OpenMed/multicare-articles huggingface dataset:
Tofacitinib Treatment of Refractory Cutaneous Leukocytoclastic Vasculitis: A Case Report
Authors: Kai-Jun Zhu, Pei-Dan Yang, Qiang Xu
Journal: Front Immunol (2021)
Keywords: jak inhibitor, case report, cutaneous leukocytoclastic vasculitis, inflammation, tofacitinib
MeSH terms: Janus Kinase Inhibitors / therapeutic use, Piperidines / therapeutic use, Pyrimidines / therapeutic use, Skin Ulcer / drug therapy, Vasculitis, Leukocytoclastic, Cutaneous / drug therapy, Case Reports, Adult, Drug Therapy, Combination, Female, Glucocorticoids / therapeutic use, Humans, Prednisone / therapeutic use, Skin Ulcer / diagnosis, Skin Ulcer / enzymology, Treatment Outcome, Vasculitis, Leukocytoclastic, Cutaneous / diagnosis, Vasculitis, Leukocytoclastic, Cutaneous / enzymology, Wound Healing / drug effects, Glucocorticoids, Janus Kinase Inhibitors, Piperidines, Pyrimidines, tofacitinib, Prednisone

Introduction:
To date, there is no treatment with proven efficacy for cutaneous leukocytoclastic vasculitis (CLV). Several reports have suggested that CLV responds favorably to corticosteroids, colchicine, nonsteroidal anti-inflammatory drugs (NSAIDs), azathioprine, and hydroxychloroquine (HCQ). To the best of our knowledge, the oral small molecule Janus kinase inhibitor, tofacitinib, plays an important role in the treatment of autoimmune and inflammatory diseases. Therefore, tofacitinib may be a prospective therapy in patients with CLV.
Case presentation:
A 29-year-old woman presented to our hospital with a 5-year history of symmetric skin lesions mainly affecting both lower extremities. The results for anti-neutrophil cytoplasmic antibodies (ANCA), anti-extracted nuclear antigens (ENA) autoantibodies, anti-double-stranded deoxyribonucleic acid (dsDNA) antibodies, and antinuclear antibodies (ANA) were all negative. The definite diagnosis of CLV was determined by a skin biopsy. However, the patient exhibited a poor response to prednisone, HCQ, methotrexate, colchicine, azathioprine, and tripterygium wilfordii polyglycoside tablets (TGTs) treatments. She was then treated with oral tofacitinib (5 mg twice daily) and oral prednisone (25 mg daily).
Outcomes:
Her skin lesions gradually improved over a period of 4 weeks. Two months later, the skin ulcers completely resolved. No evidence of recurrence of skin ulcers was observed during a 6-month follow-up.
Conclusion:
We present the first case of a female patient receiving short-term tofacitinib therapy for refractory CLV. Tofacitinib may be a promising oral alternative for patients with CLV. However, its efficacy and safety require further appraisal through clinical trials.
Case Report: Sudden death related to unrecognized cardiac hydatid cyst
Authors: Med Amin Mesrati, Yosra Mahjoub, Nouha Ben Abdejlil, Marwa Boussaid, Meriem Belhaj, Hiba Limem, Ali Chadly, Abdelfeteh Zakhama, Abir Aissaoui
Journal: F1000Res (2020)
Keywords: autopsy, hydatid cyst, pathology, right ventricle, sudden death
MeSH terms: Case Reports

Echinococcosis, also known as hydatid disease, is a common parasitic human infestation found in sheep-breeding areas. It is caused by the larvae stage of Echinococcus granulosus, and cysts develop mostly in the lungs and the liver. Cardiac involvement is unusual and silent until acute complications or a fatal outcome occurs. Herein, we report an autopsy case of a young healthy adult who died suddenly. The autopsy revealed an external bulging on the right heart ventricle outlet with a fluid-filled cystic cavity discovered on sectioning. Dissection of other organs did not reveal other cyst locations. Histological examination ascertained the diagnosis of hydatid cyst, and death was attributed to cardiac arrhythmias. Pathologists should keep in mind that hydatid cysts can develop anywhere in the body. Solitary cardiac cyst is rare and can simulate a "silent bomb". Unfortunately, sudden death remains the frequent manner of revelation of this disease in endemic areas.
From Public Health Policy to Impact for COVID-19: A Multi-Country Case Study in Switzerland, Spain, Iran and Pakistan
Authors: Maryam Tavakkoli, Aliya Karim, Fabienne Beatrice Fischer, Laura Monzon Llamas, Azam Raoofi, Shamsa Zafar, Carmen Sant Fruchtman, Don de Savigny, Amirhossein Takian, Marina Antillon, Daniel Cobos Muñoz
Journal: Int J Public Health (2022)
Keywords: covid-19, covid-19 restrictions, cross-country comparison, governance, health system, pandemic, policy responses, public healh
MeSH terms: COVID-19 / epidemiology, Health Policy, Humans, Iran / epidemiology, Pakistan / epidemiology, Pandemics, Spain, Switzerland / epidemiology

