OpenMed/multicare-articles huggingface dataset:
Langerhans Cell Histiocytosis: A Diagnostic Challenge in the Oral Cavity
Authors: Mehmet Ali Altay, Alper Sindel, Öznur Özalp, Burak Kocabalkan, İrem Hicran Özbudak, Ramazan Erdem, Ozan Salim, Dale A Baur
Journal: Case Rep Pathol (2017)
MeSH terms: Case Reports

Background:
Langerhans cell histiocytosis (LCH) is a rare disorder of the reticuloendothelial system with unknown etiology. This report aims to present a case of LCH with diffuse involvement of the oral cavity and to raise awareness of the distinguishing features of this diagnostically challenging entity.
Case report:
A 26-year-old male patient presented with complaints of teeth mobility, intense pain, and difficulty in chewing. Intraoral and radiological examinations revealed generalized gingival hyperplasia and severe teeth mobility with widespread alveolar bone loss. Periodontal therapy was performed with no significant improvement. An incisional biopsy revealed Langerhans cells and positive reaction to S-100 and CD1, and the patient was diagnosed with LCH. The patient underwent systemic chemotherapy with vinca alkaloids and corticosteroids. Regression of gingival lesions, as well as significant decrease in mobility of the remaining teeth and severity of pain, was achieved during 12 months of follow-up.
Conclusion:
The rarity and variable system involvement of LCH necessitate a multidisciplinary approach be carried out for accurate diagnosis, effective treatment, and an uneventful follow-up. Awareness of oral manifestations of LCH may aid clinicians greatly in reducing morbidity and mortality associated with this debilitating condition.
Percutaneous management of a long saphenous vein graft aneurysm: a case report and review of literature
Authors: Carmelo J Panetta, Williaim Schneider, Max A Boller
Journal: Cardiol Res Pract (2009)
MeSH terms: Case Reports

Aneurysms of saphenous vein grafts are rare but can result in complications such as myocardial infarction or death. Percutaneous treatment has included a variety of approaches, including covered stents. Long aneurysms in saphenous vein grafts pose an additional challenge due to the lack of coronary covered stents with sufficient length. We present successful treatment of a long saphenous vein graft aneurysm with use of peripheral covered stents over two coronary guidewires, a 55-centimeter 8-French sheath and no guide catheter.
Low-grade myxofibrosarcoma following a metal implantation in femur: a case report
Authors: Weisong Li, Dan Li, Xiansen Zhu, Shaohui Lu, Chunlei He, Qingchun Yang
Journal: Diagn Pathol (2014)
MeSH terms: Bone Neoplasms / etiology, Bone Plates / adverse effects, Bone Screws / adverse effects, Femur / pathology, Histiocytoma, Malignant Fibrous / etiology, Titanium / adverse effects, Case Reports, Adult, Alloys / adverse effects, Bone Neoplasms / pathology, Histiocytoma, Malignant Fibrous / pathology, Humans, Male, Neoplasm Grading, Alloys, Titanium

Myxofibrosarcoma is a myxoid variant of malignant fibrous histiocytoma that most commonly involves the extremities of elderly people. However, a primary myxofibrosarcoma with bone invasion in young adults is extremely rare. Herein, we report the case of a 31-year-old male with a gradually enlarging left thigh mass, who had a history of left femur fracture and received an open reduction and internal fixation with titanium alloy plates and screws 33 months previously. Imaging investigations revealed an irregularly shaped soft tissue mass around the left femur shaft and a partial bone defect in the middle one-third of the left femur. Pathological examination of the resected specimen showed a multi-nodular appearance, abundant myxoid matrix and elongated curvilinear capillaries. Immunohistochemical studies revealed that the tumor cells was positive for VIM and MDM2, and was negative for CK, MSA, SMA, DES, S-100 and CD34. Labeling index of Ki-67 was 25%. Based on the morphological finding and immunostaining, it was diagnosed as a low-grade myxofibrosarcoma. The clinical and imaging examinations did not reveal the evidence of a primary cancer elsewhere, and the patient had no personal or family history of malignancy. To our knowledge, this is the first case of a primary myxofibrosarcoma developed following a fracture and metal implantation in young adults.
Virtual slides:
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1745984882113605.
Sustained Remission in Patients with Primary Immune Thrombocytopenia after Romiplostim Tapering and Discontinuation: A Case Series in Real Life Management in Spain
Authors: María-Eva Mingot-Castellano, Carlos Grande-García, David Valcárcel-Ferreiras, Clara Conill-Cortés, Loreto de Olivar-Oliver
Journal: Case Rep Hematol (2017)
MeSH terms: Case Reports

