OpenMed/multicare-articles huggingface dataset:
Neurofascin (NF)155- and NF186-Specific T Cell Response in a Patient Developing a Central Pontocerebellar Demyelination after 10 Years of CIDP
Authors: Juliane Klehmet, Max Staudt, Jan-Markus Diederich, Eberhard Siebert, Edgar Meinl, Lutz Harms, Andreas Meisel
Journal: Front Neurol (2017)
Keywords: ccpd, cidp, atypical, neurofascin, neurofascin 155
MeSH terms: Case Reports

Background:
Information and pathobiological understanding about central demyelinating manifestation in patients, who primarily suffer from chronic inflammatory demyelinating polyneuropathy (CIDP), are scarce.
Methods:
IFN-γ-response as well as antibodies against the (para)nodal antigens neurofascin (NF)155 and NF 186 had been tested by Elispot assay and ELISA before clinical manifestation and at follow-up.
Case description and results:
The patient described here developed a subacute brainstem syndrome more than 10 years after diagnosis of CIDP under low-dose maintenance treatment of intravenous immunoglobulins (IVIG). MRI revealed enhancing right-sided pontocerebellar lesion. CSF examination showed mild pleocytosis and elevated protein, and negative oligoclonal bands. Further diagnostics exclude differential diagnoses such as tuberculoma, sarcoidosis, or metastasis. Specific IFN-γ response against NF155 and NF186 as measured by Elispot assay was elevated before clinical manifestation. NF155 and NF186 antibodies were negative. Escalation of IVIG treatment at 2 g/kg BW followed by 1.4 g/kg BW led to clinical remission albeit to a new asymptomatic central lesion. Follow-up NF155 and NF186-Elispot turned negative.
Conclusion:
The case reported here with a delayed central manifestation after an initially typical CIDP and NF155 and NF186 T cell responses does not resemble described cases of combined central and peripheral demyelination but may reflect a novel subtype within the great clinical heterogeneity of CIDP.
Outcome of intensive outpatient rehabilitation and bracing in an adult patient with Scheuermann's disease evaluated by radiologic imaging-a case report
Authors: Hagit Berdishevsky
Journal: Scoliosis Spinal Disord (2016)

Background:
No studies examine the efficacy of intensive specific physical therapy (PT) exercises along with brace for the adult with Scheuermann's kyphosis (SK). The aim of this study was to examine the effects of intensive PT based on the Barcelona Scoliosis Physical Therapy School (BSPTS) and SpinoMed brace on a 76-year-old female with SK.
Case description:
A 76-year-old female, diagnosed with SK as an adolescent, presented in October 2014 with thoracic hyperkyphosis T1 to T12 Cobb angle of 85° and lumbar hyper lordosis L1 to L5 Cobb angle of 70°. Lumbar scoliosis T12-L5 with 21° Cobb and vertebral rotation 2. Trunk translation in the sagittal plan was 4.5 cm. Intermittent low back pain 6/10 at worst. Quality-of-life score was 3.8 (SRS 22 questionnaire).
Method:
The PT regimen included one-hour Schroth exercise sessions three times per week for 6 months. In addition, a home exercise program (HEP) was recommended. Patient also wore a SpinoMed brace for 2 h per day. All tests and measurements were recorded before and after treatment.
Results:
After a six-month treatment period the kyphosis Cobb angle was reduced to 70° and the lordosis Cobb angle improved to 57°. A recent x-ray (October 2015) showed another improvement in the sagittal plane with thoracic kyphosis measuring 64° and lumbar lordosis 55°. Lumbar curvature decreased to 12° and vertebral rotation to 1. The quality-of-life score showed improvement with a score of 4.5 on the SRS 22. Pain score diminished to 2. Trunk deviation improved by 2.2 cm.
Conclusion:
These findings suggest that intensive and specific PT and bracing were successful for the treatment of this adult patient with SK.
Fatal basilar thrombosis possibly related to minor cervical trauma: a case report
Authors: Elmaz Shaqiri, Gentian Vyshka, Admir Sinamati, Besim Ymaj, Zija Ismaili
Journal: Case Rep Med (2010)
MeSH terms: Case Reports