Objectives: With the application of a systems thinking lens, we aimed to assess the national COVID-19 response across health systems components in Switzerland, Spain, Iran, and Pakistan. Methods: We conducted four case studies on the policy response of national health systems to the early phase of the COVID-19 pandemic. Selected countries include different health system typologies. We collected data prospectively for the period of January-July 2020 on 17 measures of the COVID-19 response recommended by the WHO that encompassed all health systems domains (governance, financing, health workforce, information, medicine and technology and service delivery). We further monitored contextual factors influencing their adoption or deployment. Results: The policies enacted coincided with a decrease in the COVID-19 transmission. However, there was inadequate communication and a perception that the measures were adverse to the economy, weakening political support for their continuation and leading to a rapid resurgence in transmission. Conclusion: Social pressure, religious beliefs, governance structure and level of administrative decentralization or global economic sanctions played a major role in how countries' health systems could respond to the pandemic.
Giant aortic pseudoaneurysm with stanford type a aortic dissection after aortic valve replacement
Authors: Mertay Boran, Ali İhsan Parlar, Ertay Boran
Journal: Case Rep Med (2012)
MeSH terms: Case Reports

Giant pseudoaneurysm of the ascending aorta is a rare but dreadful complication occurring several months or years after aortic surgery. Thoracic aortic aneurysms tend to be asymptomatic and were previously often diagnosed only after a complication such as dissection or rupture. We present a rare case of giant ascending aneurysm with Stanford type A aortic dissection occurring 6 years after aortic valve replacement and also illustrate the potential dimensions the ascending aorta may reach by a pseudoaneurysm and dissection after AVR.
Synchronous Ganglioneuroma and Schwannoma Mistaken for Carotid Body Tumor
Authors: Konstantinos Paraskevopoulos, Angeliki Cheva, Styliani Papaemmanuil, Konstantinos Vahtsevanos, Konstantinos Antoniades
Journal: Case Rep Otolaryngol (2017)
MeSH terms: Case Reports

Ganglioneuromas are a very rare benign neural tumor, commonly derived from the ganglia of the sympathetic system, and are composed of mature Schwann cells, ganglion cells, and nerve fibres. They may arise anywhere from the base of the skull to the pelvis along the paravertebral sympathetic plexus. We report a rare case of synchronous ganglioneuroma and schwannoma, mistaken for carotid body tumor. The coexistence of these two entities in head and neck region is very rare.
The Combined Efficacy of a Two-Year Period of Cybernic Treatment With a Wearable Cyborg Hybrid-Assistive Limb and Leuprorelin Therapy in a Patient With Spinal and Bulbar Muscular Atrophy: A Case Report
Authors: Hideaki Nakatsuji, Tetsuhiko Ikeda, Atsushi Hashizume, Masahisa Katsuno, Gen Sobue, Takashi Nakajima
Journal: Front Neurol (2022)
Keywords: anti-androgen therapy, case report, creatine kinase, cybernic treatment, hybrid assistive limb, leuprorelin acetate, spinal and bulbar muscular atrophy
MeSH terms: Case Reports