Romiplostim, a thrombopoietin-receptor agonist (TPO-ra), is a highly effective option in primary immune thrombocytopenia (ITP), with 80-90% of patients achieving platelet responses after few weeks of treatment. The evidence showing remissions, that is, sustained platelet counts after romiplostim discontinuation, in patients with ITP refractory to immunosuppressive therapy is steadily increasing. However, there is a lack of guidelines or recommendations addressing how and when to taper romiplostim in clinical practice in patients maintaining elevated and stable platelet counts. Furthermore, given the high heterogeneity of ITP patients, no associated predictive factors have been currently identified. Here, we present 4 representative clinical cases of the daily clinical practice in Spain comprising newly diagnosed, persistent, and both splenectomized and nonsplenectomized chronic ITP patients treated with romiplostim, achieving and maintaining clinical remission (platelet count ≥ 50 × 109/L for 24 consecutive weeks in the absence of any treatment for ITP) after treatment tapering and discontinuation, without observed safety concerns. Prospective studies identifying clinical and biological predictive factors of sustained response are warranted.
Hyperacute corticosteroid treatment of optic neuritis at the onset of pain may prevent visual loss: a case series
Authors: G T Plant, N A Sibtain, D Thomas
Journal: Mult Scler Int (2011)

Aim. To show that high-dose corticosteroids may prevent visual loss in patients with optic neuritis (ON) treated at the prodromal, hyperacute, phase of retrobulbar pain. Method. Prospective case series: patients were recruited with a history of ON associated with pain. The patients were advised to report immediately to the investigators should the pain recur in either eye. Where possible, orbital magnetic resonance imaging (MRI) was performed to confirm a recurrence of ON and treatment with high-dose corticosteroids was commenced. Visual function and the patient's subjective account were monitored. Results. Eight patients (including cases of MS, CRION and NMO) presented in the hyperacute phase. MRI confirmed optic nerve inflammation in 5/5. Treatment was commenced immediately, and, in all cases, no visual loss ensued. Conclusion. MRI can be used to confirm acute optic neuritis prior to visual loss in the hyperacute phase. We suggest that treatment with high-dose corticosteroids may abort the attack and prevent loss of vision in patients with ON who are treated at the onset of pain. This has potential implications for the management of acute ON and also for our understanding of the pathogenesis and potential therapeutic targets in the neuroinflammatory conditions associated with ON.
Transesophageal Echocardiography Guided Partial Right Atrial Inflow Occlusion - A Case Study
Authors: Lucas Barboza, Rob Tanzola
Journal: POCUS J (2022)
Keywords: right atrial inflow occlusion, transesophageal echocardiography, balloon occlusion, thoracic endovascular aneurysm repair, transient hypotension
MeSH terms: Case Reports

Deployment of stent-grafts and other endovascular devices is a common technique for various vascular repair procedures. Induced, transient, periods of hypotension are essential to the precise deployment of a device as this minimizes displacement that can result from high pressure aortic flow. Partial inflow occlusion of the right atrium is a reliable, precise, and safe method of achieving this. We present a case where intraoperative transesophageal echocardiography (TEE) was used to guide and confirm balloon placement for right atrium inflow occlusion during a thoracic endovascular aneurysm repair (TEVAR) procedure for repair of an aortic dissection in a 67 year old male. This highlights a novel use of TEE in the context of endovascular surgery, and showcases an alternative method of reliably achieving transient hypotension.
Porphyromonas asaccharolytica as a Rare Causative Agent for Lemierre's Syndrome
Authors: Mridul Gupta, Radhika Annam, Joseph Bahgat, Margaret Eng
Journal: Case Rep Infect Dis (2018)
MeSH terms: Case Reports

