OpenMed/multicare-articles huggingface dataset:
Refractory Myasthenia Gravis and Concurrent Alopecia Areata Postthymectomy With Improvements After Cortisone Taper: A Case Report
Authors: Baraa Alghalyini, Huda Dahman, Abdul Rehman Zia Zaidi, Fathima Aasiya Tehreemah Raziq, Mohammad Amin Alswes
Journal: Case Rep Endocrinol (2024)
Keywords: alopecia areata, cortisone taper, postthymectomy, refractory myasthenia gravis
MeSH terms: Case Reports

This case report presents a unique clinical scenario of a 58-year-old male suffering from severe refractory myasthenia gravis and concurrent alopecia areata postthymectomy. Myasthenia gravis, a common autoimmune disorder, is characterized by muscle weakness due to autoantibodies targeting neuromuscular junction proteins. Alopecia areata, another autoimmune disease, is often seen in individuals with myasthenia gravis, suggesting a shared immunological basis. The patient's condition was resistant to conventional treatment, and he developed alopecia areata following thymectomy. Despite the challenges in managing refractory myasthenia gravis and the associated alopecia areata, significant improvements were observed following a cortisone taper. This case highlights the potential therapeutic role of cortisone tapering in managing refractory myasthenia gravis and associated alopecia areata. This case also prompts further exploration into the immunological shifts following thymectomy, particularly its potential role in triggering alopecia areata.
Case Report: Successful endovascular treatment of acute type A aortic dissection
Authors: Leonard Pitts, Roland Heck, Matteo Montagner, Adam Penkalla, Markus Kofler, Volkmar Falk, Jörg Kempfert, Semih Buz
Journal: Front Cardiovasc Med (2023)
Keywords: tevar, aortic dissection, ascending aorta, endovascular, type a
MeSH terms: Case Reports

Introduction:
Open surgical repair remains the current gold standard for the treatment of acute type A aortic dissection. However, especially elderly patients with relevant comorbidities who are deemed unfit for open surgery may benefit from a minimally invasive endovascular approach.
Methods:
We report a case of an 80-year-old male with retrograde acute type A aortic dissection and peripheral malperfusion after receiving thoracic endovascular aortic repair due to thoracic aortic aneurysm. Our individualized endovascular approach consisted of left carotid-subclavian bypass, proximal extension of thoracic endovascular aortic repair using a covered stent graft and a single covered stent graft for the ascending aorta in combination with an uncovered stent for the aortic arch.
Results:
Postoperative computed tomographic angiography demonstrated excellent outcome with no signs of endoleak or patent false lumen. Follow-up after 3.5 years showed a stable result with no signs of stent failure or dissection progress. No aortic re-interventions were needed in the further course.
Discussion:
An individualized endovascular approach may be justified for acute type A aortic dissection in elderly patients with high surgical risk if performed in specialized aortic centers. Additional short-length stent graft devices are needed to address the anatomical challenges of the ascending aorta. For enhanced remodeling of the dissected aorta, the use of an additional uncovered stent may be advisable.
Case Report: Diabetes mellitus type MODY5 as a feature of 17q12 deletion syndrome with diabetic gastroparesis
Authors: Sixu Xin, Xiaomei Zhang
Journal: Front Endocrinol (Lausanne) (2023)
Keywords: hnf1b, mody 5, chromosome 17q12 deletion syndrome, diabetic gastroparesis, maturity-onset diabetes of the young type 5
MeSH terms: Diabetes Mellitus, Type 2 / complications, Diabetes Mellitus, Type 2 / diagnosis, Diabetes Mellitus, Type 2 / genetics, Gastroparesis, Kidney Diseases, Cystic / genetics, Case Reports, Adult, Humans, Male, Syndrome, Mason-Type Diabetes

