OpenMed/multicare-articles huggingface dataset:
Systemic capillary leak syndrome: is methylene blue the silver bullet?
Authors: Michele Umbrello, Marco Gardinali, Davide Ottolina, Giancarlo Zanforlin, Gaetano Iapichino
Journal: Case Rep Crit Care (2014)

Background. Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by unexplained, recurrent episodes of transient, abrupt increase in endothelial permeability, leading to severe hypotension, generalized edema, and hemoconcentration. Case Report. We report the case of a patient suffering from systemic capillary leak syndrome and present a possible interpretation of the pathophysiology of this condition. Besides the classical triad of hypotension, edema, and hemoconcentration, we recorded increased levels of methemoglobin, an index of NO overproduction. We present a possible interpretation of the pathophysiology of this condition based on the fast and complete reversal of symptoms after methylene blue administration (which opposes NO-induced effects) and speculate that increased NO levels could be implicated in the pathophysiology of the capillary leak phase. Why should an emergency physician be aware of this? The safety of this treatment and its fluid- and cathecolamine-sparing effect deserve consideration and further research.
Hypophosphatemic Osteomalacia Associated with Tenofovir: a Multidisciplinary Approach is Required
Authors: Giuseppe Vittorio L De Socio, Gianluigi Fabbriciani, Marco Massarotti, Salvatore Messina, Enisia Cecchini, Bianca Marasini
Journal: Mediterr J Hematol Infect Dis (2012)
MeSH terms: Case Reports

Tenofovir is widely used as first-line treatment of HIV infection, although its use is sometimes complicated by a reversible proximal renal tubulopathy.We report the case of a 45-year-old woman with chronic HIV infection and personality disorder, who after 12 months of tenofovir, complained of fatigue, diffuse bone pain and gait disturbances. The elevated level of alkaline phosphatase, hypophosphatemia and inappropriate phosphaturia suggested the diagnosis of hypophosphatemic osteomalacia secondary to proximal renal tubulopathy. A dual-energy x-ray absorptiometry showed a bone mineral density below the expected range for age (lumbar spine Z-score -3.3, femoral neck Z-score -2.1). A whole body (99m)Tc-methylene diphosphonate bone scan showed multiple areas of increased focal activity in the lumbar and thoracic spine and in sacroiliac and hip joints consistent with pseudofractures. Two months after tenofovir discontinuation and administration of vitamin D and phosphate, osteomalacia-related symptoms disappeared. Eleven months later, bone and mineral metabolism data were normal and bone scintigraphy did not show any pathological findings.This report highlights the importance of considering the diagnosis of osteomalacia in patients treated with tenofovir and emphasizes the need for monitoring alkaline phosphatase, blood and urinary phosphate and creatinine, especially in patients with risk factors for bone disease.
Disseminated Candida lusitaniae: Nosocomial Acquisition Secondary to an Indwelling Urinary Catheter
Authors: Ali Raja, Julia Park
Journal: Case Rep Infect Dis (2021)
MeSH terms: Case Reports

Candida lusitaniae is a rare opportunistic pathogen, and its most common risk factors include immunocompromised patients often with an underlying malignancy. It commonly displays resistance to amphotericin B, and historically, echinocandins have been considered first-line treatment. We present a 77-year-old male with a history of diabetes mellitus. He was treated for cellulitis and discharged to a skilled nursing facility with an indwelling urinary catheter. Despite recommendations from the medicine team to remove the catheter, the patient refused even after discussing the risks and benefits. He returned to the hospital 3 weeks later with symptoms of dysarthria, right-sided facial droop, and right-sided weakness. Ultimately, he was determined to have fungemia and native valve endocarditis due to Candida lusitaniae stemming from his indwelling urinary catheter. He was treated with micafungin, but repeated blood cultures continued to grow C. lusitaniae, and he eventually expired following withdrawal of care. We present this case report to illustrate a rare occurence of Candida lusitaniae in a patient without typical risk factors. C. lusitaniae fungemia is an extremely uncommon disease in patients without underlying malignancy. Despite this lack of apparent, classic risk factors, our patient developed endocarditis of his native valve due to C. lusitaniae fungemia from an indwelling urinary catheter. The ability of this organism to form biofilms, and its rapid mutation rate, makes treating C. lusitaniae very difficult. The treatment of choice for C. lusitaniae endocarditis is surgical intervention due to biofilm formation on the cardiac valves. Medical treatment recommendations are currently fluconazole, which is in contrast to the historical use of echinocandins. Infection due to Candida lusitaniae, though rare, should be remembered by clinicians. This particular fungal agent is especially difficult to treat due to its multiple virulence factors. Additionally, the use of indwelling urinary catheters should only occur when proper indications are present and should be promptly discontinued when their placement is no longer necessary.
Complement Factor H Gene Variant in a Patient with Thrombotic Microangiopathy on a Mixed Clinical Background
Authors: Yoichi Iwafuchi, Tetsuo Morioka, Yuko Oyama, Shin Goto, Ichiei Narita
Journal: Case Rep Nephrol (2021)
MeSH terms: Case Reports

