OpenMed/multicare-articles huggingface dataset:
Juvenile dermatomyositis with central nervous system involvement: two case reports from a retrospective single-center cohort, with literature review
Authors: Ling Yang, Wanzhen Guan, Haimei Liu, Yifan Li, Yinv Gong, Qianying Lv, Qiaoqian Zeng, Qijiao Wei, Xiaomei Zhang, Weiming Chen, Chao Chen, Li Sun
Journal: Front Pediatr (2024)
Keywords: anti-nxp2 antibody, cutaneous vasculitis, juvenile dermatomyositis, macrophage activation syndrome, outcomes, plasma exchange, seizure

Background:
Juvenile dermatomyositis (JDM) is a systemic autoimmune disease primarily involving the muscles and skin; it can also affect the central nervous system (CNS). The relevant literature provides limited information regarding the characteristics of JDM with CNS involvement.
Method:
We reviewed patients with JDM who were hospitalized at our center between January 2016 and August 2023, with a focus on those with CNS involvement. The aim was to provide detailed case reports on these patients, and to summarize the relevant literature about the characteristics of similar cases.
Results:
Among 193 hospitalized patients with JDM, two (1.03%) had CNS involvement. Two patients, a 5.5-year-old girl and an 11-year-old boy, were admitted with severe proximal muscle weakness and seizures, and presented with active cutaneous vasculitis. Both were ultimately diagnosed with JDM, with CNS involvement. Both patients had confirmed presence of anti-NXP2 antibody through myositis-specific antibody analysis. Additionally, they all exhibited hyperferritinemia and thrombocytopenia. Salvage therapies like intravenous methylprednisolone (IVMP) pulse therapy and/or plasma exchange were administered successfully. At final follow-up, both patients had achieved complete clinical response and full neurological recovery. Our literature review identified nine similar case studies. CNS involvement usually occurred within the first 10 months of the disease course, and most of these patients had fatal outcomes, with a mortality rate of 66.6% (6/9). Including the two patients described herein, the median age for disease onset is 10.5 years (range 4-17 years), and the male: female ratio is 6:5. Seizures are the most common neurological symptom, accompanied by active cutaneous vasculitis. The brain biopsies showed two distinct pathological presentations: one was central nervous system vasculitis, and the other was cerebral macrophage activation syndrome.
Conclusions:
CNS involvement is a rare but life-threatening JDM complication. Herein, our cases and the literature indicate that it typically occurs within the first 10 months of the disease course and manifests as seizures, often accompanied by active cutaneous vasculitis, with fatal outcomes. Timely implementation of salvage therapies, like IVMP pulse therapy and plasma exchange, may significantly impact patient outcomes.
Case report: An uncommon presentation of extramedullary plasmacytoma without a concurrent diagnosis of multiple myeloma
Authors: Katarzyna Stawarz, Adam Galazka, Anna Gorzelnik, Monika Durzynska, Karolina Bienkowska-Pluta, Jakub Zwolinski
Journal: Front Oncol (2024)
Keywords: case report, extramedullary plasmacytoma, multiple myeloma, radiotherapy, surgery
MeSH terms: Case Reports