Background:
Maturity-onset diabetes of the young type 5 (MODY5) is an uncommon, underrecognized condition that can be encountered in several clinical contexts. It is challenging to diagnose because it is considered rare and therefore overlooked in the differential diagnosis. Moreover, no typical clinical features or routine laboratory tests can immediately inform the diagnosis.
Case presentation:
We report a 28-year-old man who was once misdiagnosed with type 1 diabetes due to decreased islet function and recurrent diabetic ketosis or ketoacidosis. However, he had intermittent nausea, vomiting, abdominal distension, and abdominal pain 6 months prior. Further examinations revealed agenesis of the dorsal pancreas, complex renal cyst, kidney stone, prostate cyst, hypomagnesaemia, and delayed gastric emptying. Accordingly, whole-exon gene detection was performed, and a heterozygous deletion mutation was identified at [GRCh37 (hg19)] chr17:34842526-36347106 (1.5 Mb, including HNF1B gene). The patient was eventually diagnosed with 17q12 deletion syndrome with gastroparesis.
Conclusion:
We report a novel case of diabetes mellitus type MODY5 as a feature of 17q12 deletion syndrome caused by a new 17q12 deletion mutation, which will further broaden the genetic mutation spectrum of this condition. With the help of gene detection technology, these findings can assist endocrinologists in making the correct diagnosis of MODY5 or 17q12 deletion syndrome. Additionally, they can formulate an appropriate therapy and conduct genetic screening counseling for their family members to guide and optimize fertility.
Hodgkin Lymphoma Involving the Brain: Report of a Rare Case
Authors: Muhammad Tahir, Osama Elkadi, Jacek Polski
Journal: Cureus (2024)
Keywords: anti-cd30, hodgkin lymphoma, hodgkin lymphoma involving the brain, hodgkin lymphoma of the brain, relapse lymphoma
MeSH terms: Case Reports

Hodgkin lymphoma (HL) is a systemic neoplastic process that mostly affects the lymph nodes of the cervical, supraclavicular, and mediastinal regions. The involvement of the central nervous system by HL is rare, and the diagnosis is typically determined based on distinct morphological and immunohistochemical analyses performed on tissue biopsy specimens. Intracranial HL is so scarce that any of the intracranial space-occupying pathology in a patient with established HL must be meticulously investigated to rule out a secondary disease process. Here, we report a unique case of intracranial HL in a 31-year-old African American male who was previously treated with anti-CD30 antibodies.
Case report: Anesthetic management for removal of tumor thrombus in the inferior vena cava and pulmonary artery in renal cell carcinoma
Authors: Suli Chen, Liangyuan Lu, Xiangli Zheng, Yanjun Lin, Liming Bao, Bao Zhang, Zhanmin Yang
Journal: Front Oncol (2024)
Keywords: renal cell carcinoma, anesthesia management, extracorporeal circulation, inferior vena cava tumor thrombus, pulmonary artery thrombus, tumor thrombus
MeSH terms: Case Reports

Anesthetic management of patients with renal cell carcinoma with tumor thrombus in the inferior vena cava (IVC) is challenging. This paper reports the experience of anesthesia management in a patient with advanced renal cell carcinoma with thrombus accumulation in the IVC, right atrium, and pulmonary artery who underwent radical nephrectomy and tumor thrombus removal assisted by cardiopulmonary bypass. The emboli, measuring approximately 3 × 6 cm in the left inferior pulmonary artery and 4 × 13 cm in the right main pulmonary artery, were removed completely. During incision of the IVC under systemic heparinization, significant blood loss occurred in the surgical field. The surgery took 724 min, and cardiopulmonary bypass took 396 min. Intraoperative blood loss was 22,000 ml. The patient was extubated 39 hours after surgery and stayed in intensive care unit for 3 days. At 1 year follow-up, the patient was in good health and leading a normal life.
From Rarity to Recognition: Infantile Botulism and the Broad Spectrum of Differential Diagnoses
Authors: Matthew C Authement, Brandon M Jones, Robert J Kahoud, Elizabeth H Ristagno
Journal: Case Rep Pediatr (2024)
MeSH terms: Case Reports