We report the case of a patient with complement factor H gene variant, who developed thrombotic microangiopathy on a mixed clinical background. A 79-year-old woman was transferred to Sanjo General Hospital for maintenance hemodialysis. She suffered from gastric non-Hodgkin lymphoma about two years ago and received chemotherapy and radiation therapy, leading to complete remission. About 13 weeks prior to her transfer to our hospital, she was referred to another hospital due to acute kidney injury, hemolytic anemia, and thrombocytopenia. Hemodialysis was immediately initiated, after which intravenous methylprednisolone and oral prednisolone were started; however, she became anuric within approximately week. The possibility of thrombotic microangiopathy was examined. However, she was in poor general condition and did not get the consent of her family, so no invasive searches such as a kidney biopsy were performed. Despite the cause of acute kidney insufficiency being unclear, she was transferred to us for maintenance hemodialysis. Her general condition was stable, and her renal function improved; hence, two months after transfer, a kidney biopsy was performed. Her clinical and typical renal histological findings indicated a diagnosis of thrombotic microangiopathy. There was a possible CFH gene of a very rare variant "c.526 T > C (p.Phe176Leu)" in exon 5. She was able to withdraw from hemodialysis therapy two weeks after the initiation of an angiotensin-converting enzyme inhibitor. Based on her clinical course and kidney biopsy findings, she was diagnosed with thrombotic microangiopathy with a very rare CFH variant. To ensure proper treatment choices such as eculizumab, the presence of complement dysregulation should be considered in cases of secondary thrombotic microangiopathy.
First human case report of Crohn's disease with coexistent acute appendicitis treated by endoscopic retrograde appendicitis therapy
Authors: Guang-Xing Cui, Muhammad Zulqarnain, Qi-Feng Lou, Hong-Zhang Shen, Wen Lyu, Xia Wang, Haitao Huang, Hamse Mohamoud Abdi, Lingfei Gu, Shijie Fang, Fangzhou Liu, Liqian Ling, Yu Huang, Linglin Chu, Saboor Saeed
Journal: Front Med (Lausanne) (2023)
Keywords: crohn's disease, acute appendicitis, coexistent, endoscopic retrograde appendicitis therapy, endoscopic retrograde appendicography
MeSH terms: Case Reports

Background:
The coexistence of Crohn's disease (CD) and acute appendicitis (AA) is rare. In this situation, therapeutic experience is lacking and the strategy is paradoxical and intractable. Appendectomy is the gold standard for the treatment of AA whereas a nonsurgical approach is recommended for CD.
Case summary:
A 17-year-old boy was hospitalized for right lower abdominal pain with fever of 3 days. He had the CD for 8 years. Two years ago, he underwent surgery for anal fistula with the complication of CD. His temperature was elevated at 38.3°C at admission. On physical examination, there was McBurney tenderness with mild rebound tenderness. Abdominal ultrasonography showed that the appendix was notably enlarged and dilated at 6.34 cm long and 2.76 cm wide. These findings were suggestive of uncomplicated AA in this patient with active CD. Endoscopic retrograde appendicitis therapy (ERAT) was performed. The patient had complete pain relief immediately after the procedure without tenderness in the right lower abdomen. During 18 mo follow-up, he had no more attacks in his right lower abdomen.
Conclusion:
ERAT was effective and safe in a CD patient with coexisting AA. Such cases can avoid surgery and its-related complications.
Case Report: Hyperammonemic Encephalopathy Linked to Ureaplasma spp. and/or Mycoplasma hominis Systemic Infection in Patients Treated for Leukemia, an Emergency Not to Be Missed
Authors: Manon Delafoy, Juliette Goutines, Aude-Marie Fourmont, André Birgy, Maryline Chomton, Michaël Levy, Jérôme Naudin, Lara Zafrani, Lou Le Mouel, Karima Yakouben, Aurélie Cointe, Marion Caseris, Matthieu Lafaurie, Stéphane Bonacorsi, Françoise Mechinaud, Sabine Pereyre, Nicolas Boissel, André Baruchel
Journal: Front Oncol (2022)
Keywords: mycoplasma spp, ureaplasma spp, case report, hyperammonemic encephalopathy, immunocompromised patients, systemic infection
MeSH terms: Case Reports