Introduction:
Extramedullary plasmacytoma (EMP) is an uncommon solitary tumor originating from neoplastic plasma cells located outside the bone marrow. Despite its rarity, the occurrence of EMP without a concurrent diagnosis of multiple myeloma (MM) is considered extremely rare. Approximately 80-90% of EMP cases are found in the head and neck region, with a higher incidence in men aged between 50 and 60 years. The current treatment modalities include radiotherapy (RT) as a first-line approach, with surgery or chemotherapy regarded as other therapeutic options. While RT proves effective in the majority of EMP cases, there are instances where the tumor remains refractory to radiation. In this case report, we present an unusual scenario of EMP resistant to RT without concurrent signs of multiple myeloma which was successfully treated with surgery followed by systemic therapy.
Case report:
A 72-year-old male was admitted to the Head and Neck Cancer Clinic with a 6-month history of swallowing difficulties. He denied experiencing weight loss or pain on swallowing. Basic laboratory tests yielded results within normal limits, except for beta-2 microglobulin. Physical examination revealed an enlarged submandibular lymph node on the right side. Fiberoptic examination identified a soft tissue polypoid mass within the right piriform fossa, slightly protruding into the vocal slit. A CT scan displayed a well-circumscribed 2 cm polypoid, homogeneously enhancing soft tissue mass adjacent to the posterior surface of the epiglottis and the right side of the tongue base. Bone marrow biopsy revealed no abnormalities, and there were no clinical or laboratory signs of multiple myeloma. Based on the tumor biopsy results and imaging studies, a diagnosis of EMP was made. Due to the lack of response to RT, surgical removal of the tumor was pursued, followed by systemic therapy. Ultimately, the patient achieved full recovery with effective disease control.
Conclusion:
In conclusion, EMP without concurrent multiple myeloma is an exceedingly rare condition that demands a multidisciplinary approach for both diagnosis and treatment. Moreover, although RT continues to be the primary standard treatment for EMP, in some cases other therapeutic regimens prove to be successful.
Determinants of primary healthcare providers' readiness for integration of ART services at departmental levels: A case study of Lira City and District, Uganda
Authors: Emmanuel Asher Ikwara, Lakeri Nakero, Maxson Kenneth Anyolitho, Rogers Isabirye, Syliviah Namutebi, Godfrey Mwesiga, Sean Steven Puleh
Journal: PLoS One (2023)
MeSH terms: Delivery of Health Care, HIV Infections / drug therapy, Research Support, N.I.H., Extramural, Research Support, U.S. Gov't, Non-P.H.S., Anti-Retroviral Agents / therapeutic use, Cross-Sectional Studies, Health Personnel, Humans, Uganda, Anti-Retroviral Agents

Background:
Decreasing or flattening funding for vertical HIV services means that new and innovative ways of providing care are necessary. This study aimed to assess the determinants of readiness for integration of Antiretroviral Therapy (ART) services at the departmental level among primary health care providers (PHCP) at selected health facilities in Lira District.
Methods:
A cross-sectional survey employing mixed methods approaches was conducted between January and February 2022 among 340 primary healthcare practitioners (PHCP) at selected health facilities in Lira district. An interviewer-administered questionnaire was used to collect quantitative data. Quantitative data was analyzed using Stata version 15. and presented as proportions, means, percentages, frequencies, and odds ratios. Logistic regression was used to determine associations of the factors with readiness for ART integration at a 95% level of significance. Thematic analysis was used to analyze qualitative data.
Results:
The majority 75.2% (95% CI; 0.703-0.795) of the respondents reported being ready for the integration of ART services. PHCPs who were aware of the integration of services and those who had worked in the same facility for at least 6 years had higher odds of readiness for integration of ART, compared with their counterparts [aOR = 7.36; 95% CI = 3.857-14.028, p-value <0.001] for knowledge and duration at the current facility [aOR = 2.92; 95% CI = 1.293-6.599, p-value < 0.05] respectively. From the qualitative data, the dominant view was that integration is a good thing that should be implemented immediately. However, several challenges were noted, key among which include limited staffing and drug supplies at the facilities, coupled with limited space.
Conclusions:
The study reveals a high level of readiness for the integration of ART services at departmental levels among Primary Healthcare Providers. Notably, PHCPs knowledgeable about integration and those who spent at least six years at the current health facility of work, were strong determinants for the integration of ART services in resource limited settings. In light of these findings, we recommend that policymakers prioritize the implementation of training programs aimed at upskilling healthcare workers. Furthermore, we advocate that a cluster randomized controlled trial be conducted, to evaluate the long-term effects of this integration on overall health outcomes.
The known and unknown of post-pump chorea: a case report on robust steroid responsiveness implicating occult neuroinflammation
Authors: Muhammad Iqbal, Muizz Zaman, Niranjan Ojha, Yung-Tian A Gau, Eufrosina I Young
Journal: Front Immunol (2024)
Keywords: bioprosthetic aortic valve, cardiopulmonary bypass (cpb), chorea (non-huntington’s), hypoxia inducible factor, neuroinflammation, post-pump chorea
MeSH terms: Cardiopulmonary Bypass / adverse effects, Chorea / drug therapy, Chorea / etiology, Neuroinflammatory Diseases / etiology, Neuroinflammatory Diseases / immunology, Case Reports, Aortic Valve / pathology, Aortic Valve / surgery, Female, Heart Valve Prosthesis Implantation / adverse effects, Humans, Male, Middle Aged, Postoperative Complications / etiology