Lemierre's syndrome is a rare disease associated with significant morbidity and mortality. It begins with an oropharyngeal infection, which spreads locally to involve the internal jugular vein causing thrombophlebitis, followed by distant spread and metastatic infections. Affected individuals are commonly young adults. Causative organisms are usually oropharyngeal flora, most commonly being the anaerobe Fusobacterium necrophorum. Porphyromonas asaccharolytica is a rare etiological agent with only three cases being reported in the literature. This case report describes a previously healthy 22-year-old man who initially presented with acute tonsillitis and was later found to have left internal jugular vein thrombophlebitis along with bilateral septic emboli to the lungs. The patient was treated with a five-week course of ampicillin-sulbactam and metronidazole. Subsequent imaging also showed progression of internal jugular vein thrombus, for which warfarin was given for three months for anticoagulation.
Two Consecutive Runs of Veno-Venous Extracorporeal Membrane Oxygenation in a Peripartum Patient with COVID-19 Acute Respiratory Distress Syndrome
Authors: Nicolò Sella, Tommaso Pettenuzzo, Michele Della Paolera, Giulio Andreatta, Annalisa Boscolo, Alessandro De Cassai, Luisa Muraro, Arianna Peralta, Paolo Persona, Enrico Petranzan, Francesco Zarantonello, Eugenio Serra, Paolo Navalesi
Journal: Case Rep Crit Care (2021)
MeSH terms: Case Reports

Veno-venous extracorporeal membrane oxygenation (V-V ECMO) may be required to treat critically ill patients with COVID-19-associated severe acute respiratory distress syndrome (ARDS). We report the case of a 43-year-old peripartum patient, who underwent two sequential V-V ECMO runs. The first extracorporeal support was established for COVID-19 ARDS, as characterized by severe hypoxemia and hypercapnia (arterial partial pressure of oxygen to inspired oxygen fraction ratio 85 mmHg and arterial partial pressure of carbon dioxide 95 mmHg) and reduction of respiratory system static compliance to 25 mL/cmH2O, unresponsive to mechanical ventilation and prone positioning. After 22 days of lung rest, V-V ECMO was successfully removed and ventilator weaning initiated. A second V-V ECMO was required 7 days later, because of newly onset ARDS due to Pseudomonas aeruginosa ventilator-associated pneumonia. The second V-V ECMO run lasted 12 days. During both V-V ECMO runs, anticoagulation and ventilator settings were titrated through bedside thromboelastometry and electrical impedance tomography, respectively, without major complications. The patient was successfully decannulated, weaned from mechanical ventilation, and finally discharged home without oxygen therapy. At one-month follow-up, she showed good general conditions and no sign of respiratory failure.
Immunotherapy and Gene Therapy: New Challenges in the Diagnosis and Management of Drug-Induced Liver Injury
Authors: Bénédicte Delire, Eleonora De Martin, Lucy Meunier, Dominique Larrey, Yves Horsmans
Journal: Front Pharmacol (2022)
Keywords: drug-induced liver injury (dili), gene therapy, immune checkpoint inhibitors, immune-mediated hepatitis, immunotherapy
MeSH terms: Review

In the last 5 years, the landscape of oncologic treatment has been deeply modified with the development and use of immune checkpoint inhibitors (ICIs) that exert their antitumoral effect by reverting the exhausted phenotype of tumor-infiltrating lymphocytes. This innovative therapeutic strategy has widely changed the prognosis of some advanced neoplastic diseases such as melanoma and lung cancer, providing durable remission for a significant number of patients. Unfortunately, immune-related adverse events (irAEs), especially ICI-induced hepatitis, may be very severe in some cases, impairing the prognosis of the patient. Guidelines available today on the diagnosis and management of ICI-induced hepatitis are mainly based on expert opinions and case series. This lack of large data is explained not only by the low incidence of hepatic adverse events but also by their clinical heterogeneity and variable severity. In this article, we will review the clinical, biological, and histological characteristics of ICI-induced liver injury. We will discuss the current knowledge on their pathological mechanisms and their therapeutic strategy based on immunosuppressive treatment for more severe cases. Regarding severity assessment, we will discuss the gap between the oncologist and the hepatologist's point of view, highlighting the need for multidisciplinary management. While initially developed for notably less frequent diseases than neoplastic ones, gene therapy is going to be a revolution for the treatment of diseases not responding to pharmacological therapy. Limited but growing data describe liver injury after the administration of such therapy whose exact physiopathology remains unknown. In this article, we will discuss the available data supporting the role of gene therapies in the onset of drug-induced liver injury and related mechanisms. We will describe the clinical context, the biological and histological features, and the management currently proposed.
Congenital tracheobronchomegaly (Mounier-Kuhn syndrome) in a 28-year-old Zambian male: a case report
Authors: Saifurrahman Shahin, Thijs Hoffman, Wouter van Es, Jan Grutters, Kondwelani Mateyo
Journal: Pan Afr Med J (2021)
Keywords: case report, mounier-kuhn syndrome, tracheobronchomegaly, zambia, bronchiectasis, recurrent respiratory tract infections
MeSH terms: Bronchiectasis, Tracheobronchomegaly / diagnosis, Case Reports, Adult, Bronchi, Bronchoscopy, Child, Humans, Male, Trachea