Background. Basilar thrombosis is a potentially fatal event, whose traumatic etiology has been repeatedly stated. Methods. We performed the autopsy and the microscopic examination of the brain stem structures from an individual, whose sudden death raised logical suspicions regarding the causative factor. Results. The brain was swollen and clearly hyperemic; a massive basilar thrombus with complete occlusion of the median segment of the basilar artery was macroscopically seen. The brainstem showed an infarcted zone in the pons, with secondary hemorrhagic changes, mainly in the form of multiple petechial hemorrhages. Pontine arteries showed extensive alterations, mainly in the form of severed endothelium, which suggested a thrombotic-traumatic mechanism as the main etiological factor. Conclusions. Minor injuries, such as slight whiplashes, abrupt neck movements, neck trauma related to the slowing down of the vehicles, and critical neck positioning, can all of them explain a thrombotic event in the basilar artery, leading to a fatal occurrence. Other risk factors may obviously concur, but their importance seems unclear.
Atypical hemolytic uremic syndrome post-kidney transplantation: two case reports and review of the literature
Authors: Sami Alasfar, Nada Alachkar
Journal: Front Med (Lausanne) (2014)
Keywords: ahus, eculizumab, genetic mutation, kidney transplant, recurrence
MeSH terms: Review

Atypical hemolytic uremic syndrome (aHUS) is a rare disorder characterized by over-activation and dysregulation of the alternative complement pathway. Its estimated prevalence is 1-2 per million. The disease is characterized by thrombotic microangiopathy, which causes anemia, thrombocytopenia, and acute renal failure. aHUS has more severe course compared to typical (infection-induced) HUS and is frequently characterized by relapses that leads to end stage renal disease. For a long time, kidney transplantation for these patients was contraindicated because of high rate of recurrence and subsequent renal graft loss. The post-kidney transplantation recurrence rate largely depends on the pathogenetic mechanisms involved. However, over the past several years, advancements in the understanding and therapeutics of aHUS have allowed successful kidney transplantation in these patients. Eculizumab, which is a complement C5 antibody that inhibits complement factor 5a and subsequent formation of the membrane-attack complex, has been used in prevention and treatment of post-transplant aHUS recurrence. In this paper, we present two new cases of aHUS patients who underwent successful kidney transplantation in our center with the use of prophylactic and maintenance eculizumab therapy that have not been published before. The purpose of reporting these two cases is to emphasize the importance of using eculizumab as a prophylactic therapy to prevent aHUS recurrence post-transplant in high-risk patients. We will also review the current understanding of the genetics of aHUS, the pathogenesis of its recurrence after kidney transplantation, and strategies for prevention and treatment of post-transplant aHUS recurrence.
Looks like Tuberculous Meningitis, But Not: A Case of Rhinocerebral Mucormycosis with Garcin Syndrome
Authors: HongNa Yang, CuiLan Wang
Journal: Front Neurol (2016)
Keywords: garcin syndrome, rhinocerebral mucomycosis, tuberculous meningitis
MeSH terms: Case Reports

Rhinocerebral mucomycosis (RCM) as an emerging opportunistic, angioinvasive, and devastating fungi infection with high mortality is difficult to be diagnosed early because of the lack of specific clinical features or manifestations. Garcin syndrome is more often caused by skull base and rhinopharyngeal tumors or metastases, and basal meningitis. We reported that an aged diabetic man, involved nearly all cranial nerves (Garcin syndrome), who was at first suspected to be suffered from tuberculous meningitis, ultimately developed typically progressing RCM. Diagnosis was made to find the presence of mucormycosis in the infected tissue by biopsy.
Laparoscopic Management of Bouveret's Syndrome after Failed Endoscopic Approach
Authors: Ariel Nicolas Tchercansky, Guido Luis Busnelli, Matías Mihura, Rafael José Maurette
Journal: Case Rep Surg (2019)
MeSH terms: Case Reports

Bouveret's syndrome is a complication of cholelithiasis that presents with gastric outlet obstruction due to an impacted gallstone in the duodenum following cholecystoduodenal fistula. This is a rare presentation of biliary-enteric fistula; therefore, there are no standardized guidelines for the management of this disease. We present a case of a patient with Bouveret's syndrome managed with laparoscopic surgery after an unsuccessful attempt of endoscopic removal.
Unusual Endoscopic Findings in Children: Esophageal and Gastric Polyps: Three Cases Report
Authors: Smaranda Diaconescu, Ingrith Miron, Nicoleta Gimiga, Claudia Olaru, Ileana Ioniuc, Iulia Ciongradi, Ioan Sarbu, Gabriela Stefanescu
Journal: Medicine (Baltimore) (2016)
MeSH terms: Endoscopy, Digestive System, Esophageal Diseases / diagnosis, Polyps / diagnosis, Stomach Diseases / diagnosis, Case Reports, Adolescent, Esophageal Diseases / pathology, Esophagus / pathology, Female, Humans, Male, Polyps / pathology, Stomach / pathology, Stomach Diseases / pathology