Post-pump chorea (PPC) is characterized by the development of choreiform movements following cardiopulmonary bypass (CPB) surgery. PPC occurs almost exclusively in children, and its pathophysiology remains unclear. Here we present an adult case of PPC after bovine aortic valve replacement (AVR) which exhibited dramatic and reproducible response to steroid, suggesting the presence of occult neuroinflammation. This observation suggests a novel underlying mechanism in certain subgroups of PPC, which is likely a heterogeneous condition to start with. Further research into the pathomechanisms of PPC could offer insights into managing this otherwise symptomatic control-only condition.
VExUS to Guide Ultrafiltration in Hemodialysis: Exploring a Novel Dimension of Point of Care Ultrasound
Authors: Abhilash Koratala, Mohamed Ibrahim, Sirisha Gudlawar
Journal: POCUS J (2024)
Keywords: congestion, dialysis, pocus, vexus
MeSH terms: Case Reports

Venous Excess Ultrasound (VExUS) is a valuable bedside tool for nephrologists within a multi-organ point of care ultrasound (POCUS) framework. VExUS can address limitations of conventional physical examination in identifying hemodynamic congestion and monitoring treatment efficacy. A 53-year-old man with heart failure and end-stage kidney disease on hemodialysis presented with elevated liver function tests. Despite an unremarkable right upper quadrant ultrasound done by radiology, the review of images by the nephrology team uncovered severe venous congestion, evidenced by a dilated inferior vena cava (IVC) and abnormal hepatic and portal vein flow. Follow-up assessments included VExUS scans and daily ultrafiltration that resulted in a notable 8-liter fluid removal. The dynamic changes in IVC shape and improvement in Doppler waveforms underscored successful decongestion. This case demonstrates the clinical utility of VExUS in guiding therapy for fluid overload in complex patients.
Case report: Uniportal video-assisted thoracoscopic sleeve lobectomy in a 6-year-old patient with inflammatory myofibroblastic tumor (IMT)
Authors: Zhen-Yang Geng, Zi-Hao Li, Shi-Hao Li, Bin Wu, Yin-Liang Sheng, Ping Yuan, Feng Li, Yu Qi
Journal: Front Pediatr (2023)
Keywords: inflammatory myofibroblastic tumor, pediatric patients, sleeved lobectomy, thoracic surgery, uniportal thoracoscopic
MeSH terms: Case Reports

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm that can occur in various organs, including the lung. Surgical resection is usually the preferred treatment for localized IMT.A 6-year-old female was admitted to our hospital with complaints of "coughing and vomiting for 6 days". A chest CT scan revealed occlusion of the left main bronchus, segmental atelectasis of the left lower lung, and cystic low-density shadows along the bronchial pathway. Subsequent fiberoptic bronchoscopy confirmed the diagnosis of IMT through pathological biopsy. After excluding surgical contraindications, the patient underwent uniportal video-assisted thoracoscopic sleeve lobectomy for treatment. The patient had an uneventful postoperative course and was discharged four days after surgery. After one month, the patient received a follow-up examination and reported no significant discomfort. A chest CT scan revealed no postoperative complications.Our experience suggests that uniportal video-assisted thoracoscopic surgery may be a safe and effective approach for the treatment of pediatric patients with IMT requiring complex surgical procedures such as sleeve lobectomy and tracheoplasty.
Case report: Serious unexpected vascular events in two patients with lymphocytic variant hypereosinophilic syndrome
Authors: Nathan Torcida, Giulia Casalino, Antoine Bondue, Lise Jodaitis, Frederic Vanden Eynden, Florence Roufosse
Journal: Front Cardiovasc Med (2023)
Keywords: arterial dissection, coronary aneurism, hypereosinophilc syndrome, lymphocytic variant hypereosinophilic syndrome, stroke, valsalva sinus aneurysm
MeSH terms: Case Reports