Congenital tracheobronchomegaly, also known as Mounier Kuhn Syndrome (MKS) is a rare respiratory disorder characterized by dilatation of the trachea and bronchi. We report a case of a 28-year-old male of African descent in Zambia, who presented with a history of chronic productive cough and repeated chest infections since childhood. He had been treated numerous times for lower respiratory tract infections, and had received empiric tuberculosis (TB) treatment based on chest radiograph findings, despite negative sputum microscopy and molecular tests for TB. Investigations revealed normal baseline blood results and sputum results. He however, had markedly increased levels of serum immunoglobulin E, and spirometry showed an obstructive pattern with significant post bronchodilator improvement. High-resolution computed tomography scan revealed tracheal dilatation, extensive bilateral bronchiectasis and tracheal and bronchial diverticula. The latter were also seen on bronchoscopy, confirming the diagnosis of Mounier-Kuhn syndrome. The patient was treated with combined inhaled corticosteroids and bronchodilators, as well as chest physiotherapy for mucus clearance, which led to improvement in his symptoms. Our case highlights how in low-resource settings, chronic lung diseases, particularly bronchiectasis, are often clinically and radiologically mistaken for and presumptively treated as TB (or its sequelae). Mounier-Kuhn syndrome, albeit rare, should be considered in the differential diagnosis of patients with recurrent lower respiratory tract infections or bronchiectasis. Multidisciplinary team meetings can help in the diagnosis of rare lung diseases.
Depressive Disorder With Panic Attacks After Replacement of an Intrauterine Device Containing Levonorgestrel: A Case Report
Authors: René Zeiss, Carlos Schönfeldt-Lecuona, Maximilian Gahr, Heiko Graf
Journal: Front Psychiatry (2020)
Keywords: anxiety, case report, depression, intrauterine device, levonorgestrel
MeSH terms: Case Reports

The levonorgestrel-releasing intrauterine system (LNG-IUS) is used as hormonal contraception by millions of women worldwide. It is considered as a safe device with low rates of systemic adverse drug reactions (ADRs). However, an emerging evidence suggest mood changes as ADRs. Whereas most of these studies report psychiatric ADRs after the first implantation of the LNG-IUS, it has to be considered that these may also occur after replacement, even when psychiatric symptoms were not evident at the time of the initial insertion. A potential explanation for the development of psychiatric ADRs in subsequent LNG-IUS may rely on fluctuations of sex hormones throughout the female life cycle with changing windows of vulnerabilities for developing mood disorders. Thus, the reliable contraception for women remains a continual challenge. We present the case of a 41-year-old woman that used the LNG-IUS (Mirena®) for contraception over 5 years without any complaints. Within the first weeks after insertion of the second LNG-IUS, she developed a depressive syndrome and anxieties. An extensive somatic, including gynecological examination revealed no pathological findings and a mental disorder was suggested. Due to the patient´s request and the recommendation of her psychiatrist, the device was removed and led to a remission of her mental complaints up to a 6- and 12-months follow-up. Beyond the mood changes considerably affecting her quality of life, the patient raised the concerns that she has never been informed about potential ADRs on mental health and her remarks regarding the potential association between psychiatric symptoms and the LNG-IUS were considered as groundless. With this case, we strengthen previous observations regarding mood changes under LNG-IUS. Moreover, we illustrate that psychiatric symptoms may also occur as ADRs during the subsequent insertion. Thus, we emphasize that psychiatric symptoms have to be clearly communicated as ADRs to patients with LNG-IUS within a written informed consent and should be routinely examined by gynecologists.
Case Report: Complicated Meckel Diverticulum Spectrum in Children
Authors: Gunadi, Wahyu Damayanti, Robin Perdana Saputra, Ramadhita, Ibnu Sina Ibrohim, Andi Lestiono, Devy Melati, Winda Intan Permatahati, Titis Widowati, Akhmad Makhmudi
Journal: Front Surg (2021)
Keywords: meckel diverticulum (complications), meckel diverticulum (diagnosis), meckel diverticulum—surgery, children, diagnostic challenges, exploratory laparotomy, segmental small bowel resection and primary anastomosis
MeSH terms: Case Reports