Spinal and bulbar muscular atrophy (SBMA), also known as Kennedy's disease, is a rare, slowly progressive, incurable, and hereditary neurodegenerative disease caused by the testosterone-dependent accumulation of pathogenic polyglutamine-expanded androgen receptor protein. After extensive review, two treatments for SBMA have recently been approved in Japan; this decision was based on the results of randomized controlled trials: First, anti-androgen therapy using leuprorelin acetate (leuprorelin), a disease-modifying drug that can inhibit the progression of dysphagia but has not yet been proved to improve gait function; second, cybernic treatment with a wearable cyborg hybrid assistive limb (HAL®) (Cyberdyne Inc. Tsukuba, Japan). The HAL is an innovative walking exercise system that has been shown to significantly improve gait function in eight neuromuscular diseases without reduction in muscle function, including SBMA. It is possible that the combination of these two approaches might yield better outcomes. However, the long-term effects of such a combined approach have yet to be clinically evaluated. Here, we describe the case of a 39-year-old male with SBMA who commenced anti-androgen therapy with leuprorelin 1 year previously; this was followed by cybernic treatment with HAL. The duration of walking exercise with HAL was 20-30 min a day in one session. Over 2 weeks, the patient underwent nine sessions (one course). The efficacy of HAL was evaluated by gait function tests before and after one course of cybernic treatment. Then, leuprorelin treatment was combined with cybernic sessions every 2 months for 2 years (13 courses in total). Walking ability, as evaluated by the 2-min walk test, improved by 20.3% in the first course and peaked 10 months after the commencement of combined therapy (a 59.0% improvement). Walking function was maintained throughout the period. Generally, SBMA is characterized by moderately increased serum levels of creatine kinase (CK), reflecting neuromuscular damage; interestingly, the patient's CK levels decreased dramatically with combined therapy, indicating remarkable functional improvement. Long-term combined therapy improved the patient's gait function with a steady reduction in CK levels. The combination of leuprorelin with cybernic treatment can, therefore, improve and maintain gait function without damaging the motor unit and may also suppress disease progression.
Aortic shelf as a normal variant diagnosed primarily as the aortic dissection: A case report
Authors: Mehrnoush Toufan Tabrizi, Naser Khezerlouy-Aghdam, Venus Shahabi Raberi, Ahmad Jamei Khosroshahi
Journal: J Cardiovasc Thorac Res (2020)
Keywords: aortic shelf, descending thoracic aorta, transesophageal echocardiography, traumatic aortic dissection
MeSH terms: Case Reports

Traumatic aortic dissection is most commonly caused following sudden deceleration injury. It most commonly involves descending thoracic aorta (DTA) and is associated with high mortality and morbidity if not treated urgently. Confirmation of diagnosis often requires contrast-enhanced computed tomography (CECT) or magnetic resonance imaging (MRI), which is time consuming, expensive, and often not available at many health-care facility. Transesophageal echocardiography (TEE) is equally efficient to CECT and MRI in diagnosing aortic dissection. It may also provide additional information that can be very useful for the management of the patient. In some cases, the likelihood of error in the diagnosis of such a critical condition with normal cardiovascular variations is expected. Herein, we describe a case with primary diagnosis of aortic dissection that led to final diagnosis of an aortic shelf that medically managed with a good long-term prognosis. In patients suspected to aortic dissection due to any cause, the specialized evaluation using the most accurate and sensitive tools is strongly recommended to discriminate normal vascular variations from major vascular defects requiring emergent surgical interventions.
Case Report: Pediatric Chronic Active Epstein-Barr Virus Infection With Giant Sinus of Valsalva Aneurysms and Aorta and Its Branch Dilations
Authors: Qirui Li, Guyu Li, Daming Shao, Tharak Yarrabolu, Yuan Yue
Journal: Front Pediatr (2022)
Keywords: chronic active epstein-barr virus infection, multiple artery aneurysms, pediatrics–children, sinus of valsalva aneurysms, systemic vasculitis
MeSH terms: Case Reports

Chronic active Epstein-Barr virus infection (CAEBV), which often manifests as persistent infectious mononucleosis-like symptoms and can involve multiple organs, is a prolonged or reactivated status of primary EBV infection. Cardiovascular damage is one of the rare but severe complications correlated with poor prognosis among all CAEBV patients. Few published articles have demonstrated systemic arterial lesions involving branches of the aorta as cardiovascular complications. Herein, we report a rare pediatric case of CAEBV associated with giant sinus of Valsalva aneurysms and aorta and its branch dilations.
Scalp Angiosarcoma Remission with Bevacizumab and Radiotherapy without Surgery: A Case Report and Review of the Literature
Authors: Jocelyn T De Yao, Danyu Sun, Angela T Powell, Esther H Rehmus
Journal: Sarcoma (2011)
MeSH terms: Case Reports