Background:
Lymphocytic-variant hypereosinophilic syndrome (L-HES) is a form of reactive hypereosinophilia, most commonly associated with interleukin-5 over-production by clonal, most commonly CD3-CD4+CD2hiCD5hiCD45RO+ T-cells. Patients often present with predominant cutaneous and soft-tissue manifestations, while cardiovascular involvement is uncommon.
Methods:
We reviewed the medical files of two L-HES patients followed in our center who developed serious vascular complications and performed a literature review for similar cases.
Results:
Patient 1, a 52-year-old female, presented with an ischemic stroke secondary to left middle cerebral artery dissection after 10 years of indolent L-HES. Blood eosinophilia was controlled with oral corticosteroids (OCS), but OCS-tapering attempts with hydroxyurea and pegylated interferon failed, prompting the introduction of mepolizumab with rapid normalization. Patient 2, a 62-year-old female, had been asymptomatic for 10 years without treatment when a NSTEMI occurred, due to coronary artery occlusion secondary to a large cauliflower-aneurysm of the proximal aorta and aneurysmal dilatation of several coronary arteries, requiring semi-urgent surgical management. Aortic wall staining for eosinophil major basic protein showed eosinophils in the adventitia. Blood eosinophilia was controlled with OCS.
Conclusions:
Patients with apparently clinically benign L-HES may develop arterial complications, consisting in dissection and/or aneurysm dilatation of medium-to-large vessels with serious consequences. The value of performing regular vascular imaging and monitoring during follow-up has yet to be determined.
Alzheimer's Type Neuropathological Changes in a Patient with Depression and Anxiety: A Case Report and Literature Review of Neuropathological Correlates of Neuropsychiatric Symptoms in Alzheimer's Disease
Authors: Sumit Das
Journal: Case Rep Neurol Med (2023)
MeSH terms: Case Reports

Alzheimer's disease (AD) is classified as a tauopathy and is the most common neuropathological correlate of dementia/cognitive impairment. AD is neuropathologically characterized by the presence of beta-amyloid immunoreactive senile plaques and tau positive neurofibrillary tangles. Neuropsychiatric symptoms of AD however continue to be underscored, and therefore, neuropathological correlates of these neuropsychiatric symptoms are not readily studied. Presented here is a case of 60-year-old female who initially presented with anxiety and depression, and continued to be the predominant symptoms although mild cognitive impairment was noted as per the available clinical notes. Postmortem examination of the brain revealed severe Alzheimer's type neuropathological changes, which included significant tau and beta-amyloid pathology in limbic regions, which were thought to represent correlates of the patient's depression and anxiety. This case report illustrates the possible neuropathological correlates of neuropsychiatric symptoms in patients with AD. The author hopes that such a case will promote more in-depth studies into the pathophysiology of neuropsychiatric manifestations in AD.
Temporomandibular Joint Ankylosis as a Sequel of an Overlooked Condylar Fracture in a Child
Authors: Manel Gharbi, Rym Kammoun, Imen Chaabani, Touhami Ben Alaya
Journal: Case Rep Dent (2024)
MeSH terms: Case Reports

Temporomandibular joint ankylosis is an important entity that dentists and maxillofacial surgeons should know about. It clinically manifests through a permanent limitation of mandibular movements coupled with mouth opening inferior to 3 cm. This serious pathology can have serious functional repercussions, such as mastication problems, speech troubles, eating disorders, and jaw growth hindrance, in addition to the psychological difficulties in coping with such a condition in daily life. Herein, we present a radiological and chronological illustration of the evolution of temporomandibular joint ankylosis following an overlooked traumatic fracture of the mandibular condyle. The present case report involves an 8-year-old patient referred for a gradually evolving mouth opening limitation following a car accident. Tomodensitometry was helpful as it revealed an osseous block between the left temporomandibular joint surfaces, showing an ankylosis. Posttraumatic cerebral computed tomography scan was performed. It revealed an undetected fracture of the left condyle. The aim of this paper was to show how a traumatic ankylosis could have been avoided if enough attention was paid to the interpretation of immediate posttraumatic computed tomography scans. A thorough dental examination must be carried out once vital emergency is over. Early diagnosis of temporomandibular joint trauma is a crucial factor in preventing complications, such as ankylosis and its consequent oral dysfunctions. The dentist must automatically suspect condylar fracture when a child presents a history of head trauma, especially a mandibular trauma. This case should be a reminder that although temporomandibular joints are very often left out in patients' vital emergency first examination, temporomandibular joints/they are still a highly important structure which omission, and thus, dysfunction, if lesions are present, can lead to nonnegligible medico-legal consequences/that temporomandibular joints should be taken into account during patients' vital emergency first examination because if they are neglected, in the presence of lesions, they cause dysfunction, thus leading to nonnegligible medico-legal consequences.
Curious Case of a Giant Retroperitoneal Cyst
Authors: Viral Surani, Sanjay Chatterjee, Sunila Jaggi, Niranjan Banka, Shivani Desai, Gajanan Rodge
Journal: Cureus (2024)
Keywords: adrenal cyst, giant cyst, retroperitoneal cyst, serous fluid, uniloculated cyst
MeSH terms: Case Reports