This case illustrates a 5-week-old girl who presented with decreased activity, decreased feeds, poor suck, weak cry, lethargy, hypotonia, and areflexia. The child was found to have infant botulism. The case demonstrates the importance of a full history and broad differential in an ill-appearing infant. The differential for an ill-appearing infant should always include infectious etiologies and may include metabolic disorders, congenital anomalies, nonaccidental trauma, neurologic disorders, and endocrine disorders. The broad differential diagnosis may make rapid diagnosis and treatment for infantile botulism a challenge.
Case report: Combination of veno-arterial extracorporeal membrane oxygenation and intra-aortic balloon pump in a young male patient with refractory cardiogenic shock due to aluminum phosphide poisoning
Authors: Oleg Dukhin, Danila Bala, Evgeny Felker, Polina Golovina, Mariya Tretyakova, Boris Haes, Polina Savvinova
Journal: Front Cardiovasc Med (2023)
Keywords: iabp, va-ecmo, aluminium phosphide, cardiogenic shock, mechanical circulatory support
MeSH terms: Case Reports

Background:
Acute toxic myocardial damage may be accompanied by the development of cardiogenic shock (CS), the mortality from which is still unacceptably high. Since there is no specific antidote for many types of toxins, treatment of such patients includes various measures of hemodynamic and respiratory support. The paper presents a case of refractory CS due to possible aluminum phosphide (AP) poisoning.
Case summary:
A 20-year-old man was admitted to the emergency department 4 days after home inhalation of AP due to complaints of nausea, vomiting, abdominal and chest pain. Over the next few hours, he rapidly developed CS, which was refractory to conservative treatment. Therefore, veno-arterial membrane oxygenation (VA-ECMO) was performed, during which hemodynamics stabilized, but later there were signs of left ventricular overload. To unload the left ventricle (LV), an intra-aortic balloon pump (IABP) was implanted, which significantly improved the patient's condition. After 6 days the patient was decannulated, and a few more days later IABP was discontinued. Subsequently, the patient was treated for sepsis due to bilateral pneumonia and acute respiratory distress syndrome and optimal medical therapy for heart failure was prescribed. The patient was discharged after 34th day of hospitalization.
Shear fractures of the capitellum in children: a case report and narrative review
Authors: Jean-Marc Pilotto, Silvia Valisena, Giacomo De Marco, Oscar Vazquez, Vincenzo De Rosa, Mario Mendoza Sagaon, Geraldo De Coulon, Romain Dayer, Dimitri Ceroni, Christina Steiger
Journal: Front Surg (2024)
Keywords: capitellum, fracture, osteosynthesis, pediatric, shear fracture
MeSH terms: Case Reports

The shear fractures of the capitellum are rare fractures in the pediatric population. Their diagnosis is challenging because of the high cartilaginous component of the growing elbow, requiring a high level of clinical suspicion especially in the case of small osteochondral or chondral fragments. The literature on capitellar shear fractures is mainly represented by case reports, which provides a patchy view of the topic. For this reason, we aimed to draw a narrative review presenting the available management strategies and their outcomes, and present two cases treated in our institution.
Discovering the sensory, emotional, and interactive experiences of a place
Authors: Luis Alfonso de la Fuente Suárez
Journal: Front Psychol (2024)
Keywords: architecture, emotions, environmental experience, place preference, sensory experience

This article proposes a data collection technique for describing experiences of a built environment. Besides the experiences of the visual and physical aspects of the place, this technique helps describe the sensory, bodily, emotional, interactive, and social experiences occurring during the human-environment encounter. The enabling technique presented is called Reactions and Actions Description Survey (RADES). It employs 120 images depicting people going through different situations involving all the senses, showing expressions related to positive and negative emotions, and realizing varied activities. Forty-five participants visited the esplanade in the exterior of a historic building called Obispado. The case study is located on a hill and is a scenic viewpoint of Monterrey, Mexico. The participants answered the RADES and the Environmental Description Survey (ENVIDES), which focuses on describing the qualities of the place and the appraisals with which it is experienced. The comments about the experiences of the place obtained through both surveys were grouped into 133 categories. Qualitative and quantitative data about the experiences of the place were obtained through both techniques. A quantitative analysis of the data was realized since the participants not only described their experiences with words but also indicated numerically the intensity of such experiences. Spearman correlations between the experiences were calculated, and a general map of the experiences of the place was created through multidimensional scaling analysis (MDS). The study revealed the connections between the elements and qualities of the site and the views with specific positive and negative experiences occurring during the visit. Furthermore, MDS allowed the discovery of 10 dimensions of environmental experience-pleasure/displeasure, high/low arousal, dominance/submissiveness, knowing/inhabiting, environment/self, higher/lower cognition, spatiality/materiality, states/processes, natural/built, and visual/sensory. The presented techniques and the findings obtained through them can assist architects in recognizing valuable environmental features for the design of livable spaces.
A case report of neonatal incontinentia pigmenti complicated by severe cerebrovascular lesions in one of the male monozygotic twins
Authors: Xiaofeng Lin, Wei Zhang, Ping Zhou
Journal: Front Pediatr (2024)
Keywords: ikbkg gene, cerebrovascular lesions, incontinentia pigmenti, monozygotic twins, neonatal
MeSH terms: Case Reports

