopy results showed that there were grade 4 adhesions in the uterine cavum, with a pale colored connective tissue in the uterine cavum, which refers to the diagnosis of secondary amenorrhea suspect tuberculosis endometritis, while the result of tissue curettage was examined on the Anatomical Pathology department. | [
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The histopathology slides show a well-defined mass composed of bland looking spindle cells. | [] | The patient was a 35-year old female who presented with urinary frequency for 2 months. Her past medical history showed a nonsmoking history and no significant family history. The physical examination was unremarkable. She was treated as urinary tract infection, but there was no improvement in her symptoms. Urine analysis and culture were performed several times that was negative for infection. Other laboratory data were also insignificant. Through a routine evaluation, urinary system ultrasonography (US) revealed a bladder lesion measuring 7 mm. In order to confirm the diagnosis, magnetic resonance imaging (MRI) of the abdomen and pelvic cavity was done; it revealed a lesion in the dome of the bladder. To confirm the diagnosis and for histopathological examination, the patient underwent Transurethral Resection of Bladder Tumor (TURBT) in April 2018. Before starting the procedure, the urethra was dilated till Fr: 28; at first, the tumor location was specified by cystoscopy. Then, it was resected by monopolar cautery from superficial to deeper parts with removal of the muscle layers. Proper bleeders were taken and three way Foley catheter was applied for a continuous irrigation. The patient was transferred to the recovery room with complete stability. The histopathology slides show a well-defined mass composed of bland looking spindle cells. Subsequent immunohistochemistry (IHC) was done which showed diffuse immunoreactivity for S100, but other markers including smooth muscle actin (SMA), desmin, cytokeratin, CD34 and beta catenin were negative. Ki67 was 2-3%, so the diagnosis of schwannoma was confirmed. Then, the neck, abdomen, pelvic and chest computed tomography (CT) scans were done that were normal. Brain and spine MRI and physical examination showed no evidence of other schwannoma or evidence of neurofibromatosis. She had no history of neurofibromatosis in her family. Skin examination was normal and had no café au lait spot. She was not a case of von Recklinghausen disease.
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The patient's history revealed swellings and pain at knee joint for nearly last 20 years. | [
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Moreover we strengthened the dorsal inguinal wall, fixated the testis in a subdartos pouch and closed the wound. | [] | Male infant 9.5 month old was examined in the emergency department of our hospital because of an empty scrotum and the ipsilateral testis lying in a high iliac crest position. With the increase in intrabdominal pressure [crying etc.] there was an ever-increasing swelling with spiral course from the groin to ipsilateral iliac crest. On clinical examination, a reducible mass was present in the right ileac crest. The swelling looked like a spigelian hernia [4]. However no abdominal wall defect could be palpated at that area. Moreover the ipsilateral scrotum was empty and the testis could be palpated with difficulty lying in a high inguinal position. Ultrasonography showed a loop of herniated small bowel and an undescended testis on the right. At operation, under general anesthesia, an extended transverse inguinal incision was made. Upon entering the inguinal canal a large hernia sac was identified. This followed a course through the superficial inguinal ring, extended upwards to the anterior abdomninal wall below the subcutaneous plane and was fixed in the area of the right iliac crest. We noticed that the hernia orifice was in a higher than normal position 5 cm from the pubic bone. This bulk was easily reduced back to the abdomen along with an ipsilateral mobile intravaginal testis. After careful isolation of the sac at a length of 3 cm the ipsilateral testis was isolated. The vas and the vessels were intravaginal, the gubernaculum was fixed in an ectopic position and the dorsal inguinal wall was lax. We removed the large sac after separating the vas and vessels and the testis. Moreover we strengthened the dorsal inguinal wall, fixated the testis in a subdartos pouch and closed the wound. Patient stayed for a day in the hospital. No postoperative complications happened. Three years later the boy was operated for left hydrocele. The boy remains in perfect health now five years after the initial operation.
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A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. | [] | A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.
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After the first week of this treatment her headaches were slightly decreased by heaviness but the frequency was the same. | [
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Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. | [] | A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.
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At the age of 15 years, when the patient's sexual development was almost completed and her menstrual cycle occurred irregularly, her height was 129.8 cm, which is 4.71 standard deviations below the median for normal girls her age. | [] | We report the case of a 15-year-old Caucasian girl who was diagnosed with Silver-Russell syndrome at the age of four and a half years. Recombinant growth hormone was administered for 18 months without an appropriate increase in growth velocity. At the age of seven years, her serum growth hormone levels were high, and an insulin-like growth factor 1 generation test did not increase insulin-like growth factor 1 levels (baseline insulin-like growth factor 1 levels, 52 μg/L; reference range, 75 μg/L to 365 μg/L; and peak, 76 μg/L and 50 μg/L after 12 and 84 hours, respectively, from baseline). The genetic analysis showed that the patient was homozygous for the R217X mutation in the growth hormone receptor gene, which is characteristic of Laron syndrome. On the basis of these results, the diagnosis of primary growth hormone insensitivity syndrome was made, and recombinant insulin-like growth factor 1 therapy was initiated. The patient's treatment was well tolerated, but unexplained central hypothyroidism occurred at the age of 12.9 years. At the age of 15 years, when the patient's sexual development was almost completed and her menstrual cycle occurred irregularly, her height was 129.8 cm, which is 4.71 standard deviations below the median for normal girls her age.
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Three years later the boy was operated for left hydrocele. | [
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On exploring his history, the child was born from yemenite father and Moroccan mother in Germany at-term weighing 3500 g with an Apgar score of 9 at 1 minute and 10 at 5 minutes after an uneventful pregnancy and delivery. | [] | We received a boy 3-year-old boy with autistic disorder on hospital of pediatric ward A at university hospital Mohammed VI of Marrakesh. He has no family history of illness or autistic spectrum disorder. The history revealed that at 2 years of age. The child was diagnosed with a ‘‘severe communication disorder,'' with social interaction difficulties and sensory processing delay. A composite follow-up of all previous assessments and investigations was undertaken. Blood work was normal (thyroid-stimulating hormone (TSH), hemoglobin, mean corpuscular volume (MCV), and ferritin). Genetic testing was unremarkable (normal karyotype, negative for fragile X) and the magnetic resonance imaging (MRI) in search of a demyelinating attack of the white matter, electroencephalography (EEG), optometry assessment were also normal. At the conclusion of this composite assessment, the boy was given a primary diagnosis of autistic spectrum disorder. A plan was instituted including speech-language therapy, intensive individualized educational programming, and contact was encouraged with the Autism Society. For that purpose, the parents moved to morocco to be surrounded by family without any huge improvement. Upon direct questioning at initial presentation, the 3-year-old child was reported by the parents to have unexplained fatigue, Gastrointestinal symptoms included bloating, constipation and diarrhea. Psychiatric symptoms included a frequently depressed mood, disproportionate anger, and emotional lability. On exploring his history, the child was born from yemenite father and Moroccan mother in Germany at-term weighing 3500 g with an Apgar score of 9 at 1 minute and 10 at 5 minutes after an uneventful pregnancy and delivery. No concerns were present in the neonatal period. Development in the first 24 months of life appeared fine according to the parents-his motor skills seemed to progress normally and he achieved expected milestones. After 2 years of age, however, his language skills slowly began to regress. He also demonstrated a change in temperament as he started to whine repeatedly and to scream without provocation. Physical examination revealed a height and weight normal for age. He was uncommunicative, restless, and somewhat agitated. General examination was unremarkable other than dark rings around the eyes. There were no dysmorphic features evident. He had difficulty maintaining eye contact, and he appeared disinterested in what was taking place. No abnormality was found on abdominal assessment. Anti-tissue transglutaminase antibodies levels were 76 U (normal < 10). The patient underwent upper endoscopy as duodenal biopsy to confirm a celiac disease diagnosis. It shows a total villous atrophy corresponding to a stage 4 of Marsh classification. Given the positive screen for celiac disease (positive anti-tissue transglutaminase antibodies and results of duodenal biopsy), dietary intervention was immediately commenced. All gluten was eliminated from the boy's diet. Within 1 month, the boy's gastrointestinal symptoms were relieved and his behavior had changed. The mother reported that her boy became progressively more communicative. The child has continued on the gluten-free diet and has progressed well and remained healthy over the following 6 months.
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Brucella agglutination test was negative. | [] | A 33-year man working in Gulf, presented with hemetemesis 15 days ago in the month of February this year. This was preceeded by low grade fever, bone pain, generalised bodyache, low back ache and generalised macular skin rashes. There was no history of abdominal pain, jaundice or features of chronic liver disease. He gave history of intake of analgesics for backache. After symptomatic treatment he returned back. He came to our OPD with Complete blood count which showed a haemoglobin of 8g/dl, total count 6700/mm3 platelet count 24000/mm3 and ESR 55mm/hr. On examination patient was pale with non pruritic macular rashes and mild splenomegaly. Residing in Middle East with background history of fever, backache, skin rash, splenomegaly and a low platelet count possibility of atypical infection like Brucellosis with NSAID induced gi bleed was considered. He was admitted for further evaluation and endoscopy done showed a nodular ulcerative lesions in the antrum of stomach and histopathologic examination confirmed adenocarcinoma of stomach. Liver function tests showed a high ALP (1536 U/l), elevated INR (2.15) though bilirubin, transaminases and serum proteins were normal. Brucella agglutination test was negative. Xray Chest was normal. Ultrasound of abdomen showed normal echotexture of liver, no SOL, normal portal vein and no IHBRD. CT scan of the abdomen revealed gross pylorus thickening with regional lymphadenopathy and skeletal metastasis with sclerotic foci involving vertebrae and pelvic bones and lytic areas in bilateral iliac blades with no liver metastasis or ascites. A skeletal survey was done which showed lytic lesions in the iliac and femoral bones. Bone marrow aspirate showed decreased red cells and platelets and marrow biopsy done was reported as metastasis from adenocarcinoma. Coagulation profile done was suggestive of DIC due to the raised levels of FDP (5,170 ng/dl) and a low fibrinogen \(152mg/dl). He was transferred to Medical Oncology department for subsequent management and was treated with chemotherapy. He succumbed to his illness within 10 months of diagnosis.
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Brain and spine MRI and physical examination showed no evidence of other schwannoma or evidence of neurofibromatosis. | [
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The lateral edge of anterior rectus sheath incision was dissected off from the rectus muscle laterally up to semilunar line of rectus muscle in 1cm width. | [] | A 26-year-old pregnant patient with a history of two previous cesarean deliveries was referred to our tertiary clinic. The patient was having severe right sided abdominal pain with sudden onset. She was in the 18 th week of her pregnancy based on last menstrual period. Obstetric ultrasound measurements revealed a live pregnancy compatible with 18 weeks gestation without any abnormality. However, a 15x12cm in size edematous multicystic adnexal mass occupying the mid and upper right abdomen without vascularity was identified. The mass was suspected to originate from right ovary. It was pushing the pregnant uterus into the left and anterior direction. There was no ultrasonographic evidence of malignancy. Considering the severe sudden pain complaint and adnexal mass finding, explorative laparotomy was planned for the suspected diagnosis of adnexal torsion. A mediolateral (near vertical) skin incision was made originated from infra-umbilical level on the lateral side of right rectus muscle. Specifically, the starting point for this slightly oblique incision was at 3cm lateral to the midline and near to lateral one third of the rectus muscle. The skin and fascia incisions were approximately 4cm and 2.5cm in length, respectively and this difference was due to the thick subcutaneous fat tissue. The oblique paramedian incision of the anterior rectus sheath was approximately 2cm medial to the lateral border of the rectus muscle and it was extended to caudal-lateral direction, down to 1/2cm to the lateral border of rectus muscle. The lateral edge of anterior rectus sheath incision was dissected off from the rectus muscle laterally up to semilunar line of rectus muscle in 1cm width. Then, the rectus muscle was retracted medially to expose the posterior sheath of rectus (below the arcuate line, only the transversalis fascia and parietal peritoneum are exposed). An oblique incision was made on the posterior sheath of rectus and peritoneum behind the rectus muscle, in a projection of the anterior sheath incision. In this manner, the abdominal wall incisions were located at anterior and posterior sheaths of rectus muscle at least 1/2cm medial to the semilunar line and to the lateral border of rectus. In other words, incision was not made from fascial layers' merging point or by muscle splitting dissection. Therefore, traction forces from enlarging uterus in advancing pregnant abdomen could be minimized. A large edematous, necrotic and dark purple torsion of a right adnexal mass was observed. It was pulled out and was partially dissected from its surface layer following a 2cm incision. The cystic structure was aspirated and right salpingo-ooferectomy was performed. The operation was completed by avoiding the manipulation of the pregnant uterus. Frozen section pathology of the removed specimen was reported as benign (necrotic tissues, teratoma) and final pathology was mucinous cystadenoma. Follow up on the pregnant patient was uneventful a month later.