Adrenal cysts are uncommon fluid-filled masses that develop in the adrenal gland. Typically, they are non-functional, asymptomatic, and smaller than 10 cm in diameter when incidentally detected. However, the presence of giant adrenal cysts, exceeding 10 cm in diameter, creates a diagnostic challenge due to the difficulty in determining their origin. Surgical intervention is advised when the cyst surpasses 10 cm in diameter, produces symptoms, causes endocrine abnormalities, exhibits intracystic bleeding, or raises suspicion of malignancy. The preferred treatment approach involves adrenalectomy, performed either through open surgery or laparoscopy. In cases where the diagnosis is unequivocal, ultrasound-guided percutaneous drainage serves as an alternative. Here, we present an exceptional case of a massive retroperitoneal mass caused by a rare giant adrenal cyst.
Case Report: Multiple gastrointestinal stromal tumors along with numerous cutaneous neurofibromas: a case description and literature analysis
Authors: Congcan Zhao, Liquan Jin, Yunbo Tan, Yiming Chen, Ziting Su, Wenwu Li, Qing Yang
Journal: Front Oncol (2023)
Keywords: gastrointestinal stromal tumors, jejunum, neurofibroma, neurofibromatosis type 1, skin
MeSH terms: Case Reports

Multiple gastrointestinal stromal tumors (GISTs) combined with cutaneous multiple neurofibromas are clinically rare. This paper presents a case of multiple gastrointestinal stromal tumors in the jejunum of a 68-year-old mother, along with her daughter who also had coexisting cutaneous multiple neurofibromas. The mother had been experiencing repeated melena for over 2 years and had previously been diagnosed with multiple small intestinal masses at other hospitals. Additionally, her 42-year-old daughter was admitted to our department due to recurrent abdominal pain caused by cholecystolithiasis. The mother and daughter both exhibited multiple nodular masses of varying sizes on their skin, including the truncus, limbs, and face, which were diagnosed as neurofibromas. The mother underwent a partial excision of the jejunum and a lateral jejunojejunal anastomosis side-to-side, as well as excision of skin lesions in our department. The final diagnosis of wild-type GISTs associated with neurofibromatosis type 1 (NF1) was confirmed through postoperative pathology, immunohistochemistry, and genetic testing results. During preoperative gastrointestinal endoscopy and intraoperative laparoscopic exploration of the gastrointestinal tract, no obvious tumors were found in her daughter. A combination of patient observations and a review of relevant literature in the field suggests that when patients present with gastrointestinal symptoms and multiple irregular painless swellings in the skin, it is important to consider the possibility of an association with NF1 and GIST. Additionally, obtaining a detailed family history can save time and improve the diagnosis of patients with both NF1 and GIST. We recommend that even if there are no gastrointestinal manifestations of GISTs in the offspring of newly mutated NF1 patients, regular review of gastroenteroscopy, imaging examination, and long-term follow-up after middle age are still crucial for the early diagnosis and treatment of NF1-related GISTs.
Labor exploitation in the Italian agricultural sector: the case of vulnerable migrants in Tuscany
Authors: Caterina Francesca Guidi, Fabio Berti
Journal: Front Sociol (2023)
Keywords: agriculture, asylum seekers, labour exploitation, migrants, vulnerability

Labor exploitation of agricultural migrant workers is a well-documented phenomenon by investigations and field research in several Italian regions, both in the North and the South. Despite the agri-food excellencies of the "Made in Italy" brand being a source of pride for Italian entrepreneurship, including the viticulture sector, evidence shows that many of these products are the result of different levels of illegal recruitment and labor exploitation. In this article, the authors analyze the impact of recent waves of vulnerable migrants entering the Italian labor market and present the results of a qualitative field research, conducted in Tuscany between 2021 and 2022. Through 60 interviews with exploited migrant workers and 40 interviews with relevant stakeholders, the authors focus on the recruitment process of vulnerable migrants into the agriculture sector and the labor conditions granted to them regardless of their particular migratory status. The article concludes with the analysis of the peculiarities of the Tuscan case study, characterized by the presence of a legal system of labor exploitation.