Isolated polyps of the upper digestive tract are rarely diagnosed in children, being usually an incidental finding during endoscopic exploration.The diagnostic, therapy, and outcome of these lesions are based on endoscopy and pathology.In a 5-year period, clinical features, topography, size, pathology, therapeutics, and progression of esophagogastric polyps founded in children addressed to our pediatric gastroenterology unit were studied.The authors encountered 3 lesions in teenagers aged 13 to 17 years two males (2M), from a total number of 2140 upper digestive endoscopies (0.14%). All patients presented with pirosis, epigastric pain, and vomits; one of the children had end-stage renal disease and Kabuki syndrome. Endoscopic and pathologic findings were 2 esophageal polyps, an inflammatory one, and another containing goblet cells and a double-headed hyperplastic gastric polyp. Two patients received proton pump inhibitors without any improvement in subsequent endoscopic evaluations.The difficulties related to age group, underlying conditions, debatable response to acid suppression, and limited experience in pediatric therapeutic endoscopy selected significantly the effectiveness of treatment.The rarity of these lesions requires an individualized management, the endoscopic diagnostic, and therapeutic gesture depending on the symptoms, type, location, comorbidities, and team experience.
Carbon Dioxide Laser Microsurgical Median Glossotomy for Resection of Lingual Dermoid Cysts
Authors: Kristien Corvers, Greet Hens, Jeroen Meulemans, Pierre Delaere, Robert Hermans, Vincent Vander Poorten
Journal: Front Surg (2016)
Keywords: co2 laser, lingual dermoid, median glossotomy
MeSH terms: Case Reports

Dermoid cysts are epithelial-lined cavities with skin adnexae in the capsule. Only 7% is present in the head and neck. Between 2004 and 2013, four patients with a lingual dermoid cyst underwent a microsurgical carbon dioxide laser resection via a median sagittal glossotomy approach. This approach is an elegant technique combining superior visualization, hemostasis, and little postoperative edema with good wound healing, allowing for perfect function preservation of the tongue.
Tacrolimus associated posterior reversible encephalopathy syndrome - a case series and review
Authors: Susmitha Apuri, Kristin Carlin, Edward Bass, Phuong Thuy Nguyen, John N Greene
Journal: Mediterr J Hematol Infect Dis (2014)
MeSH terms: Case Reports

Tacrolimus is an immunosuppressive drug mainly used to lower the risk of transplant rejection in individuals who are post solid organ or hematopoietic transplantation. It is a macrolide which reduces peptidyl-propyl isomerase activity and inhibits calcineurin, thus inhibiting T-lymphocyte signal transduction and interleukin-2 (IL-2) transcription. It has been associated with Posterior Reversible Encephalopathy Syndrome (PRES), a disease of sudden onset that can present as a host of different symptoms, depending on the affected area of the brain. While infectious causes of encephalopathy must always be entertained, the differential diagnosis should also include PRES in the appropriate context. We report three cases of PRES in patients with acute myeloid leukemia (AML) placed on tacrolimus after receiving a bone marrow transplant (BMT). The focus of this review is to enhance clinical recognition of PRES as it is related to an adverse effect of Tacrolimus in the setting of hematopoietic transplantation.
TTF-1 Positive Primary Small Cell Carcinoma of the Breast: A Case Report and Review of the Literature
Authors: Hinda Boutrid, Mahmoud Kassem, Gary Tozbikian, Evan Morgan, Julia White, Manisha Shah, Jeffrey Vandeusen, Sagar Sardesai, Nicole Williams, Daniel G Stover, Maryam Lustberg, Robert Wesolowski, Vinay Pudavalli, Terence M Williams, Bhavana Konda, Stephanie Fortier, David Carbone, Bhuvaneswari Ramaswamy, Mathew A Cherian
Journal: Front Endocrinol (Lausanne) (2020)
Keywords: pdl-1, ttf-1, breast cancer, neuroendocrine cancer, small cell cancer
MeSH terms: Breast Neoplasms / pathology, Carcinoma, Small Cell / pathology, DNA-Binding Proteins / metabolism, Transcription Factors / metabolism, Case Reports, Review, Breast Neoplasms / metabolism, Breast Neoplasms / therapy, Carcinoma, Small Cell / metabolism, Carcinoma, Small Cell / therapy, Combined Modality Therapy, Female, Humans, Middle Aged, Prognosis, DNA-Binding Proteins, TTF1 protein, human, Transcription Factors