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The genetic analysis showed that the patient was homozygous for the R217X mutation in the growth hormone receptor gene, which is characteristic of Laron syndrome. | [
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Patient underwent chest CT scan with contrast which show evidence of thymoma benign tumor of the thymus that has been the possible causes of MG. | [
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Intraoral examination showed fair oral hygiene multiple fixed prosthodontic restorations and multiple carious teeth, multiple remaining roots and lipomatous tongue atrophy.
The patient was referred to her neurologist to provide a detailed history about her medical condition and to coordinate with him regarding the steroid dose take to avoid any complication during patient management. A consultation letter had been received from her neurologist explaining the patient medical condition which starts with difficulty in breathing, chewing and swallowing, there was no external ocular weakness, motor weakness, sphincter dysfunction, sensory deficits, muscle wasting and no signs of diplopia, dizziness, double vision, ptosis or any difficulty in maintaining balance. Patient underwent chest CT scan with contrast which show evidence of thymoma benign tumor of the thymus that has been the possible causes of MG. Diagnosis was confirmed with measuring serum level of acetylcholine receptor antibodies, later treatment plan had been formulated including drugs to control the condition and then thymectomy to avoid sever complication to the patient.
Before proceeding to the dental management of the case; CBC and blood chemistry had been requested form the patient. Creatinine level was below normal (42.23 umol/l), with normal levels of albumin, urea, uric acid, AST, ALT, cholesterol, triglycerides and bilirubin. Patient had elevated WBC “12.52 x 10 9 /L” and Platelet “453 x 10 9 /L” with reduction in RBC “4.4 x 10 9 /L” and Eosinophil count “0.07”. Consultation with patient neurologist regarding the steroid dose to reduce the susceptibility of developing adrenal crisis.
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Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. | [] | A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago. In the family history, her mother, sisters and cousins underwent a surgery for MNG. Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs. She never suffered from thyroid dysfunction. Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. The mass took the front and the two sides of the neck. Its surface was embossed and covered by a thin normal skin. There were some veins of the collateral circulation limited to the neck. The goiter measured 18 x 11 cm. The mass was firm, painless, and mobile with the swallowing movements. Lymphadenopathy research was difficult and found no palpable lymph nodes. The laboratory tests (T 3, T 4 and TSH) were normal. Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea. Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum. It took heterogeneously the contrast and then evocate a large MNG. The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx. The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward. The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum. Endotracheal intubation was relatively easy by the laryngoscope. The incision performed was a Kocher cervicotomy. There was a multinodular, hypervascularized goiter. Its lower end plunges behind the sternal manubrium. The larynx was deviated towards the right side. The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy. The retrosternal part of the goiter was released using the finger by the same incision. Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage. One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle. The operation was finished by double aspiration drainage. In the first hours after surgery, the patient developed a large cervical hematoma. She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding. The operation was completed with a double suction drainage. In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine. After 48 hours of hemodynamic support, the blood pressure stabilized and dobutamine was stopped. Histological study concluded in multinodular colloid goiter. The patient was discharged from the hospital after 20 days in good health.
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Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. | [] | It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.
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A 20-year-old right-handed female factory worker with no medical history is consulting in the emergency ward with an open trauma of the left hand resulting from an occupational accident. | [] | A 20-year-old right-handed female factory worker with no medical history is consulting in the emergency ward with an open trauma of the left hand resulting from an occupational accident. Her hand was crushed under a heavy object. Physical examination found an important swelling and obvious distortion at the dorsum of the hand associated with wounds of the ulnar and radial edges of the left hand. The neurovascular examination was normal; in particular no sensitive deficit in the median nerve area was noted. Plain radiographs with front and lateral views showed a dislocation of the three lesser fingers associated with fractures of the hamatum and the second metacarpal. The three lesser metacarpals and the distal fragment of the hamatum were medially displaced. The patient was immediately brought to the operating theatre. The treatment consisted of a reduction and stabilisation using a multiple carpo-metacarpal and cross inter-metacarpal pinning. The wound was cleaned up trimmed and sutured. The had and the wrist were immobilized with a splint for six weeks. The patient had an intense and regular rehabilitation program. At one year follow-up, the outcome is good: the patient is painless with good bone consolidation in right position, strictly normal range of motion (metacarpophalangeal 90°, proximal interphalagienne 100° and distal interphalageal 90°) and a 80% grip strength compared to the right side.
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Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. | [
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] | A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine
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New bone formation and granulation tissue rich in mononuclear inflammatory cells was revealed. | [] | A 12-year-old girl was referred to Department of Oral Surgery, with a complaint of pain and swelling in the left of maxilla that appeared 3 months ago. The swelling was slowly progressive, associated with pain. However, the patient did not refer any motor or sensory deficit. There was no family history of similar swelling.
Physical examination revealed a left maxillary swelling. There was no facial palsy. No cervical lymph node enlargement was seen. The oral examination showed a 4 cm x 5 cm, tender, compressible mass in the left maxilla, from the lateral incisor to the second premolar teeth. The tumor surface was smooth and red-purple. Several teeth were involved and displaced in the tumor mass. We noted the absence of canine. Vitality tests proved negative on the central and lateral incisor. Hight mobility of lateral incisor was noted. Orthopantomography and occlusal radiography showed a wide osteolytic area of the anterior left maxilla, extending from the first permanent molar to the central incisor. The canine was impacted. No root resorption was observed. The radiolucency was closely related to maxillary sinus and involving the nasal cavity. Haematological investigations showed normal serum calcium, phosphorus and parathormone (PTH) levels. The provisional diagnosis of benign tumor of the maxilla was made.
The patient underwent excision and curettage of the mass with extraction of the canine and lateral incisor tooth. The wound was closed with interrupted sutures. The post-operative histopathological report revealed multinuclear giant cells scattered randomly throughout the cellular and fibrous vascular-rich tissue. New bone formation and granulation tissue rich in mononuclear inflammatory cells was revealed. The giant cells were multinucleated with bland-appearing nuclei, and the background stromal cells displayed no evidence of atypical mitoses. A diagnosis of giant cell tumor was established.
During a 1-year serial clinical and radiological follow-up, there was no evidence of recurrence. The facial contour and masticatory function were well-preserved.
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The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. | [
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] | Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV.
The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.
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P test showed a negative result, because after 14 days of progesterone consumption the prothyra is not bleeding. | [] | A 33-year-old woman came to Department of Fertility, Endocrinology and Reproduction at Saiful Anwar public Hospital and consulted that she has not menstruated since 5 years ago (28 years old). Her initial menarche was at the age of 14 years and it is in accordance with the normal growth of a child at her age. The patient had a regular menstrual period of 5-7 days, she replaced the pads for 2-3 times / day, and had no menstrual pain. The patient has a history of injective contraceptive that was done every once a month in 2010, and after the injection, she menstruated for two months and then it stopped. Based on clinical examination and a physical examination, the patient was diagnosed with secondary amenorrhea. Investigations that were used are ultrasonography, thorax X-ray and CT Scan Head. Ultrasonography and thorax X-Ray showed that patient is suspected to have right pleural effusion. CT-Scan examination showed that bilateral ethmoidal sinusitis and pituitary gland was within normal limits. The treatment plan in this patient is P test therapy with Prothyra 1x10 mg for 7 days, with control on 2 weeks after the use. P test showed a negative result, because after 14 days of progesterone consumption the prothyra is not bleeding. The treatment is followed by a E+P test (Estrogen+Progesterone) with Estrogen 1x0.625 mg for 21 days and an addition of Progesterone 1x10 mg on 12th -21st days, while the control was performed one week after the drug runs out. Based on the E+P test, the obtained results were FSH: 8.71 MIU/mL, LH: 3.1 IU/L, Prolactin: 319.4 ng/mL. The E+P test showed a negative result, so it was necessary to do hysteroscopy and curettage for the uterus evaluation. Hysteroscopy results showed that there were grade 4 adhesions in the uterine cavum, with a pale colored connective tissue in the uterine cavum, which refers to the diagnosis of secondary amenorrhea suspect tuberculosis endometritis, while the result of tissue curettage was examined on the Anatomical Pathology department. Results of anatomical pathology hysteroscopy showed endometrial tissue with stromal looks granulomas with lymphocytes, histiocytes, epithelioid and Langhans multinucleated giant cells and no malignancy was found in this preparation, which showed that patient's diagnosis is secondary amenorrhea suspected tuberculosis endometritis. Based on the Anatomical Pathology hysteroscopy result, treatment was done by using anti-tuberculosis drug category I (FDC 1X3 tab) for 6 months. Anatomical pathology result through macroscopic observation showed visible network of approximately 0.3 cm and 0.4 cm in diameter with a grayish-white color, while the microscopic observations showed fibromuscular tissue at high cylindrical epithelium with polyps shaped vacuolar, and inflammatory granulomatic and aplastic cells were not found.
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The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. | [] | We report a case of a male near-term neonate with gestational age of 35 weeks. He was the third born child to a 27-year-old mother with no significant past history. He was derived from a third degree consanguineous marriage. He was born by means of normal vaginal delivery with a normal extra-uterine adaptation. Meconium-stained amniotic fluid was noted. His birth weight was 3030g (75 th Percentile). The baby had normal physical examination at birth aside transitory respiratory distress. He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. He had developed no symptoms during his hospitalization. The output's weight was 2400g which signify the loss of 630g.
Back home, he received exclusive maternal breastfeeding. On his fifth day of life, he presented multiple yellowish-watery stools (ab 12 times) and refuse feeding with apyrexia context. He had clinical signs of severe dehydration without fever or vomiting. He lost 930g relative to the birth's weight. The abdomen appeared neither tender nor distended, and the rest of his physical examination was normal. The laboratory findings revealed hypernatremia of 152mEq/L, hyperkalemia of 7,8mEq/L and severe metabolic acidosis: blood PH: 7,1, HCO3:4mEq/L. The osmotic gap was 45. There was no leukocytosis, and the serum level of C-reactive protein was normal. The infant was treated with intravenous fluids and electrolytes as well as antibiotics after obtaining blood and stool cultures, assuming an infectious etiology. After 26 hours of rehydration therapy, the dehydration resolved, however, a functional acute renal failure complicated by acidosis and hyperkalemia appeared. The chest radiograph showed "bat wings" pattern and the cardiothoracic index was 0, 45. The renal ultrasound showed discretely hyperechoic kidneys cortex and the absence of renal malformations.
On the 11 th day of life, he was transferred to the pediatric intensive care unit. He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter. The evolution was burdened by cholestasis due to the prolonged parenteral feeding and nosocomial infection by staphylococcus epidermidis. Since his admission, he had developed a slimy diarrhea with 3 to 4 stools per day. He presented a profuse diarrhea during each attempt to introduce enteral feeding by hydrolyzed milk formula. He was discharged after 32 days with output's weight 3250g.
The oesophago-gastro-duodenoscopy and duodenal biopsy showed partial villous atrophy associated to the brush border abnormalities. The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. In the submucosa, there were numerous Brunner glands. We find the existence of numerous hemorrhagic suffusions. The CD10 immunostain was positive. Thus, a diagnosis hypothesis of MVID was raised. The electron microscopic examination confirmed the diagnosis.