Background:
This article reports a case of neonatal incontinentia pigmenti onset in only one male monozygotic twin with characteristic skin lesions after birth followed by severe cerebrovascular lesions.
Case presentation:
A male infant, the first of monozygotic twins, was born with multiple yellow pustules all over his body, repeated new herpes at different sites during the course of the disease, aggravated by fusion, warty crusts, and hyperpigmentation; biopsy pathology suggested eosinophilic spongiform edema of the skin. Peripheral blood eosinophils were significantly elevated, and brain magnetic resonance imaging revealed diffuse multiple cystic and lamellar abnormal signal areas in the left frontal and parietal lobes. On day 30, the infant showed neurological symptoms, such as poor response and apnea, and an emergency cranial computed tomography scan revealed abnormal changes in the left cerebral hemisphere and bilateral cerebellum. After admission, he was given a potassium permanganate bath and topical mupirocin for 1 month, and the skin abnormalities improved. He was treated with mechanical ventilation and vasoactive drugs for 2 days after the cerebrovascular accident, and died the same day after the parents chose hospice care. No deletion variants or point mutations were detected in subsequent genetic tests, and chromosomal copy number variation tests revealed different degrees of chimeric duplications and deletions in different regions of chromosomes Y and 3. The parents were healthy, and his twin brother had normal growth and development with no abnormalities at multiple follow-up visits.
Conclusion:
Neonatal incontinentia pigmenti in only one male monozygotic twin is extremely rare and the genetic diagnosis is challenging. Awareness of the combined cerebrovascular lesions needs to be enhanced, and potential prevention and treatment methods need to be explored to improve the prognosis.
Health damage assessment of commuters and staff in the metro system based on field monitoring-A case study of Nanjing
Authors: Shu Su, Shuhao Li, Yujie Ding, Peng Mao, Dan Chong
Journal: Front Public Health (2024)
Keywords: air pollution, case analysis, field monitoring, health damage assessment, metro station
MeSH terms: Air Pollutants / analysis, Air Pollution / analysis, Research Support, Non-U.S. Gov't, Environmental Monitoring, Humans, Particulate Matter / analysis, Transportation, Air Pollutants, Particulate Matter

Introduction:
The metro has emerged as a major mode of transportation. A significant number of commuters and staff in the metro system are exposed to air pollutants because of its shielded environment, and substantial health damage requires quantitative assessment. Previous studies have focused on comparing the health impacts among different transportation modes, overlooking the specific population characteristics and pollutant distribution in metro systems.
Methods:
To make improvements, this study implements field monitoring of the metro's air environment utilizing specialized instruments and develops a health damage assessment model. The model quantifies health damage of two main groups (commuters and staff) in metro systems at three different areas (station halls, platforms, and metro cabins) due to particulate matter 10 and benzene series pollution.
Conclusion:
A case study of Nanjing Metro Line 3 was conducted to demonstrate the applicability of the model. Health damage at different metro stations was analyzed, and the health damage of commuters and staff was assessed and compared. This study contributes to enhancing research on health damage in the metro systems by providing a reference for mitigation measures and guiding health subsidy policies.
Case Report: Cytodiagnosis of pigmented villonodular synovitis involving carpal bones of right wrist
Authors: Dr Shreya Giri Goswami, Dr Arvind Bhake, Dr Simran Khan, Dr Soumya Agrawal, Dr Jayashree Bhawani
Journal: F1000Res (2023)
Keywords: carpal bones, cytomorphology, fine needle aspiration cytology, multiosteotic involvement, pigmented villonodular synovitis
MeSH terms: Carpal Bones / diagnostic imaging, Carpal Bones / pathology, Synovitis, Pigmented Villonodular / diagnosis, Synovitis, Pigmented Villonodular / pathology, Case Reports, Adult, Biopsy, Fine-Needle, Cytodiagnosis / methods, Female, Humans, Male, Wrist / pathology