Primary small cell carcinoma of the breast (SCCB) is a rare tumor subtype comprising <0.1% of all breast carcinomas. Here we present a case of thyroid transcription factor-1 (TTF-1) positive SCCB that recurred within 3 years of diagnosis in the lung and lymph nodes. Given the small number of cases, no clear guidelines exist on the appropriate management of patients with these aggressive tumors. We present a case study and review the current literature to highlight the knowledge gaps and needs of patients with these rare tumors. A 50-year-old premenopausal woman with no family history, presented with a palpable right breast mass. Biopsy was consistent with primary SCCB that was poorly differentiated, positive for synaptophysin and chromogranin and TTF-1 and presence of ductal carcinoma in situ component showing neuroendocrine differentiation. Imaging with PET, CT, and MRI brain excluded any other sites of primary disease. She underwent a right lumpectomy with axillary lymph node dissection and was treated with adjuvant cisplatin-based chemotherapy and concurrent radiation therapy. Thirty-four months later, routine scans showed a new right lower-lobe lung nodule and an enlarged sub-carinal node that was proven to be poorly differentiated neuroendocrine cancer. This case report sheds light on a rarely described disease and provides a comprehensive approach to diagnosis and management. Primary SCCB is an extremely rare, aggressive form of breast cancer that is molecularly and histologically similar to SCLC. However, a review of the literature highlights recent mutational analyses that show important differences between these two cancer types, including an increase in PIK3CA mutations in primary SCCB. Further studies, including genomic analyses are needed to better define this malignancy and to develop a standard treatment.
Human papillomavirus-related multiphenotypic sinonasal carcinoma: a clinico-pathological dilemma case report
Authors: Nouha Ben Abdeljelil, Samiha Mabrouk, Souheil Khalfaoui, Mahdi Farjaoui, Ahlem Bellalah, Abdefattah Zakhama, Rim Hadhri
Journal: Pan Afr Med J (2021)
Keywords: human papillomavirus, carcinoma, case report, immunohistochemistry, sinonasal tract
MeSH terms: Papillomavirus Infections / complications, Paranasal Sinus Neoplasms / diagnosis, Case Reports, Disease Progression, Humans, Immunohistochemistry, Male, Middle Aged, Paranasal Sinus Neoplasms / pathology, Paranasal Sinus Neoplasms / virology, Tomography, X-Ray Computed

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), is a new entity that is restricted to the sinonasal tract and is associated with high-risk HPV. This tumor is suggested to have an indolent behavior with a better prognosis than other carcinomas. We report a unique case of HMSC with a locally aggressive behavior. It is about a 61-year-old men presented with 12 months of unilateral progressive olfactory dysfunction accompanied by exophthalmia of the left eye, declining vision and headaches for 6 months. Computed tomography imaging revealed a voluminous mass occupying the ethmoid, maxillary and frontal sinus with bony destruction of the left ethmoidal blade. Histology showed a tumor composed of variably sized nests, separated by thick mucoid stroma. Tumor cells are plasmacytoid with hyperchromatic nuclei and frequent mitoses. Immunohistochemistry revealed that these cells were positive for cytokeratin AE1/AE3, p16 and negative for CK7, CK20, CD117, p40, p63, S100, synaptophysin and chromogranin.
Ruptured liver cavernous hemangioma - rare cause of hemoperitoneum
Authors: O C Goidescu, T Patrascu
Journal: J Med Life (2015)
Keywords: cavernous hemangioma liver hemoperitoneum, liver cirrhosis
MeSH terms: Hemangioma, Cavernous / complications, Hemoperitoneum / etiology, Liver Neoplasms / complications, Case Reports, Aged, Hemangioma, Cavernous / surgery, Hemoperitoneum / surgery, Humans, Intraoperative Care, Liver Neoplasms / surgery, Male, Rupture, Spontaneous / complications

We present the case of a 70-year-old patient admitted with strong abdominal pains and operated in our service for hemorrhagic acute abdomen. Intraoperative hepatic cavernous hemangioma was found with capsular rupture and hemoperitoneum. Due to liver cirrhosis, and no proper technical equipment we chose to perform simple hemostasis. Postoperative evolution was favorable.
Post-traumatic skin and soft tissue infection due to Aeromonas hydrophila
Authors: Bijayini Behera, Sandeep Bhoriwal, Purva Mathur, Sushma Sagar, Maneesh Singhal, M C Misra
Journal: Indian J Crit Care Med (2011)
Keywords: aeromonas hydrophila, skin and soft tissue infection, trauma
MeSH terms: Case Reports