Mutation analysis of peripheral blood samples of the neonate revealed a mutation at the homozygous status of MYO5B gene.
The baby died, after 97 days of life, of septic shock and multiple organ failure.
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Therapeutic intervention: the patient was given a pulse of methyl-prednisolone 15 mg/kg/day for 3 days followed by oral prednisone 1 mg/kg/day, associated with intravenous cyclophosphamide 750 mg/m 2 /month for 6 months. | [] | A 31-year-old woman with no pathological history was admitted to our hospital for chest pain, bone and joint pain, hair loss and asthenia for the last 3 months. On physical examination, we found fever at 38°C, polypnea at 32 cycles/min, arterial hypertension at 160/90 mmHg, malar rash, synovitis of wrists, elbows and ankles, and edema on both legs. Diagnostic assessment: laboratory tests disclosed the following values: Increase level of erythrocyte sedimentation rate 120 mm/1 st hour, C reactive protein was 20 mg/l. The blood count showed hemoglobin at 9g/dl, MCV at 85 µ 3, lymphopenia at 400/mm 3, while platelets were normal 450 000/mm 3. Direct coombs test was negative. Serum Albumin was 25g/l; proteins level was low at 50g/l and proteinuria at 3.2g/day. Creatinine was normal. Anti-nuclear antibody ANA were positive 1/320 with homogeneous pattern, anti-dsDNA level was 160U/ml. Anti-cardiolipin IgG antibodies were positive 25UGPL. Rheumatoid Factor was negative. C3 and C4 complement fractions were low (0.2 g/l and 0.05 g/l). Chest x-ray, electrocardiogram and echocardiography were normal. Chest computed tomography (CT) scan showed proximal pulmonary embolism. Renal ultrasound was normal. Renal biopsy reveals immunocomplex nephritis, lupus nephritis, segmental mesangial proliferation, mild activity lupus nephritis class III (A/C). Before starting corticosteroids, we analyzed electrolytes. Serum calcium was elevated 132 mg/l with hyper-calciuria 479 mg/24 h. 25 OHD was normal. Alcaline phosphatase was 420 UI/l. the patient did not have any symptoms of hypercalcemia. Serum protein Electrophoresis showed polyclonal hypergammaglobulinaemia. Serum and urine immunofixation as well as Bence-Jones proteinuria were negative. Intact parathyroid hormone (iPTH) was high 628 pg/ml. CT scan and ultrasound of parathyroid imaging revealed a lower left parathyroid nodule measuring 2cm x 1 cm. Femoral and lumbar bone mineral density (BMD) showed osteoporosis (T-score: -2.6). In addition, we found multiple pelvic osteolytic lesions at CT scan. Other causes of hypercalcemia and bone lysis were excluded. The diagnosis in this case was SLE with lupus nephritis class III and anti-phospholipid syndrome, complicated by pulmonary embolism associated to primary hyperparathyroidism causing severe hypercalcemia, osteoporosis. Therapeutic intervention: the patient was given a pulse of methyl-prednisolone 15 mg/kg/day for 3 days followed by oral prednisone 1 mg/kg/day, associated with intravenous cyclophosphamide 750 mg/m 2 /month for 6 months. Mycophenolate mofetil 2 g/day was prescribed as a maintenance therapy of lupus nephritis with hydroxychloroquine at a dose of 400 mg/day. Treatment of pulmonary embolism was initiated with subcutaneous low molecular weight heparin (enoxaparin 0.1 ml/10 kg/12h) followed by antivitamin K. For Hypercalcemia, patient has received intravenous fluid with furosemide. She also required Alendronate 70 mg/week with Vitamin D 400u/day for osteoporosis. The left parathyroid gland was surgically removed. Histopathological examination revealed parathyroid adenoma. Her immediate postoperative parathyroid hormone level was 64 pg/ml with a calcium level of 98 mg/l. Follow-up and outcomes: forty-eight hours after the surgery she developed oral paresthesia. She had hypocalcemia at 72 mg/l. She required oral supplementation for few months. Additional investigations for multiple endocrine neoplasia were negative. The patient remained asymptomatic. Her SLE was calm without any relapse. Control of proteinuria was negative. Corticosteroids was dropped. The follow-up was 4 years.
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The imatinib was replaced by dasatinib at the dose of 100 mg per day. | [] | A 12-year-old girl presented an abdominal pain, high persistent fever of (40°C) and severe fatigue. The physical exam revealed pallor, hepatomegaly, and grade III splenomegaly. The investigation revealed an elevated white blood cell count (WBC): 545,000/mm³. PNN: 49,050/mm³, hemoglobin: 11.6g/dl, platelets: 328,000/mm³. The patient's peripheral blood smear revealed marked leukocytosis with a significant number of immature myeloid precursors and 21% blasts. The analysis of the BCR-ABL shows 94% in the peripheral blood. The patient was diagnosed with CML. She received a chemotherapy protocol made of hyperalkalinisation, allopurinol, hydroxyurea and a treatment based on imatinib at the dose of 400mg per day. Patient achieved complete hematological remission at three months and complete cytogenetic remission at 12 months. The patient was readmitted into our pediatric department with complains of severe headache, vomiting, pain and bilateral visual loss since three days. On examination, patient was afebrile and appeared pale. Blood pressure was 100/60mm/hg and the heart rate 98 beat/minute. There was no lymphadenopathy and the nervous system examination was normal. Intraocular muscle testing was normal; however, there was an exophthalmia in both eyes. The pupil was sluggish to react to light. Fundoscopy revealed bilateral stade III disc edema and hemorrhages of the retina. She underwent a brain computed tomography (CT) scan with the suspicion of leukemic involvement or any other intracranial event (cerebral edema); however, nothing was found. A subsequent bone marrow exam showed no morphologic or molecular evidence of CML or acute leukemia. Cytological evaluation of cerebrospinal fluid (CSF) was also negative. The patient had no other known pathology that could be held responsible for retina edema. We suggested that it was a side effect of imatinib so its administration was temporary interrupted. The patient was put on an anti-edema therapy (mannitol, dexamethasone). However, few months later, the patient presented in the emergency room with a headache and severe vomiting. She described a recurrence of the same character of headaches accompanied with weakness of extremities and seizures. Nervous system examination revealed neck rigidity, presence of meningeal signs and lower limb paraplegia. CT of the brain revealed subdural chronic hematoma and a serpenginous gyriform enhancement around the contusion in the right parietal and occipital lobes. Analysis of the CSF collected showed lymphoblasts in a WBC count of 11000cells/ml. Flow cytometry of the CSF showed that blast cells were positive for a cluster of differentiation markers (CD) (CD34, CD19, CD10, CD22 and partially positive for CD45) confirming CNS extramedullary lymphoid blast infiltration. A bone marrow aspiration was done to evaluate the status of her chronic myelogenous leukemia and result was consistent with a chronic phase. Fundus examination showed bilateral optic atrophy.
The diagnosis of extramedullary isolated CNS blast crises (lymphoid type) was based on the presence of blasts in CSF (confirmed by flow cytometry).
The patient received a high-dose systemic induction chemotherapy and intrathecal therapy (methotrexate, arabinoside and dexamethasone). The imatinib was replaced by dasatinib at the dose of 100 mg per day. She was treated with 24 Gy of whole-brain radiation therapy.
Allogenic stem cell transplantation was not feasible. We noticed a rapidly progressive amelioration in her neurological status after finishing systemic chemotherapy and physical therapy; however, the patient's visual loss showed no signs of improvement.
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She was also inscribed NSAID drugs. | [] | A 21 year old female patient with the diagnosis of SWS suffering from headaches admitted to our clinic. She had a 2 year history of frequent non-pulsating headaches. Her headache was relieving with non-steroidal anti-inflammatory drugs and was not worsening with physical activity. There was no nausea or aura like symptoms accompanying the headache. Headaches were lasting for hours. The pain was bilateral, generalized and pressing in quality. The family history for headache was negative. She had a history of seizures occurring in the fifteenth day of life described as attacks of tonic clonic contractions and that's when she was diagnosed with SWS. At the age of 6 she had a history of callosotomy to control her seizures. At the age of 18 during a laser treatment done in order to get rid of her port wine birthmark she had her first seizure since callosotomy. After that she was prescribed carbamazepine 400 mg at daily dose and never had a seizure since then. According to the story taken from her parents even though she had a normal development at infancy she barely graduated from elementary school and she's hardly literate. There was nothing significant on her family history except for her elder sister's port wine stain on her face. The elder sister had no feature of SWS and no researches were done regarding her stain. She was inscribed daily doses of ketiapin 25 mg for anxiety disorder and venlafaxine 75 mg for both anxiety disorder and the chronic headaches. She was also inscribed NSAID drugs. After the first week of this treatment her headaches were slightly decreased by heaviness but the frequency was the same. At her physical examination a facial nevus -occurred due to choroid angioma-on the right forehead, right eyelid, nasal wing and the cheek was observed. Intra oral examination showed a right sided overgrowth of gingiva. Gingival overgrowth was bright red in color and showed blanching on applying pressure suggesting angiomatous enlargement. On her extremities a mild asymmetry was visible. Her left arm and leg was slightly smaller in portions and showed hemiparesis both in the upper and lower extremities of the same size. On her ophthalmological evaluation she was diagnosed with glaucoma of the right eye. On her psychiatric examination she showed signs of anxiety disorder. Her neurological examination was not remarkable except for her hemiparesis. Cranial CT scans showed diffuse atrophy in the right hemisphere and irregular double-contoured gyriform cortical calcifications in the right occipital area. Gadolinium enhanced brain MRI revealed multiple dilated pial venous vascular structures on right hemisphere also with the diffuse atrophy on the same side. Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller than the left. In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization. She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded. Proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.
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About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. | [] | A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.
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The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.
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He was advised follow-up post completion of his stay at the designated COVID-19 center, but he has not yet reported back for follow-up. | [] | A 43-year-old non-diabetic Indian male reported to our outpatient department with chief complaints of cough with expectoration, chest pain, reduced appetite, fever with chills, and night sweats for two weeks. He also complained of breathlessness on exertion and had two episodes of blood in his sputum. The patient explained that the cough was continuous and was relieved after taking cough syrup. He also mentioned that the episodes of fever were initially intermittent and then daily for the last two weeks and were relieved after taking Paracetamol. The chest pain was localized to the middle of the chest and was aggravated on exertion. He was a businessman by profession with no history of smoking, alcoholism, or any other substance abuse. Also, there was no history of any contact of TB or COVID-19 in the family or close contacts. And there was no history of foreign travel in the recent past. But he had reported having traveled by a domestic airline about twenty days back. There was no history of weight loss or any other major illness in the past.
On examination his vitals were-pulse-108/minute, arterial BP-130/80 mm of Hg, respiratory rate of 30 breaths/minute, Sp02-899% on room air, temperature- 101-degree centigrade. His Sp02 fell by 70% on room air after waking. On auscultation, there was crepitation on the bilateral middle lobes of the lungs. Also, dyspnea on exertion was noted. The rest of the systemic examination was within normal limits.
Considering this as a probable case of TB with COVID-19 he was advised a chest radiograph with sputum microscopy (Ziehl Neelsen (ZN) staining for acid-fast bacilli), Cartridge-based nucleic acid amplification test (CBNAAT) of the sputum and other routine investigations. To check for the COVID-19 he was advised qualitative polymerase chain reaction (PCR) test from the oropharyngeal swab. The results were surprising with Mycobacterium tuberculosis detected on sputum fluorescent microscopy and were also confirmed by the CBNAAT. However, there was no resistance to Rifampicin. The results of the PCR were positive for RNA specific to SARS-CoV-2. Besides, the chest radiograph PA-view was suggestive of bilateral consolidations on the middle lobes of lungs with ill-defined borders. The other investigations revealed a low lymphocyte count (1x 10 9 /L) and increased levels of C-reactive protein (CRP) (57 mg/L), lactate dehydrogenase (LDH) (580 U/L), and erythrocyte sedimentation rate (ESR) (70 mm in the 1 st hour). Also, a sample for liquid culture (MGIT BACTEC) was sent to the Intermediate Reference Laboratory (IRL) which revealed the growth of Mycobacterium tuberculosis. Computed tomography was not performed as the diagnosis was established by other cheaper and faster methods and also the patient was unwilling for the same. All the other routine investigations were within normal limits.