Background:
Hyperammonemic encephalopathy caused by Ureaplasma spp. and Mycoplasma hominis infection has been reported in immunocompromised patients undergoing lung transplant, but data are scarce in patients with hematological malignancies.
Case presentation:
We describe the cases of 3 female patients aged 11-16 years old, developing initially mild neurologic symptoms, rapidly evolving to coma and associated with very high ammonia levels, while undergoing intensive treatment for acute leukemia (chemotherapy: 2 and hematopoietic stem cell transplant: 1). Brain imaging displayed cerebral edema and/or microbleeding. Electroencephalograms showed diffuse slowing patterns. One patient had moderate renal failure. Extensive liver and metabolic functions were all normal. Ureaplasma spp. and M. hominis were detected by PCR and specific culture in two patients, resulting in prompt initiation of combined antibiotics therapy by fluoroquinolones and macrolides. For these 2 patients, the improvement of the neurological status and ammonia levels were observed within 96 h, without any long-term sequelae. M. hominis was detected post-mortem in vagina, using 16S rRNA PCR for the third patient who died of cerebral edema.
Conclusion:
Hyperammonemic encephalopathy linked to Ureaplasma spp. and M. hominis is a rare complication encountered in immunocompromised patients treated for acute leukemia, which can lead to death if unrecognized. Combining our experience with the few published cases (n=4), we observed a strong trend among female patients and very high levels of ammonia, consistently uncontrolled by classical measures (ammonia-scavenging agents and/or continuous kidney replacement therapy). The reversibility of the encephalopathy without sequelae is possible with prompt diagnosis and adequate combined specific antibiotherapy. Any neurological symptoms in an immunocompromised host should lead to the measurement of ammonia levels. If increased, and in the absence of an obvious cause, it should prompt to perform a search for Ureaplasma spp. and M. hominis by PCR as well as an immediate empirical initiation of combined specific antibiotherapy.
First Vacuum-Assisted Excision of a Breast Myofibroblastoma
Authors: B Fakim, A Abbas, M Crotch-Harvey, J Kokan
Journal: Case Rep Surg (2019)
MeSH terms: Case Reports

A 52-year-old lady was seen in the breast clinic after an 8 mm lesion was found in her left breast on screening mammogram. Clinical examination was normal. The left breast mammogram showed an 8 mm rounded density posteriorly in the inner half of breast and ultrasound also showed a 7 mm, well-defined ovoid echogenic lesion (R3, U3). Biopsy confirmed the lesion was fibro-fatty tissue containing a diffuse infiltrate of lymphoid cells macroscopically (B3/4)-findings in line with a diagnosis of a myofibroblastoma. A myofibroblastoma is a rare benign mesenchymal tumour comprising of spindle cells. Most breast myofibroblastomas described in the literature have been excised by wide local excision. In this patient's case, a vacuum-assisted technique was discussed and suggested at the multidisciplinary meeting. It was excised using ultrasound-guided Vacora® breast biopsy system. The lesion was completely excised with the encore biopsy measuring 24 × 17 mm. This is the first documented case of such a technique for the excision of a breast myofibroblastoma. There still is uncertainty about breast myofibroblastomas, their aetiology, associations, and how they are best investigated. Regarding management, vacuum-assisted technique, being less invasive and cheaper than local excisions, is adequate for small lesions.
Intradiaphragmatic pulmonary sequestrations: a surgical challenge. Case series
Authors: Chiara Oreglio, Francesca Tocchioni, Marco Ghionzoli, Annamaria Buccoliero, Antonino Morabito, Francesco Morini
Journal: Front Surg (2023)
Keywords: cpam, bronchopulmonary sequestration, children, congenital lung malformation, laparoscopy, thoracoscopy
MeSH terms: Case Reports