We report a case of posttraumatic skin and soft tissue infection in a patient who sustained laceration after being hit by a water tanker. Aeromonas hydrophila was isolated from pus and was identified to the species level by Vitek 2 and a battery of biochemical tests. The patient responded to thorough drainage, debridement of wound and 2 weeks of intravenous antibiotics. The patient was taken up for split skin grafting of the raw area. She was discharged with satisfactory graft uptake after 1 week without any further antibiotics advice. Follow-up after 3 weeks was satisfactory with healthy cover on the raw area and normal weight bearing on the left leg.
Primary burkitt lymphoma of the chest wall
Authors: Rommel Lu
Journal: Case Rep Hematol (2012)
MeSH terms: Case Reports

Burkitt lymphoma (BL) originating in the skin and soft tissue at any site is exceedingly rare. This paper is about a case of primary sporadic BL that presented as an isolated, rapidly enlarging chest wall mass arising from skin and/or soft tissue in an adult. As with other BL presentations, this patient was treated with aggressive chemotherapy with central nervous system (CNS) chemoprophylaxis, but he later died because of sepsis.
Sonographic Demonstration of Intracranial Hemorrhage in a Fetus with Hydrops Fetalis due to Rh Alloimmunization after Intrauterine Intravascular Transfusion: A Case Report and Review of the Literature
Authors: Rauf Melekoglu, Ebru Celik, Hasim Kural
Journal: Case Rep Obstet Gynecol (2018)
MeSH terms: Case Reports

Intrauterine transfusion is the most common and successful intrauterine procedure for the treatment of fetal anemia due to red cell alloimmunization. Fetal intracranial hemorrhage is a very rare complication of intrauterine transfusion in patients with Rh(D) alloimmunization and it has been demonstrated only in a few case reports in the literature. Herein, we described a case of grade IV intraventricular hemorrhage that was diagnosed following the first intrauterine transfusion and reviewed the literature about the fetal intracranial hemorrhage that occurred after intrauterine intravascular transfusion procedure.
Advancing the strategic use of HIV operations research to strengthen local policies and programmes: the Research to Prevention Project
Authors: Deanna Kerrigan, Caitlin E Kennedy, Alison Surdo Cheng, Sarah J Sandison, Virginia A Fonner, David R Holtgrave, Heena Brahmbhatt
Journal: J Int AIDS Soc (2015)
Keywords: hiv, usaid, implementation science, operations research, prevention
MeSH terms: HIV Infections / prevention & control, Health Services Research, Operations Research, Research Support, N.I.H., Extramural, Research Support, U.S. Gov't, Non-P.H.S., Africa, Northern, Caribbean Region, Health Policy, Humans

In the field of HIV prevention, there is renewed interest in operations research (OR) within an implementation science framework. The ultimate goal of such studies is to generate new knowledge that can inform local programmes and policies, thus improving access, quality, efficiency and effectiveness. Using four case studies from the USAID-funded Research to Prevention (R2P) project, we highlight the strategic use of OR and the impact it can have on shaping the focus and content of HIV prevention programming across geographic and epidemic settings and populations. These case studies, which include experiences from several sub-Saharan African countries and the Caribbean, emphasize four unique ways that R2P projects utilized OR to stimulate change in a given context, including: (1) translating findings from clinical trials to real-world settings; (2) adapting promising structural interventions to a new context; (3) tailoring effective interventions to underserved populations; and (4) prioritizing key populations within a national response to HIV. Carefully crafted OR can bridge the common gap that exists between research-generated knowledge and field-based practice, lead to substantial, real-world changes in national policies and programmes, and strengthen local organizations and the use of data to be more responsive to a given topic or population, ultimately supporting a locally tailored HIV response.
Ovarian Clear Cell Carcinoma with Functioning Stroma and Associated Endometrial Intraepithelial Neoplasia
Authors: Fabian Desimpel, Jean-Christophe Noël, Laurine Verset, Nicolas Sirtaine, Frédéric Buxant
Journal: Case Rep Obstet Gynecol (2022)
MeSH terms: Case Reports

We report the case of a 79-year-old woman with a large pelvic mass and postmenopausal bleeding, associated with hyperestrogenism. A pelvic MRI shows the presence of a large mass of 12.6 cm originating from the right ovary without signs of metastasis. A total abdominal hysterectomy with unilateral salpingooophorectomy was performed, knowing the patient underwent a left salpingooophorectomy decades ago. The pathological findings showed an ovarian clear cell carcinoma (pT1A) with associated endometrial intraepithelial neoplasia. There is convincing evidence that the production of estrogen is located in the activated ovarian stroma. This supports the view that functioning stroma of ovarian cancer can lead to hyperestrogenism and eventually endometrial cancer.