Background:
Pigmented Villonodular Synovitis (PVNS) is a rare disease of osteoskeletal tissue. Cytodiagnosis of PVNS on fine needle aspiration (FNA) smears is therefore rarely reported. The PVNS usually affects the larger joints. The involvement of the smaller joints and bones are uncommon.
Case presentation:
The reported case is one such rarity wherein the diagnosis of PVNS was carried out on the FNAC. The case showed the involvement of all carpal bones except for the pisiform. The 2 nd- 5 th metacarpal bases were also found to be involved in the disease process. The presence of sheets of synoviocytes with brown altered hue to the cytoplasm along with multinucleate giant cells and pigmented macrophages were characteristically present in the smears of FNA. The diagnosis was confirmed on the tissue biopsy. The present case is reported for its unusual multiosteotic involvement of wrist joint bones and the metacarpal bones simultaneously with radiological evidence. The cytomorphology of the lesion in the present case were noteworthy as a learning experience in reporting of PVNS of wrist joint on FNA smears.
Measure what matters: considerations for outcome measurement of care coordination for children with neurodevelopmental disabilities and medical complexity
Authors: Dércia Materula, Genevieve Currie, Xiao Yang Jia, Brittany Finlay, Catherine Richard, Meridith Yohemas, Gina Lachuk, Myka Estes, Tammie Dewan, Sarah MacEachern, Nadine Gall, Ben Gibbard, Jennifer D Zwicker
Journal: Front Public Health (2023)
Keywords: care coordination, caregivers, children, medical complexity, neurodevelopmental disorders, quality of life, resource utilization
MeSH terms: Child Health Services, Quality of Life, Research Support, Non-U.S. Gov't, Canada, Child, Consensus, Humans, Outcome Assessment, Health Care

Introduction:
Care Coordination (CC) is a significant intervention to enhance family's capacity in caring for children with neurodevelopmental disability and medical complexity (NDD-MC). CC assists with integration of medical and behavioral care and services, partnerships with medical and community-based supports, and access to medical, behavioral, and educational supports and services. Although there is some consensus on the principles that characterize optimal CC for children with NDD-MC, challenges remain in measuring and quantifying the impacts of CC related to these principles. Two key challenges include: (1) identification of measures that capture CC impacts from the medical system, care provider, and family perspectives; and (2) recognition of the important community context outside of a hospital or clinical setting.
Methods:
This study used a multilevel model variant of the triangulation mixed methods design to assess the impact of a CC project implemented in Alberta, Canada, on family quality of life, resource use, and care integration at the broader environmental and household levels. At the broader environmental level, we used linked administrative data. At the household level we used quantitative pre-post survey datasets, and aggregate findings from qualitative interviews to measure group-level impacts and an embedded multiple-case design to draw comparisons, capture the nuances of children with NDD-MC and their families, and expand on factors driving the high variability in outcome measures. Three theoretical propositions formed the basis of the analytical strategy for our case study evidence to explore factors affecting the high variability in outcome measures.
Discussion:
This study expanded on the factors used to measure the outcomes of CC and adds to our understanding of how CC as an intervention impacts resource use, quality of life, and care integration of children with NDD-MC and their families. Given the heterogeneous nature of this population, evaluation studies that account for the variable and multi-level impacts of CC interventions are critical to inform practice, implementation, and policy of CC for children with NDD-MC.