Bronchopulmonary sequestrations (BPSs) are rare congenital anomalies characterized by non-functioning embryonic lung tissue receiving anomalous blood supply. They are most commonly located within the thorax (supradiaphragmatic) or into the abdominal cavity (infradiaphragmatic). Intradiaphragmatic extralobar BPs (IDEPS) are an exceptionally rare finding, representing a diagnostic and operative challenge. We report three cases of IDEPS and their surgical management, describing our experience and approach to such rare clinical entities. From 2016 to 2022, we treated 3 cases of IDEPS. Surgical techniques, histopathological findings and clinical outcomes were retrospectively evaluated for each case and compared. Three different surgical techniques were used to approach each lesion, from open thoracotomy to a combined laparoscopic and thoracoscopic approach. Histopathological analysis of the specimens revealed hybrid pathological features, proper of both congenital pulmonary airway malformation (CPAM) and extralobar pulmonary sequestration. IDEPS represent a surgical challenge for pediatric surgeons, given their complex surgical planning. In our experience, the thoracoscopic approach is safe and feasible when performed by trained surgeons, even though a combined thoracoscopic-laparoscopic approach allows for optimal vessels control. The presence of CPAM elements within the lesions supports their surgical removal. Additional studies are required to better characterize IDEPS and their management.
Open-coil retraction spring
Authors: Pavankumar Janardan Vibhute
Journal: Case Rep Dent (2011)
MeSH terms: Case Reports

Sliding mechanic has become a popular method for space closure with developments in preadjusted edgewise appliance. Furthermore, various space closing auxiliaries have been developed and evaluated extensively for their clinical efficiency. Their effectiveness enhanced with optimum force magnitude and low-load deflection rate (LDR)/force decay. With the advent of NiTi springs in orthodontics, LDRs have been markedly reduced. For use of NiTi, clinician has to depend upon prefabricated closed coil springs. "Open Coil Retraction Spring (OCRS)" is developed utilizing NiTi open-coil spring for orthodontic space closure. This paper describes fabrication and clinical application of OCRS which have number of advantages. It sustains low LDR with optimum force magnitude. Its design is adjustable for desired length and force level. It is fail-safe for both activation and deactivation (i.e., it cannot be over activated, and decompression limit of open coil is also controlled by the operator, resp.). A possibility to offset the OCRS away from mucosa helps to reduce its soft-tissue impingement.
Epstein-Barr Virus and Cytomegalovirus induced Acute Hepatitis in Young Female Patient
Authors: İhsan Ates, Mustafa Kaplan, Nisbet Yilmaz, Filiz Çiftçi
Journal: Euroasian J Hepatogastroenterol (2015)
Keywords: acute hepatitis, cmv, ebv
MeSH terms: Case Reports

Acute hepatitis is a disorder that goes with liver cell necrosis and liver inflammation. Among the causes of acute hepatitis, the most common reasons are viral hepatitis. About 95% of the acute hepatitis generate because of hepatotropic viruses. Epstein-barr virus (EBV) and cytomegalovirus (CMV) are from the family of herpes viruses and rare causes of acute hepatitis. In this case report, acute hepatitis due to EBV and CMV coinfection will be described.
How to cite this article:
Ates İ, Kaplan M, Yilmaz N, Çiftçi F. Epstein-Barr Virus and Cytomegalovirus induced Acute Hepatitis in Young Female Patient. Euroasian J Hepato-Gastroenterol 2015;5(1):60-61.
A Case of Myeloproliferative Neoplasm with BCR-FGFR1 Rearrangement: Favorable Outcome after Haploidentical Allogeneic Transplantation
Authors: Paola Villafuerte-Gutiérrez, Montserrat López Rubio, Pilar Herrera, Eva Arranz
Journal: Case Rep Hematol (2018)
MeSH terms: Case Reports

Hematopoietic myeloproliferative neoplasms with FGFR1 rearrangement result in the 8p11 myeloproliferative syndrome that in the current Word Health Organization classification is designated as "myeloid and lymphoid neoplasm with FGFR1 abnormalities." We report the case of a 66-year-old man who had clinical features that resembled chronic myeloid leukaemia (CML), but bone marrow cytogenetic and fluorescent in situ hybridization (FISH) studies showed t(8;22)(p11;q11) and BCR-FGFR1 fusion gene. He was initially managed with hydroxyurea, and given the aggressive nature of this disease, four months later, the patient underwent an allogeneic hematopoietic stem-cell transplantation (HSCT) from an HLA-haploidentical relative. Currently, HSCT may be the only therapeutic option for long-term survival at least until more efficacious tyrosine kinase inhibitors (TKIs) become available.
Masson's tumor involving the hand: A case report
Authors: Mohammed A Almarghoub, Qutaiba N M Shah Mardan, Ahmed S Alotaibi, Norhan K Ahmed, Moraya S Alqahtani
Journal: Int J Surg Case Rep (2020)
Keywords: case report, hand, ipeh, intravascular papillary endothelial hyperplasia, masson’s tumor
MeSH terms: Case Reports