He was referred to the nearest designated COVID-19 management center, where he was managed as per national guidelines. Besides, he was also started on an antitubercular treatment of four drugs as per the National Tuberculosis Elimination Program (NTEP) guidelines. He was advised follow-up post completion of his stay at the designated COVID-19 center, but he has not yet reported back for follow-up. Written informed consent was obtained from the patient for using clinical data and images for publication in this study.
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The swelling looked like a spigelian hernia [4]. | [
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] | Male infant 9.5 month old was examined in the emergency department of our hospital because of an empty scrotum and the ipsilateral testis lying in a high iliac crest position. With the increase in intrabdominal pressure [crying etc.] there was an ever-increasing swelling with spiral course from the groin to ipsilateral iliac crest. On clinical examination, a reducible mass was present in the right ileac crest. The swelling looked like a spigelian hernia [4]. However no abdominal wall defect could be palpated at that area. Moreover the ipsilateral scrotum was empty and the testis could be palpated with difficulty lying in a high inguinal position. Ultrasonography showed a loop of herniated small bowel and an undescended testis on the right. At operation, under general anesthesia, an extended transverse inguinal incision was made. Upon entering the inguinal canal a large hernia sac was identified. This followed a course through the superficial inguinal ring, extended upwards to the anterior abdomninal wall below the subcutaneous plane and was fixed in the area of the right iliac crest. We noticed that the hernia orifice was in a higher than normal position 5 cm from the pubic bone. This bulk was easily reduced back to the abdomen along with an ipsilateral mobile intravaginal testis. After careful isolation of the sac at a length of 3 cm the ipsilateral testis was isolated. The vas and the vessels were intravaginal, the gubernaculum was fixed in an ectopic position and the dorsal inguinal wall was lax. We removed the large sac after separating the vas and vessels and the testis. Moreover we strengthened the dorsal inguinal wall, fixated the testis in a subdartos pouch and closed the wound. Patient stayed for a day in the hospital. No postoperative complications happened. Three years later the boy was operated for left hydrocele. The boy remains in perfect health now five years after the initial operation.
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Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. | [
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] | Mr F.B a 70-year-old male patient presented in our institution because of the occurrence of a right epistaxis. First of all, the patient is not smoking and reported an aneurysm of the abdominal aorta surgically treated by prosthesis under plavix. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with right epistaxis and bloody rhinorrhea with right chronic eye watering. Nasal Endoscopy examination revealed a pinnkish burgeoning mass bleeding on contact and filling the right nostril vestibule. The patient didn't presents neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product with relatively little washing at the late time. Otherwise, there is no extension involvement at the level of the cavum and the pterygo palate fossa. This finding found also a crenate appearance of the posterior nasal mucosa especially of the middle turbinates and contralateral inferior turbinate with a left deviation of the nasal septum with nasal spur. Nasosinusal MRI shows a progressive increase in size of the tissue mass compared to the previously CT occupying the right nasal fossa on almost all of its height, measuring 4 × 2.8 × 3.5cm without extension within the cavum with probable invasion of the lower part of the nasolacrimal duct and mass effect on both the sinusonasal bone septum and the intersinusonasal septum. This finding shows ethmoidal sinus fluid retention. The appearance, even if it remains unspecific, is compatible with inverted papilloma. The patient underwent complete endoscopic removal of the tumor with ethmoidectomy and right maxillary antrostomy. The extemporaneous examination is in favor of a malignant tumor lesion. Histopathologic analysis shows a tumor proliferation made of polymorphic cells with major anisocytosis, most often located in lobules. There is sometimes a somewhat palisadic seat on the periphery of the tumor mass. The mitotic index is 10 mitoses per 10 high power fields. The immunohistochemical study of the tumor found that the cytokeratin KL1 and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive. Proliferation index Ki67 is of the order of 80%. Otherwise there is an absence of expression of PS100, MelanA, HMB45, desmin, p40, CD45 and EBV.
The clear positivity of cytokeratins formally excludes the diagnosis of olfactory neuroblastoma and leads to neuroendocrine carcinoma. Given the atypia, the mitotic index, and the Ki67 proliferation index, it is a high-grade large-cell neuroendocrine carcinoma. Extension assessment, comprising a PET SCAN, was negative. Postoperative treatment consisted in adjuvant polychemotherapy, with six cycles (cisplatin and etoposide), Followed by loco regional external radiotherapy. After 6 month follow-up, the patient was in complete clinical and radiological remission.
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Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. | [] | A 35-year-old mentally ill man was transferred from a local psychiatric hospital after attempted suicide by fire 3 days before admission. He acquired full-and partial-thickness injury in approximately 38% of his TBSA, including face and neck (5%), both upper extremities (8%), right thigh (4%), left thigh and leg (3%), and most of his anterior and posterior trunk (16%). The patient was hospitalized for 66 days and underwent five debridement surgeries. The psychiatric diagnosis was schizophrenia type ICD 10 F20.3, treated with Haloperidol 1.5 mg and Diazepam 5 mg daily. The patient was occasionally restrained due to rebellious and self-destruction behavior. The nutrition management of this patient was started after a week of hospitalization, which is considered late. In the initial nutrition assessment, the patient was 40 kg weight and 150 cm tall thus the body mass index (BMI) was 17,57 kg/m 2 or underweight. Gastrointestinal symptoms and signs were not found. Information regarding the patient's dietary intake in the last two weeks and weight change within the last 6 months could not be obtained, but his mother claimed that the patient seemed to lose weight because he refused any food that had been offered to him; he only smoked and drank coffee. Albumin level was 3.6 gr/L with imbalanced electrolyte levels. Signs of fluid accumulation were not found. According to the subjective global assessment (SGA) tool, the patient belonged to group C (severely malnourished). Energy and protein were given starting from 30 kcal/kg, increasing gradually to 50 kcal/kg, and 2 gp/kg divided into oral nutrition and oral nutrition supplements (ONS) with a 50:50 ratio. The patient was also given micronutrient supplementations, specifically vitamin A 100,000 units for the first week, vitamin C 100 mg 3 times a day, and zinc 20 mg once a day. During the early weeks of hospitalization, the patient's weight loss continued. At the end of the second week, his body weight was 36 kilograms while his serum albumin level was 2.7 gr/L. On the 28 th day, the patient's body weight was 33 kilograms while his serum albumin level was 2.1 gr/L. Energy and protein intake was maintained, but the ratio of oral intake and ONS was modified to 70:30. During the treatment, the patient's appetite was unstable. The patient was also hardly cooperative with enteral access. The patient often writhed in pain and became more aggressive. The patient did not respond well to analgesics and sedations. Partially parenteral nutrition (PN) enriched with branched-chain amino acid (BCAA) was administered to support protein needs. However, his BMI continued to decline, and on the 42 nd day, his body weight dropped to 32 kilograms with a serum albumin level of 2 gr/L. On the 66 th day, granulation tissues appeared on the wound bed. Considering the mental and nutrition status of the patient, it was decided not to continue with the skin graft surgery. The patient had lost 24% of body weight (BMI 11.96 kg/m 2) and the serum albumin level was 2.5 gr/L. The patient displayed severe muscle wasting and subcutaneous fat loss. However, the general and mental condition was relatively better. The patient was discharged from the hospital.
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Resuscitation measures were taken and an transfusion 1120 ml whole blood, red cell pellets 350 ml and 860 ml fresh frozen plasma have helped to management coagulopathy and anemia. | [
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] | Mrs B.S. 28 years old multiparous that we had received 02 hours after a vaginal delivery of a newborn alive weighing 2750 grams in a peripheral maternity 3 km away. It was referred for postpartum hemorrhage. His medical history was featureless.
On admission, she presented hypovolemic shock with blood pressure 76/44 mmHg, regular tachycardia at 136 beats per minute, polypnea to 39 cycles per minute. The obstetrical examination objectified an involuted uterus, an absence of vaginal and perineal cervical lesions, a fluid and red blood originating of endouterine. Furthermore, there was purpura. We therefore concluded a significant postpartum hemorrhage complicated by coagulopathy. Resuscitation measures were taken and an transfusion 1120 ml whole blood, red cell pellets 350 ml and 860 ml fresh frozen plasma have helped to management coagulopathy and anemia. Blood count at admission highlighted severe thrombocytopenia 23 0000 platelets and hemoglobin levels of 3.9 g/dl. The post natal care was also administered.
After seven days of hospitalization, she was going after an amendment to the clinical symptoms and normal laboratory tests. Twenty five days later, she returned to obstetric emergencies for significant bleeding with severe anemia with hemoglobin 9 g/dL without signs of hypovolemic shock. The gynecological examination was normal. The balance sheet of the coagulation was normal including prothrombin to 92% and a normal activated partial thromboplastin time. The treatments were based on oxytocin, tranexamic acid, etamsylate and strict regular monitoring. Transvaginal ultrasound coupled Doppler was normal. In the absence of arterial embolization, she was going with ambulatory monitoring. Two weeks later she presented again rebleeding requiring hospitalization resulting in a total hysterectomy after conditioning of the patient. Histological examination of the surgical specimen objectified in the cervix and uterine body the presence of arterial vessels proliferation homes large and small caliber associated with tissue hemorrhage. The postoperative course was simple.
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No abnormality was found on abdominal assessment. | [] | We received a boy 3-year-old boy with autistic disorder on hospital of pediatric ward A at university hospital Mohammed VI of Marrakesh. He has no family history of illness or autistic spectrum disorder. The history revealed that at 2 years of age. The child was diagnosed with a ‘‘severe communication disorder,'' with social interaction difficulties and sensory processing delay. A composite follow-up of all previous assessments and investigations was undertaken. Blood work was normal (thyroid-stimulating hormone (TSH), hemoglobin, mean corpuscular volume (MCV), and ferritin). Genetic testing was unremarkable (normal karyotype, negative for fragile X) and the magnetic resonance imaging (MRI) in search of a demyelinating attack of the white matter, electroencephalography (EEG), optometry assessment were also normal. At the conclusion of this composite assessment, the boy was given a primary diagnosis of autistic spectrum disorder. A plan was instituted including speech-language therapy, intensive individualized educational programming, and contact was encouraged with the Autism Society. For that purpose, the parents moved to morocco to be surrounded by family without any huge improvement. Upon direct questioning at initial presentation, the 3-year-old child was reported by the parents to have unexplained fatigue, Gastrointestinal symptoms included bloating, constipation and diarrhea. Psychiatric symptoms included a frequently depressed mood, disproportionate anger, and emotional lability. On exploring his history, the child was born from yemenite father and Moroccan mother in Germany at-term weighing 3500 g with an Apgar score of 9 at 1 minute and 10 at 5 minutes after an uneventful pregnancy and delivery. No concerns were present in the neonatal period. Development in the first 24 months of life appeared fine according to the parents-his motor skills seemed to progress normally and he achieved expected milestones. After 2 years of age, however, his language skills slowly began to regress. He also demonstrated a change in temperament as he started to whine repeatedly and to scream without provocation. Physical examination revealed a height and weight normal for age. He was uncommunicative, restless, and somewhat agitated. General examination was unremarkable other than dark rings around the eyes. There were no dysmorphic features evident. He had difficulty maintaining eye contact, and he appeared disinterested in what was taking place. No abnormality was found on abdominal assessment. Anti-tissue transglutaminase antibodies levels were 76 U (normal < 10). The patient underwent upper endoscopy as duodenal biopsy to confirm a celiac disease diagnosis. It shows a total villous atrophy corresponding to a stage 4 of Marsh classification. Given the positive screen for celiac disease (positive anti-tissue transglutaminase antibodies and results of duodenal biopsy), dietary intervention was immediately commenced. All gluten was eliminated from the boy's diet. Within 1 month, the boy's gastrointestinal symptoms were relieved and his behavior had changed. The mother reported that her boy became progressively more communicative. The child has continued on the gluten-free diet and has progressed well and remained healthy over the following 6 months.