Angiosarcoma (AS) is a rare and aggressive vascular neoplasm with very poor prognosis. Patients with extensive cutaneous AS who are not surgical candidates have very limited options since there is no standard treatment. Treatment options include radiation, chemotherapy, and angiogenesis inhibitor with varying success rates. Here, we report a case an 88 year old patient with extensive scalp angiosarcoma having biopsy proven remission with bevacizumab and radiotherapy without undergoing surgery.
Hepatic Iron Overload following Liver Transplantation from a C282Y/H63D Compound Heterozygous Donor
Authors: E Veitsman, E Pras, O Pappo, A Arish, R Eshkenazi, C Feray, J Calderaro, D Azoulay, Z Ben Ari
Journal: Case Reports Hepatol (2018)
MeSH terms: Case Reports

Hereditary hemochromatosis (HH) is a genetic disease associated with progressive iron overload, eventually leading in some cases to damage of parenchymal organs, such as the liver, pancreas, and heart. Although the gene had been identified (HFE), HH pathogenesis remains to be fully elucidated. We report here, for the first time, a case of inadvertent transplantation of a liver from a donor with C282Y/H63D compound heterozygosity into a nonhemochromatotic 19-year-old Caucasian male recipient with primary sclerosing cholangitis. Progressive iron overload occurred over 1.5 years, as observed in liver biopsies and iron studies, after ruling out secondary causes of iron overload. This case strengthens the hypothesis that the liver, rather than the small intestine, plays a primary role in the maintenance of iron homeostasis.
Case Report: CTC1 mutations in a patient with diffuse hepatic and splenic hemangiomatosis complicated by Kasabach-Merritt syndrome
Authors: Xin He, Zi-Wen Guo, Xiao-Min Niu
Journal: Front Oncol (2023)
Keywords: ctc1 gene, adult kasabach-merritt syndrome, combination therapy, diffuse hepatic hemangiomatosis, vascular disorder
MeSH terms: Case Reports

Diffuse hemangiomatosis of the liver and spleen is rare. Currently, few studies are available on diffuse hepatic and splenic hemangiomatosis accompanied by Kasabach-Merritt syndrome (KMS). The conserved telomere maintenance component 1 (CTC1) gene contributes to telomere maintenance and replication by forming the telomeric capping complex. Herein, we report a case of diffuse hemangiomatosis in the liver and spleen accompanied by KMS in a 59-year-old woman who carried two novel heterozygous CTC1 variants: c.435+9A>C and c.3074C>T (p.Ala1025Val). Using next-generation sequencing, we detected mutations in the CTC1 gene in our patient, who had chief complaints of fatigue and abdominal distension complicated by severe thrombocytopenia and consumptive coagulopathy. Clinical symptoms, laboratory tests, and imaging findings led to the diagnosis of diffuse hepatic and splenic hemangiomatosis accompanied by KMS. The patient was treated with prednisone, thalidomide, and sirolimus, and her general condition was ameliorated at the 4-month follow-up with improved platelet count and coagulation function. A CTC1 gene mutation may be involved in the pathological process of vascular diseases. A combination treatment regimen of prednisone, thalidomide, and sirolimus may be effective for KMS.
Remote Cerebellar Haemorrhage: A Potential Iatrogenic Complication of Spinal Surgery
Authors: Muhammad Atif Naveed, Rajiv Mangla, Hajra Idrees, Rashi I Mehta
Journal: Case Rep Neurol Med (2018)
MeSH terms: Case Reports

We report the case of a 51-year-old man with no significant past medical history, who underwent elective revision spinal surgery and subsequently developed intracranial hypotension, remote cerebellar haemorrhage (RCH), and mild hydrocephalus on the fourth postoperative day. Remote cerebellar haemorrhage is a known complication of supratentorial surgery. This iatrogenic phenomenon may also occur following spinal surgery, due to dural tearing and rapid cerebral spinal fluid (CSF) leakage, resulting in intracranial hypotension and cerebellar haemorrhage. This complication may result in severe permanent neurologic sequelae; hence, it is of pertinence to diagnose and manage it rapidly in order to optimise patient outcome.
Potential Association Between Anabolic Androgenic Steroid Abuse and Pituitary Apoplexy: A Case Report
Authors: Agne Andriuskeviciute, Giulia Cossu, Adelina Ameti, Georgios Papadakis, Roy Thomas Daniel, Vincent Dunet, Mahmoud Messerer
Journal: Front Endocrinol (Lausanne) (2022)
Keywords: anabolic steroid abuse, case report, pituitary apoplexy, pituitary neuroendocrine tumor, risk factors, testosterone abuse
MeSH terms: Pituitary Apoplexy / chemically induced, Pituitary Apoplexy / complications, Pituitary Apoplexy / diagnosis, Pituitary Neoplasms / chemically induced, Pituitary Neoplasms / complications, Case Reports, Research Support, Non-U.S. Gov't, Adult, Headache / complications, Humans, Male, Steroids, Testosterone, Steroids, Testosterone