Introduction:
Masson's tumor or IPEH represents a rare exuberant endothelial proliferation within a thrombus through an uncomprehended phenomenon. Being reported for the 1st time in Saudi Arabia, plastic surgeons should keep it in the list of differential diagnosis.
Case:
We report a case of 17-year-old-girl who presented with a 6-month-old, painful mass on the volar side of her left 4th MCP. Radiology was inconclusive. Histopathology reported Masson's tumor following surgical excision with good functional outcome and no recurrence.
Discussion:
Comprising 2%-4% of overall skin vascular tumor and with no identifying clinical or radiological feature, IPEH poses as a diagnostic challenge. The literature reports similar tumors in the hand with different locations and presentations. Surgical excision remains the cornerstone of management, yet the role of radiotherapy remains undefined. Incomplete excision may result in recurrence, which requires a consensus on the extend of marginal excision. Rare cases of recurrence were reported. Histopathology is the only reliable method of diagnosis.
Conclusion:
We present the 1st reported case of IPEH involving the hand in Saudi Arabia. Plastic surgeons should be aware of such rare conditions and be able to differentiate them from relatively identical yet more sinister tumors, especially angiosarcoma.
Case report: Magnetic resonance imaging-based three-dimensional printing for reconstruction of complex cloacal malformations
Authors: Suiin Gang, Sang Hoon Song, Jaeyoung Kwon, Hyunhee Kwon, Suhyeon Ha, Jueun Park, Namkug Kim, Hee Mang Yoon, Jung-Man Namgoong
Journal: Front Pediatr (2023)
Keywords: cloaca, contrast, magnetic resonance imaging, reconstruction, three-dimensional printing (3d printing)
MeSH terms: Case Reports

Background:
Surgical reconstruction of the urinary tract, anus, and vagina is the definitive treatment for cloacal malformation. However, this procedure may be technically challenging in patients with a long common channel (>3 cm), because further reconstructive procedures, such as vaginal replacement or vaginal switch maneuver, may be required. Thus, accurate determination of spatial anatomy is essential during surgical planning. Three-dimensional (3D) reconstruction using rotational fluoroscopy, computed tomography (CT), and magnetic resonance imaging (MRI) has recently been reported to help in determining the relationship between the rectum, vagina, and bladder, and provides a more accurate measurement of the channel length compared to conventional cloacography. MRI-based 3D reconstruction provides substantial information regarding soft tissue structures around the cloaca, including the pelvic floor musculature and anus.
Case:
A 2-year-old girl with cloacal malformation required reconstructive surgery. Colostomy and cystostomy had been performed on the first day of her life. Preoperative loopogram revealed a cloaca with a long common channel (35 mm) and short urethra (9 mm), single vaginal opening in the bladder neck, and the colon anterior to the vagina with a fistula at the vaginal neck. Because the vagina was too short to be pulled through, 3D printing based on MRI was performed to visualize structural relationships prior to surgical correction. Saline was used for cloacal visualization. Furthermore, endoscopy-assisted urogenital mobilization was performed, and vaginal substitution was performed using the rectum. No postoperative complications were observed.
Conclusions:
We believe this is the first report of the use of MRI-based 3D imaging and printing, with saline as a contrast agent during surgical planning for correction of cloacal malformation. MRI-based 3D printing is a potentially promising technique for surgical planning of cloacal malformation correction in patients with a long common channel, as it provides detailed information about the surrounding soft tissue structures without exposure to radiation or contrasting agents.
Atypical Amniotic Fluid Embolism Managed with a Novel Therapeutic Regimen
Authors: Shadi Rezai, Alexander C Hughes, Tracy B Larsen, Paul N Fuller, Cassandra E Henderson
Journal: Case Rep Obstet Gynecol (2017)
MeSH terms: Case Reports

Amniotic fluid embolism (AFE) is the second leading cause of maternal mortality in the USA with an incidence of 1 : 15,200 births. The case fatality rate and perinatal mortality associated with AFE are 13-30% and 9-44%, respectively. This rare but devastating complication can be difficult to diagnose as many of the early signs and symptoms are nonspecific. Compounding this diagnostic challenge is a lack of effective treatment regimens which to date are mostly supportive. We present the case of a 26-year-old woman who suffered from suspected AFE and was successfully treated with the novel regimen of Atropine, Ondansetron, and Ketorolac (A-OK). The authors acknowledge that this case does not meet the new criteria proposed, by Clark in 2016, but feel that it is important to share this case report, due to dramatic patient response to the provided supportive therapy presented in this case report. We hope this case report will prompt further research into this novel approach to treating AFE with Atropine, Ondansetron, and Ketorolac.