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Patient had elevated WBC “12.52 x 10 9 /L” and Platelet “453 x 10 9 /L” with reduction in RBC “4.4 x 10 9 /L” and Eosinophil count “0.07”. | [] | A 28-year-old female patient presented to outpatient dental clinics for full mouth rehabilitation. On physical examination, the patient was awake, alert, oriented, and her blood pressure, temperature and heart rate were within normal limits. Upon taking medical history the patient revealed that was diagnosed with thymoma MG and on medication including pyridostigmine bromide 60 mg, azathioprine 50 mg and prednisone 20 mg.
Intraoral examination showed fair oral hygiene multiple fixed prosthodontic restorations and multiple carious teeth, multiple remaining roots and lipomatous tongue atrophy.
The patient was referred to her neurologist to provide a detailed history about her medical condition and to coordinate with him regarding the steroid dose take to avoid any complication during patient management. A consultation letter had been received from her neurologist explaining the patient medical condition which starts with difficulty in breathing, chewing and swallowing, there was no external ocular weakness, motor weakness, sphincter dysfunction, sensory deficits, muscle wasting and no signs of diplopia, dizziness, double vision, ptosis or any difficulty in maintaining balance. Patient underwent chest CT scan with contrast which show evidence of thymoma benign tumor of the thymus that has been the possible causes of MG. Diagnosis was confirmed with measuring serum level of acetylcholine receptor antibodies, later treatment plan had been formulated including drugs to control the condition and then thymectomy to avoid sever complication to the patient.
Before proceeding to the dental management of the case; CBC and blood chemistry had been requested form the patient. Creatinine level was below normal (42.23 umol/l), with normal levels of albumin, urea, uric acid, AST, ALT, cholesterol, triglycerides and bilirubin. Patient had elevated WBC “12.52 x 10 9 /L” and Platelet “453 x 10 9 /L” with reduction in RBC “4.4 x 10 9 /L” and Eosinophil count “0.07”. Consultation with patient neurologist regarding the steroid dose to reduce the susceptibility of developing adrenal crisis.
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There was no lymphadenopathy and the nervous system examination was normal. | [] | A 12-year-old girl presented an abdominal pain, high persistent fever of (40°C) and severe fatigue. The physical exam revealed pallor, hepatomegaly, and grade III splenomegaly. The investigation revealed an elevated white blood cell count (WBC): 545,000/mm³. PNN: 49,050/mm³, hemoglobin: 11.6g/dl, platelets: 328,000/mm³. The patient's peripheral blood smear revealed marked leukocytosis with a significant number of immature myeloid precursors and 21% blasts. The analysis of the BCR-ABL shows 94% in the peripheral blood. The patient was diagnosed with CML. She received a chemotherapy protocol made of hyperalkalinisation, allopurinol, hydroxyurea and a treatment based on imatinib at the dose of 400mg per day. Patient achieved complete hematological remission at three months and complete cytogenetic remission at 12 months. The patient was readmitted into our pediatric department with complains of severe headache, vomiting, pain and bilateral visual loss since three days. On examination, patient was afebrile and appeared pale. Blood pressure was 100/60mm/hg and the heart rate 98 beat/minute. There was no lymphadenopathy and the nervous system examination was normal. Intraocular muscle testing was normal; however, there was an exophthalmia in both eyes. The pupil was sluggish to react to light. Fundoscopy revealed bilateral stade III disc edema and hemorrhages of the retina. She underwent a brain computed tomography (CT) scan with the suspicion of leukemic involvement or any other intracranial event (cerebral edema); however, nothing was found. A subsequent bone marrow exam showed no morphologic or molecular evidence of CML or acute leukemia. Cytological evaluation of cerebrospinal fluid (CSF) was also negative. The patient had no other known pathology that could be held responsible for retina edema. We suggested that it was a side effect of imatinib so its administration was temporary interrupted. The patient was put on an anti-edema therapy (mannitol, dexamethasone). However, few months later, the patient presented in the emergency room with a headache and severe vomiting. She described a recurrence of the same character of headaches accompanied with weakness of extremities and seizures. Nervous system examination revealed neck rigidity, presence of meningeal signs and lower limb paraplegia. CT of the brain revealed subdural chronic hematoma and a serpenginous gyriform enhancement around the contusion in the right parietal and occipital lobes. Analysis of the CSF collected showed lymphoblasts in a WBC count of 11000cells/ml. Flow cytometry of the CSF showed that blast cells were positive for a cluster of differentiation markers (CD) (CD34, CD19, CD10, CD22 and partially positive for CD45) confirming CNS extramedullary lymphoid blast infiltration. A bone marrow aspiration was done to evaluate the status of her chronic myelogenous leukemia and result was consistent with a chronic phase. Fundus examination showed bilateral optic atrophy.
The diagnosis of extramedullary isolated CNS blast crises (lymphoid type) was based on the presence of blasts in CSF (confirmed by flow cytometry).
The patient received a high-dose systemic induction chemotherapy and intrathecal therapy (methotrexate, arabinoside and dexamethasone). The imatinib was replaced by dasatinib at the dose of 100 mg per day. She was treated with 24 Gy of whole-brain radiation therapy.
Allogenic stem cell transplantation was not feasible. We noticed a rapidly progressive amelioration in her neurological status after finishing systemic chemotherapy and physical therapy; however, the patient's visual loss showed no signs of improvement.
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It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. | [] | It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.
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The physical examination was unremarkable. | [] | The patient was a 35-year old female who presented with urinary frequency for 2 months. Her past medical history showed a nonsmoking history and no significant family history. The physical examination was unremarkable. She was treated as urinary tract infection, but there was no improvement in her symptoms. Urine analysis and culture were performed several times that was negative for infection. Other laboratory data were also insignificant. Through a routine evaluation, urinary system ultrasonography (US) revealed a bladder lesion measuring 7 mm. In order to confirm the diagnosis, magnetic resonance imaging (MRI) of the abdomen and pelvic cavity was done; it revealed a lesion in the dome of the bladder. To confirm the diagnosis and for histopathological examination, the patient underwent Transurethral Resection of Bladder Tumor (TURBT) in April 2018. Before starting the procedure, the urethra was dilated till Fr: 28; at first, the tumor location was specified by cystoscopy. Then, it was resected by monopolar cautery from superficial to deeper parts with removal of the muscle layers. Proper bleeders were taken and three way Foley catheter was applied for a continuous irrigation. The patient was transferred to the recovery room with complete stability. The histopathology slides show a well-defined mass composed of bland looking spindle cells. Subsequent immunohistochemistry (IHC) was done which showed diffuse immunoreactivity for S100, but other markers including smooth muscle actin (SMA), desmin, cytokeratin, CD34 and beta catenin were negative. Ki67 was 2-3%, so the diagnosis of schwannoma was confirmed. Then, the neck, abdomen, pelvic and chest computed tomography (CT) scans were done that were normal. Brain and spine MRI and physical examination showed no evidence of other schwannoma or evidence of neurofibromatosis. She had no history of neurofibromatosis in her family. Skin examination was normal and had no café au lait spot. She was not a case of von Recklinghausen disease.
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He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. | [] | We report a case of a male near-term neonate with gestational age of 35 weeks. He was the third born child to a 27-year-old mother with no significant past history. He was derived from a third degree consanguineous marriage. He was born by means of normal vaginal delivery with a normal extra-uterine adaptation. Meconium-stained amniotic fluid was noted. His birth weight was 3030g (75 th Percentile). The baby had normal physical examination at birth aside transitory respiratory distress. He had been hospitalized for four days in a private clinic, for the suspicion of materno-foetal infection, where he had antibiotics for 48 hours. He had developed no symptoms during his hospitalization. The output's weight was 2400g which signify the loss of 630g.
Back home, he received exclusive maternal breastfeeding. On his fifth day of life, he presented multiple yellowish-watery stools (ab 12 times) and refuse feeding with apyrexia context. He had clinical signs of severe dehydration without fever or vomiting. He lost 930g relative to the birth's weight. The abdomen appeared neither tender nor distended, and the rest of his physical examination was normal. The laboratory findings revealed hypernatremia of 152mEq/L, hyperkalemia of 7,8mEq/L and severe metabolic acidosis: blood PH: 7,1, HCO3:4mEq/L. The osmotic gap was 45. There was no leukocytosis, and the serum level of C-reactive protein was normal. The infant was treated with intravenous fluids and electrolytes as well as antibiotics after obtaining blood and stool cultures, assuming an infectious etiology. After 26 hours of rehydration therapy, the dehydration resolved, however, a functional acute renal failure complicated by acidosis and hyperkalemia appeared. The chest radiograph showed "bat wings" pattern and the cardiothoracic index was 0, 45. The renal ultrasound showed discretely hyperechoic kidneys cortex and the absence of renal malformations.
On the 11 th day of life, he was transferred to the pediatric intensive care unit. He needed mechanical ventilation for three days and exclusive parenteral feeding by central catheter. The evolution was burdened by cholestasis due to the prolonged parenteral feeding and nosocomial infection by staphylococcus epidermidis. Since his admission, he had developed a slimy diarrhea with 3 to 4 stools per day. He presented a profuse diarrhea during each attempt to introduce enteral feeding by hydrolyzed milk formula. He was discharged after 32 days with output's weight 3250g.
The oesophago-gastro-duodenoscopy and duodenal biopsy showed partial villous atrophy associated to the brush border abnormalities. The periodic acid Schiff (PAS) stain revealed abnormalities in the brush border characterized by loss of the linear part of enterocytes with the presence of intra-cytoplasmic PAS + band in the apical pole of enterocytes uneven thickness and sometimes with double contours pattern. In the submucosa, there were numerous Brunner glands. We find the existence of numerous hemorrhagic suffusions. The CD10 immunostain was positive. Thus, a diagnosis hypothesis of MVID was raised. The electron microscopic examination confirmed the diagnosis.
Mutation analysis of peripheral blood samples of the neonate revealed a mutation at the homozygous status of MYO5B gene.
The baby died, after 97 days of life, of septic shock and multiple organ failure.
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The vas and the vessels were intravaginal, the gubernaculum was fixed in an ectopic position and the dorsal inguinal wall was lax. | [] | Male infant 9.5 month old was examined in the emergency department of our hospital because of an empty scrotum and the ipsilateral testis lying in a high iliac crest position. With the increase in intrabdominal pressure [crying etc.] there was an ever-increasing swelling with spiral course from the groin to ipsilateral iliac crest. On clinical examination, a reducible mass was present in the right ileac crest. The swelling looked like a spigelian hernia [4]. However no abdominal wall defect could be palpated at that area. Moreover the ipsilateral scrotum was empty and the testis could be palpated with difficulty lying in a high inguinal position. Ultrasonography showed a loop of herniated small bowel and an undescended testis on the right. At operation, under general anesthesia, an extended transverse inguinal incision was made. Upon entering the inguinal canal a large hernia sac was identified. This followed a course through the superficial inguinal ring, extended upwards to the anterior abdomninal wall below the subcutaneous plane and was fixed in the area of the right iliac crest. We noticed that the hernia orifice was in a higher than normal position 5 cm from the pubic bone. This bulk was easily reduced back to the abdomen along with an ipsilateral mobile intravaginal testis. After careful isolation of the sac at a length of 3 cm the ipsilateral testis was isolated. The vas and the vessels were intravaginal, the gubernaculum was fixed in an ectopic position and the dorsal inguinal wall was lax. We removed the large sac after separating the vas and vessels and the testis. Moreover we strengthened the dorsal inguinal wall, fixated the testis in a subdartos pouch and closed the wound. Patient stayed for a day in the hospital. No postoperative complications happened. Three years later the boy was operated for left hydrocele. The boy remains in perfect health now five years after the initial operation.