Background: Meckel diverticulum (MD) is the most common congenital anomaly of the intestines, with an incidence of 2% of the general population. It can present as various clinical features with complications and be life threatening if diagnosis is delayed and treatment late. Case Presentation: We report three pediatric cases with complicated MD: one female presented with small-bowel obstruction, one male with peritonitis, and one female with severe iron-deficiency anemia, without gross gastrointestinal bleeding nor any ectopic gastric mucosa. All patients underwent exploratory laparotomy, segmental small-bowel resection, and primary anastomosis. They successfully recovered and were uneventfully discharged on the fourth, seventh, and 10th postoperative days, respectively. Conclusions: MD can present with various complication spectrums, including small-bowel obstruction, peritonitis, and severe iron-deficiency anemia, which may cause difficulty in definitive diagnosis, particularly in children. Segmental small-bowel resection and primary anastomosis are effective surgical approaches and show good outcomes for MD patients.
Can takotsubo cardiomyopathy be diagnosed by autopsy? Report of a presumed case presenting as cardiac rupture
Authors: Andrew Mitchell, François Marquis
Journal: BMC Clin Pathol (2017)
Keywords: autopsy, cardiac, cardiomyopathy, rupture, stress, takotsubo
MeSH terms: Case Reports

Background:
Takostsubo (stress) cardiomyopathy (TC) is a clinical syndrome featuring transient left ventricular dysfunction and wall-motion abnormalities, usually following emotional or physical stress. The diagnosis of TC depends on fulfillment of multiple clinical criteria. Although the pathogenesis has not been firmly established, myocardial cathecholamine toxicity is thought to represent a primary mechanism. The vast majority of patients with TC survive. However, a rare cause of death in TC is myocardial rupture. All documented cases of rupture have followed known, recently diagnosed or suspected TC. However, in this report we propose that an initial diagnosis of TC with myocardial rupture can be made by autopsy when supported by a compelling clinical history and appropriate histologic changes in the myocardium.
Case presentation:
An 82 year-old female underwent elective craniotomy for a recently discovered craniopharyngioma. The surgery was uneventful; the initial postoperative course featured diabetes insipidus and delirium. With no prior warning, on the third postoperative day she was found unresponsive in bed. Two prolonged cardiopulmonary resuscitations were successful, however, during a third arrest maneuvers were stopped at the request of the family. An autopsy was conducted which revealed hemopericardium due to cardiac rupture. Coronary artery atherosclerosis, valve disease, and renal and extra-renal pheochromocytoma were absent. Microscopy of the myocardium showed a recent, localized, transmural myocardial infarction and diffuse changes (all four ventricles) typical of cathecholamine cardiomyopathy. The findings were considered compatible with TC with secondary myocardial rupture.
Conclusion:
An initial diagnosis of TC with myocardial rupture can be reasonably made by autopsy in the context of an appropriate clinical history and the presence of the characteristic microscopic features of cathecholamine excess in the myocardium.
Pregnancy Outcome and Postnatal Chromosome Analysis of the Cord Blood and Chorionic Villi in Two Cases after Intrauterine Transfer of Mosaic Aneuploid Blastocysts
Authors: Yuki Ito, Taizan Kamide, Kosuke Taniguchi, Taisuke Sato, Michihiro Yamamura, Akiko Konishi, Ken Takahashi, Hiroshi Kishi, Kenichiro Hata, Osamu Samura, Aikou Okamoto
Journal: Case Rep Obstet Gynecol (2022)
MeSH terms: Case Reports

The long-term prognosis and genetic mechanism of pregnancy after intrauterine mosaic aneuploid blastocyst transfer remain unknown. We report the case of two babies after the aforementioned procedure and chromosomal analysis of their cord blood and chorionic villi. Case Report 1. A 41-year-old primipara, with two previous spontaneous abortions, was pregnant after intrauterine transfer of a blastocyst carrying 40% mosaicism of long-arm monosomy of chromosome 5. The amniocentesis results were 46,XX. A cesarean section was performed at 39 weeks. The female infant was 3,315 g at birth. Case Report 2. A 44-year-old primipara, with two spontaneous abortions, was pregnant after intrauterine transfer of a blastocyst carrying 40% mosaicism of long-arm monosomy of chromosome 9 and monosomy of chromosome 14. After genetic counselling, she decided not to undergo amniocentesis. No abnormalities were found by ultrasound. A cesarean section was performed at 38 weeks. The male infant was 3,340 g at birth. Chromosome analyses of postnatal cord blood and chorionic villi were performed using SNP arrays. The cord blood and chorionic villi showed no chromosomal structural abnormalities or mosaicism. For both, no disorders were observed at 10 months of age. We experienced the birth of babies after intrauterine transfer of mosaic aneuploid blastocysts.