Introduction:
Pituitary apoplexy (PA) is a rare, and potentially life-threatening condition, caused by hemorrhage or infarction into the pituitary gland with a rapid expansion of the contents of the sella turcica, associated with sudden intense headache, neurological and endocrinological deterioration. The identification of risk factors is crucial for prevention and optimal management. Herein we report a case of PA occurring 1 month after the initiation of anabolic androgenic steroid abuse for bodybuilding.
Case report:
A 40-year-old male patient presents with abrupt onset headache associated with left partial third cranial nerve palsy. The MRI shows a sellar lesion involving left cavernous sinus with a heterogenous anterior aspect of the lesion with hemorrhagic zones in favor of PA. Endocrine work-up shows high testosterone level in patient who was using exogenous testosterone without a medical prescription for a month.
Conclusion:
We report a case of PA of a pituitary neuroendocrine tumor occurring shortly after AAS. The association between PA and AAS should be considered as a potential risk.
Cystic schwannoma of the pelvis
Authors: T Jindal, S Mukherjee, M R Kamal, R K Sharma, N Ghosh, S N Mandal, A K Das, D Karmakar
Journal: Ann R Coll Surg Engl (2013)
MeSH terms: Neurilemmoma / surgery, Pelvic Neoplasms / surgery, Case Reports, Calcinosis / diagnosis, Calcinosis / surgery, Femoral Nerve, Humans, Hydronephrosis / etiology, Hypesthesia / etiology, Male, Middle Aged, Neurilemmoma / complications, Neurilemmoma / diagnosis, Pelvic Neoplasms / complications, Pelvic Neoplasms / diagnosis, Psoas Muscles, Scrotum / innervation, Urinary Bladder Neck Obstruction / etiology

Schwannomas are benign tumours that arise from the Schwann cells of nerve fibres. They commonly occur in the head and neck, mediastinum and extremities. They are extremely rare in the pelvis. These are usually slow-growing tumours and are often detected incidentally. Preoperative diagnosis is extremely difficult as there are no definitive signs on imaging. Aspiration biopsy is often inconclusive or misleading. Surgical excision is both diagnostic and therapeutic. As these tumours are often large in size, open excision is most commonly performed. We describe a case of a large, cystic schwannoma of the pelvis causing bladder outlet obstruction and bilateral hydroureteronephrosis. Complete surgical excision was performed laparoscopically.
Optimal Sense-Making and Resilience in Times of Pandemic: Integrating Rationality and Meaning in Psychotherapy
Authors: Pninit Russo-Netzer, Matti Ameli
Journal: Front Psychol (2021)
Keywords: covid-19 pandemic, logotherapy, meaning, rational emotive behavior therapy, rationality, resilience, self-transcendence
MeSH terms: Review

The global COVID-19 pandemic has triggered a wide variety of psychological crises worldwide. In order to respond rapidly and efficiently to the complex challenges, mental health professionals are required to adopt a multidimensional and integrative view. Rational Emotive Behavior Therapy (REBT) founded by Albert Ellis promotes rationality and self-acceptance. Logotherapy, pioneered by Viktor Frankl potentiates meaning and resilience. Both approaches are complementary and mutually enriching. The goal of this paper is to propose an integrative model of "optimal sense-making," a concept that combines both rationality and meaning, as well as the role of self-transcendence and healthy negative emotions. The model offers a theoretical and clinical foundation for efficient and effective psychological intervention plans for those affected by the pandemic. Along with theoretical background, illustrating case studies are presented to support potential application of the integrative model to affected individuals as well as the work of first-line health professionals during these times of pandemic. Implications are considered for utilizing theoretical and applied insights from the model to cultivate resilience in face of adversity and suffering.