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Hormonal study and dynamic biochemical tests performed indicated ECS. | [] | A 46-year-old man with hypertension and dyslipidemia diagnosed 4-months before, as well as new-onset diabetes mellitus unveiled 1-month earlier, was referred to emergency department for hypokalemia. Hormonal study and dynamic biochemical tests performed indicated ECS. Imaging and cytological findings pointed toward a likely primary right parotid malignancy with liver metastases. Somatostatin receptor scintigraphy has shown an increased uptake in the parotid gland and mild expression in liver metastasis. The patient underwent right parotidectomy, and histopathologic examination confirmed ACC. Meanwhile, hypercortisolism was managed with metyrapone, ketoconazole, and lanreotide. Despite chemotherapy onset, a rapid disease progression and clinical course deterioration was observed.
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Echocardiogram and electrocardiogram were normal. | [] | A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine
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He denied having any prior episodes of acute pancreatitis and had no history of alcohol use. | [
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] | A 21 year old female patient with the diagnosis of SWS suffering from headaches admitted to our clinic. She had a 2 year history of frequent non-pulsating headaches. Her headache was relieving with non-steroidal anti-inflammatory drugs and was not worsening with physical activity. There was no nausea or aura like symptoms accompanying the headache. Headaches were lasting for hours. The pain was bilateral, generalized and pressing in quality. The family history for headache was negative. She had a history of seizures occurring in the fifteenth day of life described as attacks of tonic clonic contractions and that's when she was diagnosed with SWS. At the age of 6 she had a history of callosotomy to control her seizures. At the age of 18 during a laser treatment done in order to get rid of her port wine birthmark she had her first seizure since callosotomy. After that she was prescribed carbamazepine 400 mg at daily dose and never had a seizure since then. According to the story taken from her parents even though she had a normal development at infancy she barely graduated from elementary school and she's hardly literate. There was nothing significant on her family history except for her elder sister's port wine stain on her face. The elder sister had no feature of SWS and no researches were done regarding her stain. She was inscribed daily doses of ketiapin 25 mg for anxiety disorder and venlafaxine 75 mg for both anxiety disorder and the chronic headaches. She was also inscribed NSAID drugs. After the first week of this treatment her headaches were slightly decreased by heaviness but the frequency was the same. At her physical examination a facial nevus -occurred due to choroid angioma-on the right forehead, right eyelid, nasal wing and the cheek was observed. Intra oral examination showed a right sided overgrowth of gingiva. Gingival overgrowth was bright red in color and showed blanching on applying pressure suggesting angiomatous enlargement. On her extremities a mild asymmetry was visible. Her left arm and leg was slightly smaller in portions and showed hemiparesis both in the upper and lower extremities of the same size. On her ophthalmological evaluation she was diagnosed with glaucoma of the right eye. On her psychiatric examination she showed signs of anxiety disorder. Her neurological examination was not remarkable except for her hemiparesis. Cranial CT scans showed diffuse atrophy in the right hemisphere and irregular double-contoured gyriform cortical calcifications in the right occipital area. Gadolinium enhanced brain MRI revealed multiple dilated pial venous vascular structures on right hemisphere also with the diffuse atrophy on the same side. Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller than the left. In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization. She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded. Proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.
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Yet, the patient remained febrile. | [] | A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.
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Cultures for bacteria and mycobacteria gave no growth. | [] | A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine
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] | A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago. In the family history, her mother, sisters and cousins underwent a surgery for MNG. Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs. She never suffered from thyroid dysfunction. Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification. The mass took the front and the two sides of the neck. Its surface was embossed and covered by a thin normal skin. There were some veins of the collateral circulation limited to the neck. The goiter measured 18 x 11 cm. The mass was firm, painless, and mobile with the swallowing movements. Lymphadenopathy research was difficult and found no palpable lymph nodes. The laboratory tests (T 3, T 4 and TSH) were normal. Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea. Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum. It took heterogeneously the contrast and then evocate a large MNG. The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx. The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward. The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum. Endotracheal intubation was relatively easy by the laryngoscope. The incision performed was a Kocher cervicotomy. There was a multinodular, hypervascularized goiter. Its lower end plunges behind the sternal manubrium. The larynx was deviated towards the right side. The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy. The retrosternal part of the goiter was released using the finger by the same incision. Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage. One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle. The operation was finished by double aspiration drainage. In the first hours after surgery, the patient developed a large cervical hematoma. She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding. The operation was completed with a double suction drainage. In the immediate postoperative period, the patient developed hemodynamic collapse requiring the introduction of dobutamine. After 48 hours of hemodynamic support, the blood pressure stabilized and dobutamine was stopped. Histological study concluded in multinodular colloid goiter. The patient was discharged from the hospital after 20 days in good health.
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Urgent cardiac catheterization revealed focal dissections in four small caliber coronary arteries in right and left coronary systems including right posterior decending, posterolateral, obtuse marginal and septal arteries. | [
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Histopathological examination revealed parathyroid adenoma. | [] | A 31-year-old woman with no pathological history was admitted to our hospital for chest pain, bone and joint pain, hair loss and asthenia for the last 3 months. On physical examination, we found fever at 38°C, polypnea at 32 cycles/min, arterial hypertension at 160/90 mmHg, malar rash, synovitis of wrists, elbows and ankles, and edema on both legs. Diagnostic assessment: laboratory tests disclosed the following values: Increase level of erythrocyte sedimentation rate 120 mm/1 st hour, C reactive protein was 20 mg/l. The blood count showed hemoglobin at 9g/dl, MCV at 85 µ 3, lymphopenia at 400/mm 3, while platelets were normal 450 000/mm 3. Direct coombs test was negative. Serum Albumin was 25g/l; proteins level was low at 50g/l and proteinuria at 3.2g/day. Creatinine was normal. Anti-nuclear antibody ANA were positive 1/320 with homogeneous pattern, anti-dsDNA level was 160U/ml. Anti-cardiolipin IgG antibodies were positive 25UGPL. Rheumatoid Factor was negative. C3 and C4 complement fractions were low (0.2 g/l and 0.05 g/l). Chest x-ray, electrocardiogram and echocardiography were normal. Chest computed tomography (CT) scan showed proximal pulmonary embolism. Renal ultrasound was normal. Renal biopsy reveals immunocomplex nephritis, lupus nephritis, segmental mesangial proliferation, mild activity lupus nephritis class III (A/C). Before starting corticosteroids, we analyzed electrolytes. Serum calcium was elevated 132 mg/l with hyper-calciuria 479 mg/24 h. 25 OHD was normal. Alcaline phosphatase was 420 UI/l. the patient did not have any symptoms of hypercalcemia. Serum protein Electrophoresis showed polyclonal hypergammaglobulinaemia. Serum and urine immunofixation as well as Bence-Jones proteinuria were negative. Intact parathyroid hormone (iPTH) was high 628 pg/ml. CT scan and ultrasound of parathyroid imaging revealed a lower left parathyroid nodule measuring 2cm x 1 cm. Femoral and lumbar bone mineral density (BMD) showed osteoporosis (T-score: -2.6). In addition, we found multiple pelvic osteolytic lesions at CT scan. Other causes of hypercalcemia and bone lysis were excluded. The diagnosis in this case was SLE with lupus nephritis class III and anti-phospholipid syndrome, complicated by pulmonary embolism associated to primary hyperparathyroidism causing severe hypercalcemia, osteoporosis. Therapeutic intervention: the patient was given a pulse of methyl-prednisolone 15 mg/kg/day for 3 days followed by oral prednisone 1 mg/kg/day, associated with intravenous cyclophosphamide 750 mg/m 2 /month for 6 months. Mycophenolate mofetil 2 g/day was prescribed as a maintenance therapy of lupus nephritis with hydroxychloroquine at a dose of 400 mg/day. Treatment of pulmonary embolism was initiated with subcutaneous low molecular weight heparin (enoxaparin 0.1 ml/10 kg/12h) followed by antivitamin K. For Hypercalcemia, patient has received intravenous fluid with furosemide. She also required Alendronate 70 mg/week with Vitamin D 400u/day for osteoporosis. The left parathyroid gland was surgically removed. Histopathological examination revealed parathyroid adenoma. Her immediate postoperative parathyroid hormone level was 64 pg/ml with a calcium level of 98 mg/l. Follow-up and outcomes: forty-eight hours after the surgery she developed oral paresthesia. She had hypocalcemia at 72 mg/l. She required oral supplementation for few months. Additional investigations for multiple endocrine neoplasia were negative. The patient remained asymptomatic. Her SLE was calm without any relapse. Control of proteinuria was negative. Corticosteroids was dropped. The follow-up was 4 years.
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She denied a history of hepatitis, jaundice or alcohol use. | [
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] | A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine
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Achieving a closed reduction is a challenge with ipsilateral fractures but it should be favored over open reduction due to a lower risk of complications. | [] | This case report describes the condition of a 30-year-old man involved in a motor vehicle collision. Clinical examination, X-rays, and computed tomography scan revealed a posterior hip dislocation with an ipsilateral femoral head and mid-shaft fractures. The patient was treated by closed reduction of hip dislocation using a temporarily applied external fixator followed by intramedullary nailing of the femoral shaft. Achieving a closed reduction is a challenge with ipsilateral fractures but it should be favored over open reduction due to a lower risk of complications. The type of femoral head fracture, in this case, may have aided in an easier reduction.
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Along with that, a localized oedema appeared in her left upper limb. | [] | A 23 year old female patient (weight 67kg, height 171cm, BMI 22.91kg/cm 2) presented to emergency department with fever (39.4oC), tachycardia (116 bpm), hypotension (95/45 mmHg), tachypnoea (25 breaths/min), sore throat, neck pain, diffuse pain and tissue oedema in the right-side gluteal region. She was appointed to hospital by a private outpatient surgical center where she had undergone perineal abscess drainage a week before. Recent medical history included also endometrial curettage for ERPC a month before and conservative therapy for hemorrhoids 15 days after. Past medical history included Henoch-Schönlein purpura during childhood. Social history included smoking (11 pack-years), family history was non-contributory. She had no known drug allergies. Complete blood count revealed marked neutrophilic leukocytosis (WBC 23.400 /lt). A CT scan revealed small bilateral pleural effusions, small hepatic enlargement, Gerota's fascia thickening, perineal soft tissue stranding with fascial thickening and small bubbles of air. Fournier's gangrene was set as working diagnosis (calculated FGSI 8, UFGSI 10 and LRINEC 7) and the patient was transported to operation room for immediate radical surgical debridement of necrotic tissue. Blood and surgical material was taken for microbiologic analysis. Due to the extent of the surgical debridement, the patient was admitted postoperatively to the ICU (SOFA score 6, APACHE II score 12); where sedation (midazolam 30 mcg/kg/hr c.iv), analgesia (fentanyl 3 mcg/kg/min c.iv), triple antibiotic regimen with meropenem 2gr q8h i.v., clindamycin 600mg q 6h i.v. and daptomycin 500mg q.d. i.v., gastroprotective and antithrombotic prophylaxis were initiated. During the next 3 days a second CT and an obstetrical consultation were conducted with no significant findings. Human immunodeficiency virus and diabetic screen were also negative. Yet, the patient remained febrile. Microbiology cultures from material taken from the surgical trauma area revealed Candida albicans, Staphylococcus epidermidis and Klebsiella pneumonia ; hence antibiotic regimen was modified and antimycotic treatment (caspofungin 100mg q.d. i.v.) was initiated too. Debridements were repeated every 24 hours but due to the extent of the lesions, on the 10 th day, the patient was transported to operation room for temporary ileostomy till the end of the therapy. Percutaneous tracheotomy was also performed. Hyperbaric oxygen therapy was not available at the setting. One day after the second surgery, patient's condition complicated with MRSA ventilation associated pneumonia. Along with that, a localized oedema appeared in her left upper limb. A palpable mass along the anterior margin of the sternocleidomastoid muscle was also noticed. Vascular ultrasound examination revealed occlusion of left internal jugular vein. Transcranial Doppler examination was normal. Dental consultation and examination of soft palate and peritonsilar tissues showed no abnormalities. Linezolide 600 mg q12h i.v. was added to antibiotic regimen. Since debridements were going on, simultaneous anticoagulation therapy was started with only bemiparine 2500 b.i.d. s.c. Gradually, the patient became afebrile and her clinical condition improved. She was discharged from ICU 13 days later. A follow-up CTA confirmed the disappearance of the left jugular vein thrombosis. About a month after her ICU discharge, an operation was performed for ileostomy closure and reconstruction of the right gluteal region. On a follow-up examination 6 months later, the young woman had returned to her normal everyday life.
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His medical history was featureless. | [] | Mrs B.S. 28 years old multiparous that we had received 02 hours after a vaginal delivery of a newborn alive weighing 2750 grams in a peripheral maternity 3 km away. It was referred for postpartum hemorrhage. His medical history was featureless.
On admission, she presented hypovolemic shock with blood pressure 76/44 mmHg, regular tachycardia at 136 beats per minute, polypnea to 39 cycles per minute. The obstetrical examination objectified an involuted uterus, an absence of vaginal and perineal cervical lesions, a fluid and red blood originating of endouterine. Furthermore, there was purpura. We therefore concluded a significant postpartum hemorrhage complicated by coagulopathy. Resuscitation measures were taken and an transfusion 1120 ml whole blood, red cell pellets 350 ml and 860 ml fresh frozen plasma have helped to management coagulopathy and anemia. Blood count at admission highlighted severe thrombocytopenia 23 0000 platelets and hemoglobin levels of 3.9 g/dl. The post natal care was also administered.
After seven days of hospitalization, she was going after an amendment to the clinical symptoms and normal laboratory tests. Twenty five days later, she returned to obstetric emergencies for significant bleeding with severe anemia with hemoglobin 9 g/dL without signs of hypovolemic shock. The gynecological examination was normal. The balance sheet of the coagulation was normal including prothrombin to 92% and a normal activated partial thromboplastin time. The treatments were based on oxytocin, tranexamic acid, etamsylate and strict regular monitoring. Transvaginal ultrasound coupled Doppler was normal. In the absence of arterial embolization, she was going with ambulatory monitoring. Two weeks later she presented again rebleeding requiring hospitalization resulting in a total hysterectomy after conditioning of the patient. Histological examination of the surgical specimen objectified in the cervix and uterine body the presence of arterial vessels proliferation homes large and small caliber associated with tissue hemorrhage. The postoperative course was simple.
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] | A 3 years old girl born from non-consanguineous parents, without any neonatal suffering; with good psychomotor development, from a popular district in one of our cities, without any obvious contact with dogs; presented with exophthalmia associated with unilateral blindness evolving rapidly within 3 months. Clinical examination showed a non-pulsatile, painless, axial, irreducible exophthalmia with no sign of conjunctivitis or keratitis, and right monocular blindness, right ptosis; and fundal examination had objectified right papillary oedema. The rest of the clinical examination was normal. Brain and orbital magnetic resonance imaging (MRI) revealed an extra-connal right orbital lesion near the orbital apex, measuring 28 x 18mm, of oval shape, it appeared hypo-signal in T1 and hyper-signal in T2, limited by a thin wall which took contrast product, this lesion compressed the optic nerve towards the nasal region. Chest x-ray and abdominal ultrasound did not reveal any other localization. The patient was operated by performing a right extra-dural frontal approach, a cyst puncture was done in the first intention because the cyst was adherent to the neighbouring structures making its complete removal impossible, then a microscopic extirpation of the cystic membrane, combined with abundant washing by hypertonic serum to sterilize the cystic sit and reduce chances of dissemination. Histological examination was in favour of intra-orbital hydatidosis. Postoperatively, the patient was given an antihelminthic treatment of albendazole at a dose of 10mg per kilogram per day in 3 doses separated by 2 weeks; the evolution was marked by the significant regression of the exophthalmia and the gradual improvement of the visual acuity. The patient however still retains a right unilateral nasal hemianopia after two years of follow-up. An MRI done in the same period shows a cure of the patient by a complete disappearance of the cyst.
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The posterior cervico-vaginal fornix was neither tender nor bulging. | [] | It is the case of a 29 years old woman, G2P1001, married, at 9 weeks of pregnancy. She consulted at our emergency service for the exacerbation of a left pelvic pain that has been evolving for two weeks prior to admission. A transient lull was observed following a treatment with a progesteron (hydroxyprogesterone caproate) and an antispasmodic (tiemonium methylsulfate) in a community clinic. The resurgence of that lancinating and permanent pain irradiating to the loins and associated with slight vaginal bleeding prompted consultation. She had first menses at 14 and her menstrual cycle is regular with a length of 30 days. She has never practiced contraception. She has been adequately treated for acute pelvic inflammatory disease five years earlier. Her only child is a girl born vaginally seven years ago. Her blood group is B rhesus positive and she has never undergone surgery. She presented with sympathetic signs of pregnancy and the urinary pregnancy test was positive but echography was not yet done. On admission, besides the main complain, the patient had vaginal bleeding and nausea but neither fever, nor vomiting. On physical examination her general condition was good and the blood pressure was 110/70 millimeters of mercury, the respiratory rate: 20 cycles / minute; the pulse rate: 70 pulsations/minute and the temperature: 37.3 degree Celsius. The conjunctivae were pink and the tongue was clean and moist. There were no cervical adenopathies. The breast and cardiopulmonary examination revealed no abnormalities. The abdomen was flat, and mobile with respiration. On palpation there was just a tenderness of the left iliac fossa and the bowel sounds were normal on auscultation. Inspection under speculum revealed normal gravid external cervical os and there was no vaginal discharge. On digital exploration, the cervix was posterior long and closed, the uterus was globular, increased in size and compatible with an eight weeks pregnancy. The left adnexae presented with a tender, smooth and mobile mass of seven centimeters diameter but the right one were normal. The posterior cervico-vaginal fornix was neither tender nor bulging. We suspected a non ruptured extra-uterine pregnancy with the following differentials: heterotopic pregnancy, torsion of ovarian cyst in pregnancy, and intra-cystic ovarian bleeding in pregnancy. Paraclinical investigations revealed: beta human Chorionic Gonadotropin (β hCG) level of 96 702 milli International Units per milliliter of plasma (mIU/ml). Ultrasonography revealed a heterogenous left ovarian mass of 82 millimeters in diameter, an empty uterus and no peritoneal effusion. This comforted our first diagnosis. After a normal pre-operative work up, an emergency laparotomy was done under general anesthesia. The findings were: left ovary containing a gestational sac and the corpus luteum, normal right adnexae, normal left tube and normal uterus. There was no hemoperitoneum. We dissected the ovarian capsule and carried out ablation of the gestational sac, then we did hemostasis. The specimen was analysed by the pathologist who found decidual cells and trophoblastic tissue within the ovarian capsule and thus confirmed the ovarian pregnancy. Post operative course was uneventful and the patient was discharged six days after surgery. The ?hCG level decreased and disappeared 25 days after surgery.
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Because of hepato-splenomegaly, pancytopenia, hyperferritinemia and hypofibrinogenemia the diagnosis of Macrophage activation syndrome was evoked indicating a bone marrow aspiration that showed numerous morphologically benign macrophages with haemophagocytic activity that comforting the diagnosis of a SAM according to Ravelli and HLH-2004 criteria. | [
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] | We present here a case of Macrophage activation syndrome associated with Griscelli syndrome type 2 in a 3-years-old male born of consanguineous parentage. His birth histories, his familie social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications, He was on exclusively breast-fed, food diversification was started at 6 months old, his weight, length and psychomotor development were within the normal range, the child was described as a good eater, was on a normal diet and was thriving appropriately. Furthermore the boy presented a progressive abdominal distension since birth, progressive pallor and recurrent episodes of fever since 1 year of age. There was history of blood transfusions for last 2 months. On the other side there was no history of jaundice, vomiting, urinary or bowel complaints, bleeding from any site or neurological complaints. On admission, he was very pale with silvery gray scalp hair, white eyelashes, he was hypotonic, tachycardic, fever of 40°C, lymphoadenopathy and hepato-splenomegaly. The blood count showed pancytopenia; 1980 white blood cells/µL (VN 4000-13 500/µL), neutrophils 820/µL, 910 cells/µL, hemoglobin (Hb) 6.3 g/dL (11.5-14 VN, 5 g/dL), platelets 44 000/µL (VN 150 000-400 000/µL), There were no giant cytoplasmic granules in leucocytes. The liver function tests were normal expect low albumin (2.1 mg/dL) and increased alkaline phosphatase (1196 mg/dL) with low fibrinogen (1.7g/l). Serum triglycerides, ferrintin and lactic dehydrogenase were very high; respectively 4.75g/L; 2763µg/L and 597U/L. the C reactive protein (CRP) was elevated at 83mg/l. A chest X-ray was normal while the abdominal ultrasound showed a hepatomegaly and a splenomegaly, free biliary ducts without ascites. Because of hepato-splenomegaly, pancytopenia, hyperferritinemia and hypofibrinogenemia the diagnosis of Macrophage activation syndrome was evoked indicating a bone marrow aspiration that showed numerous morphologically benign macrophages with haemophagocytic activity that comforting the diagnosis of a SAM according to Ravelli and HLH-2004 criteria. Blood culture, urine examination, malarial serology, Kala Azar serology, viral serologies such as EBV, hepatitis A, B, C and human immunodeficiency virus HIV, serologies of rickettsia, syphilis and toxoplasmosa were negatives. Serologies of CMV and rubella showed an old immunization. The immunological tests such as antinuclear antibodies (ANA), anti-LKM1, anti-mitochondria and anti-smooth muscle antibodies were negatives. Because of the consanguineous family, notion of recurrent infection and the presence of silvery-gray color of the hair and eyebrows, diagnosis of Griscelli Syndrome was evoked directing a light microscopy examination of the hair that showed a large, irregular clumps of pigments characteristic of Griscelli Syndrome. The molecular biology showed mutation in RAB27A gene confirming the diagnosis of a Griscelli syndrome type 2. The first-line therapy in management of Macrophage activation syndrome complicating Griscelli syndrome type 2 was based on the parenteral administration of high doses of corticosteroids (methylprednisolone pulses 1g/0.73m 2 /day for 3 days then 60mg/m 2) with poor response; persisting with involvement of the 3 cell lines in the blood count, elevated ferritin and triglycerides. Given the lack of response to steroids, Etoposide was started for 3 days associated with cyclophosphamide, the evolution was good with improvement of count blood cells, ferritin was lessening while the bone marrow showed decreased numerous of macrophages with low haemophagocytic activity. On the other side the infectious process was good managed by perfusion of Antibiotics such as Ceftazidim in combination with aminoside. The patient was sent home with prednisone and cyclosporine, He was placed on the list of bone marrow transplantation (BMT).
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Physical examination revealed a left maxillary swelling. There was no facial palsy. No cervical lymph node enlargement was seen. The oral examination showed a 4 cm x 5 cm, tender, compressible mass in the left maxilla, from the lateral incisor to the second premolar teeth. The tumor surface was smooth and red-purple. Several teeth were involved and displaced in the tumor mass. We noted the absence of canine. Vitality tests proved negative on the central and lateral incisor. Hight mobility of lateral incisor was noted. Orthopantomography and occlusal radiography showed a wide osteolytic area of the anterior left maxilla, extending from the first permanent molar to the central incisor. The canine was impacted. No root resorption was observed. The radiolucency was closely related to maxillary sinus and involving the nasal cavity. Haematological investigations showed normal serum calcium, phosphorus and parathormone (PTH) levels. The provisional diagnosis of benign tumor of the maxilla was made.
The patient underwent excision and curettage of the mass with extraction of the canine and lateral incisor tooth. The wound was closed with interrupted sutures. The post-operative histopathological report revealed multinuclear giant cells scattered randomly throughout the cellular and fibrous vascular-rich tissue. New bone formation and granulation tissue rich in mononuclear inflammatory cells was revealed. The giant cells were multinucleated with bland-appearing nuclei, and the background stromal cells displayed no evidence of atypical mitoses. A diagnosis of giant cell tumor was established.
During a 1-year serial clinical and radiological follow-up, there was no evidence of recurrence. The facial contour and masticatory function were well-preserved.
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One month later, there was no detectable ascitic fluid and no pleural effusions. | [] | A 77-year-old Tunisian woman was hospitalized because of massive painful ascites. Her family history did not include any autoimmune disease. She denied a history of hepatitis, jaundice or alcohol use. She had a history of diabetes, hypertension treated by glinide and calcium blocker. She was explored 4 years prior to admission for exudative pleuritis of the right lung without any established diagnosis after multiple explorations including thoracoscopy with biopsies. On admission, blood pressure was 150/70 mmHg, her rate was 80 /mn and body temperature was 37°C. The physical examination showed only abdominal distension related to the important ascites without collateral venous circulation. She had no skin lesions, lymphadenopathy, or hepatosplenomegaly and lower extremities showed no edema. Laboratory investigations showed: leucopenia with white blood cell count = 3100/mm3, lymphopenia = 840/mm3, hemoglobin = 10.5g/dL ; low serum albumin level = 29g/L, 47;-globulin = 19g/L. blood glucose levels = 180 mg/dl and hemoglobin A1c = 8.6 %. Urine analysis showed trace protein (0.03g/24h). Platelet count, activated partial thromboplastin time, liver function tests, blood urea nitrogen, serum creatinine, erythrocyte sediment rate and total cholesterol were within normal limits. Tests for HBsAg and HCV were negative. Abdominal ultrasonography showed ascitic fluid without any sign of bowel loop thickening or enteritis. Neither lymphadenopathy nor liver abnormality was present. A chest X-ray film disclosed bilateral pleural effusion. Ascitic obtained by aspiration contained 170 cells mm3 (67% lymphocytes), 46g/L protein, but no malignant cells. Cultures for bacteria and mycobacteria gave no growth. Tuberculosis investigation including initial tuberculin skin test and the research of Koch bacilli in sputum and urine were negative. Echocardiogram and electrocardiogram were normal. Computed tomographic scan showed massive ascites, no dysmorphic liver, and a mild bilateral pleural effusion. Pelvic examination was normal. Oesogastroduodenoscopy showed hiatal hernia without oesophageal varices. An exploratory laparoscopy was performed, revealing a large amount of ascite, a normal liver appearance, and no granulations suggesting tuberculosis or peritoneal carcinosis. Histological examination of the peritoneum showed non specific chronic inflammation. Systemic lupus erythematosus was suspected based on pleuritis, lymphopenia and leucopenia. An additional serologic survey revealed markedly elevated anti-nuclear antibody (ANA) titer of 1/1600 and a significantly elevated titer of antibody to double-stranded DNA (83 IU/mL ; normal < 30 IU/mL). She had also positive serum antibody against the Smith antigen and low serum level of C3 complement component: 67 mg/dL (serum normal range: 84 – 151). Relying on these findings, the patient was diagnosed with SLE since 4 of the 11 diagnostic criteria of the American College of Rheumatology were met. The SLEDAI score according to Systemic Lupus Erythematosus Disease Activity Index was estimated to be 7 on admission. Hydroxychloroquine 200 mg daily in combination with diuretics was initiated. One month later, there was no detectable ascitic fluid and no pleural effusions. Five months later she remained free from symptoms while continuing to take Hydroxychloroquine
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He no family history of heart disease or sudden death. | [
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After exposure, Proximal Tibial, Distal Femoral Cuts and measurement of extension gaps the synovium from the anterior Femur was removed and sizing was done. | [] | A 70yrs old Female was admitted in our institution diagnosed with severe bilateral Osteoarthritis. The x-rays showed bone on bone Tricompartment OA Knee with Varus Malalignment. She was posted for Single Stage Bilateral Total Knee Replacement and as planned the Left Knee Was Operated first. After exposure, Proximal Tibial, Distal Femoral Cuts and measurement of extension gaps the synovium from the anterior Femur was removed and sizing was done. The AP cut was then proceeded with. We spotted a small Osteochondral Cyst in the Anterior Femur which was curretted to remove the cystic material, which is when we realised that the cyst was large and communicating with the medulary canal. The remaining Femoral preparations was done keeping in mind the risk of iatrogenic fracture and extension Stem was used in the femur. The defect was then packed cancellous bone graft.
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Postoperatively, the patient was given an antihelminthic treatment of albendazole at a dose of 10mg per kilogram per day in 3 doses separated by 2 weeks; the evolution was marked by the significant regression of the exophthalmia and the gradual improvement of the visual acuity. | [
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] | A 3 years old girl born from non-consanguineous parents, without any neonatal suffering; with good psychomotor development, from a popular district in one of our cities, without any obvious contact with dogs; presented with exophthalmia associated with unilateral blindness evolving rapidly within 3 months. Clinical examination showed a non-pulsatile, painless, axial, irreducible exophthalmia with no sign of conjunctivitis or keratitis, and right monocular blindness, right ptosis; and fundal examination had objectified right papillary oedema. The rest of the clinical examination was normal. Brain and orbital magnetic resonance imaging (MRI) revealed an extra-connal right orbital lesion near the orbital apex, measuring 28 x 18mm, of oval shape, it appeared hypo-signal in T1 and hyper-signal in T2, limited by a thin wall which took contrast product, this lesion compressed the optic nerve towards the nasal region. Chest x-ray and abdominal ultrasound did not reveal any other localization. The patient was operated by performing a right extra-dural frontal approach, a cyst puncture was done in the first intention because the cyst was adherent to the neighbouring structures making its complete removal impossible, then a microscopic extirpation of the cystic membrane, combined with abundant washing by hypertonic serum to sterilize the cystic sit and reduce chances of dissemination. Histological examination was in favour of intra-orbital hydatidosis. Postoperatively, the patient was given an antihelminthic treatment of albendazole at a dose of 10mg per kilogram per day in 3 doses separated by 2 weeks; the evolution was marked by the significant regression of the exophthalmia and the gradual improvement of the visual acuity. The patient however still retains a right unilateral nasal hemianopia after two years of follow-up. An MRI done in the same period shows a cure of the patient by a complete disappearance of the cyst.
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Within 1 month, the boy's gastrointestinal symptoms were relieved and his behavior had changed. | [] | We received a boy 3-year-old boy with autistic disorder on hospital of pediatric ward A at university hospital Mohammed VI of Marrakesh. He has no family history of illness or autistic spectrum disorder. The history revealed that at 2 years of age. The child was diagnosed with a ‘‘severe communication disorder,'' with social interaction difficulties and sensory processing delay. A composite follow-up of all previous assessments and investigations was undertaken. Blood work was normal (thyroid-stimulating hormone (TSH), hemoglobin, mean corpuscular volume (MCV), and ferritin). Genetic testing was unremarkable (normal karyotype, negative for fragile X) and the magnetic resonance imaging (MRI) in search of a demyelinating attack of the white matter, electroencephalography (EEG), optometry assessment were also normal. At the conclusion of this composite assessment, the boy was given a primary diagnosis of autistic spectrum disorder. A plan was instituted including speech-language therapy, intensive individualized educational programming, and contact was encouraged with the Autism Society. For that purpose, the parents moved to morocco to be surrounded by family without any huge improvement. Upon direct questioning at initial presentation, the 3-year-old child was reported by the parents to have unexplained fatigue, Gastrointestinal symptoms included bloating, constipation and diarrhea. Psychiatric symptoms included a frequently depressed mood, disproportionate anger, and emotional lability. On exploring his history, the child was born from yemenite father and Moroccan mother in Germany at-term weighing 3500 g with an Apgar score of 9 at 1 minute and 10 at 5 minutes after an uneventful pregnancy and delivery. No concerns were present in the neonatal period. Development in the first 24 months of life appeared fine according to the parents-his motor skills seemed to progress normally and he achieved expected milestones. After 2 years of age, however, his language skills slowly began to regress. He also demonstrated a change in temperament as he started to whine repeatedly and to scream without provocation. Physical examination revealed a height and weight normal for age. He was uncommunicative, restless, and somewhat agitated. General examination was unremarkable other than dark rings around the eyes. There were no dysmorphic features evident. He had difficulty maintaining eye contact, and he appeared disinterested in what was taking place. No abnormality was found on abdominal assessment. Anti-tissue transglutaminase antibodies levels were 76 U (normal < 10). The patient underwent upper endoscopy as duodenal biopsy to confirm a celiac disease diagnosis. It shows a total villous atrophy corresponding to a stage 4 of Marsh classification. Given the positive screen for celiac disease (positive anti-tissue transglutaminase antibodies and results of duodenal biopsy), dietary intervention was immediately commenced. All gluten was eliminated from the boy's diet. Within 1 month, the boy's gastrointestinal symptoms were relieved and his behavior had changed. The mother reported that her boy became progressively more communicative. The child has continued on the gluten-free diet and has progressed well and remained healthy over the following 6 months.
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The chest pain was localized to the middle of the chest and was aggravated on exertion. | [
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] | A 43-year-old non-diabetic Indian male reported to our outpatient department with chief complaints of cough with expectoration, chest pain, reduced appetite, fever with chills, and night sweats for two weeks. He also complained of breathlessness on exertion and had two episodes of blood in his sputum. The patient explained that the cough was continuous and was relieved after taking cough syrup. He also mentioned that the episodes of fever were initially intermittent and then daily for the last two weeks and were relieved after taking Paracetamol. The chest pain was localized to the middle of the chest and was aggravated on exertion. He was a businessman by profession with no history of smoking, alcoholism, or any other substance abuse. Also, there was no history of any contact of TB or COVID-19 in the family or close contacts. And there was no history of foreign travel in the recent past. But he had reported having traveled by a domestic airline about twenty days back. There was no history of weight loss or any other major illness in the past.
On examination his vitals were-pulse-108/minute, arterial BP-130/80 mm of Hg, respiratory rate of 30 breaths/minute, Sp02-899% on room air, temperature- 101-degree centigrade. His Sp02 fell by 70% on room air after waking. On auscultation, there was crepitation on the bilateral middle lobes of the lungs. Also, dyspnea on exertion was noted. The rest of the systemic examination was within normal limits.
Considering this as a probable case of TB with COVID-19 he was advised a chest radiograph with sputum microscopy (Ziehl Neelsen (ZN) staining for acid-fast bacilli), Cartridge-based nucleic acid amplification test (CBNAAT) of the sputum and other routine investigations. To check for the COVID-19 he was advised qualitative polymerase chain reaction (PCR) test from the oropharyngeal swab. The results were surprising with Mycobacterium tuberculosis detected on sputum fluorescent microscopy and were also confirmed by the CBNAAT. However, there was no resistance to Rifampicin. The results of the PCR were positive for RNA specific to SARS-CoV-2. Besides, the chest radiograph PA-view was suggestive of bilateral consolidations on the middle lobes of lungs with ill-defined borders. The other investigations revealed a low lymphocyte count (1x 10 9 /L) and increased levels of C-reactive protein (CRP) (57 mg/L), lactate dehydrogenase (LDH) (580 U/L), and erythrocyte sedimentation rate (ESR) (70 mm in the 1 st hour). Also, a sample for liquid culture (MGIT BACTEC) was sent to the Intermediate Reference Laboratory (IRL) which revealed the growth of Mycobacterium tuberculosis. Computed tomography was not performed as the diagnosis was established by other cheaper and faster methods and also the patient was unwilling for the same. All the other routine investigations were within normal limits.
He was referred to the nearest designated COVID-19 management center, where he was managed as per national guidelines. Besides, he was also started on an antitubercular treatment of four drugs as per the National Tuberculosis Elimination Program (NTEP) guidelines. He was advised follow-up post completion of his stay at the designated COVID-19 center, but he has not yet reported back for follow-up. Written informed consent was obtained from the patient for using clinical data and images for publication in this study.
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