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Fibrillary glomerulonephritis (FGN) is a rare glomerular disease characterized by the deposition of fibrils within the mesangium and glomerular basement membrane. Most cases are idiopathic, but it can be linked to autoimmune diseases, neoplasms, and infections. There is limited evidence on the best treatment approach, and many patients progress to end-stage kidney disease.

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Clinical features and outcomes of total pancreatic lipomatosis with chronic pancreatitis: a case series.

Ann Gastroenterol

December 2024

Department of Gastrointestinal Surgery, Hepato-Pancreatico-Biliary and Liver Transplantation (Rajesh Gupta), Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Background: Fatty changes in the pancreas are common, whereas total pancreatic lipomatosis (PL) is rare. Commonly associated with various components of metabolic syndrome and metabolic-associated steatotic liver disease, total PL can have various etiologies and can manifest with severe pancreatic exocrine insufficiency.

Method: We retrospectively analysed the clinical profile and management outcomes of 8 patients (mean age: 37.

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Rationale: Triple gallbladder is a rare congenital anatomical abnormality because of the incomplete regression of rudimentary bile ducts and is often not found until it is accidentally detected during imaging research.

Patient Concerns: We report a rare case of triple gallbladder malformation and review the English literature on biliary tract variation caused by gallbladder malformation. The diagnosis, treatment, and postoperative situation of the patients were summarized and analyzed.

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Objective: To explore the clinical characteristics and risk factors for adverse outcomes in patients with Sjögren's Syndrome-associated pulmonary arterial hypertension (SS-PAH).

Methods: A retrospective analysis was conducted on SS-PAH patients diagnosed by right heart catheterization (RHC) between March 2013 and March 2024 across four Chinese medical centers. Patients were categorized into primary SS-PAH (pSS-PAH) and overlap SS-PAH, based on the presence of additional autoimmune diseases.

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Current approach to diagnosis and management of low-phospholipid associated cholelithiasis syndrome.

Curr Opin Gastroenterol

January 2025

Reference Center for Inflammatory Biliary Diseases and Autoimmune Hepatitis, European Reference Network on Hepatological Diseases (ERN Rare-Liver), Saint-Antoine Hospital, Assistance Publique - Hôpitaux de Paris; Sorbonne University, INSERM, Saint-Antoine Research Center (CRSA).

Purpose Of Review: Low phospholipid-associated cholelithiasis (LPAC) syndrome is a rare genetic form of intrahepatic cholesterol lithiasis, affecting mainly young adults. This review describes the recent advances in genetic and clinical characterization, diagnosis and management of LPAC syndrome.

Recent Findings: Recent publications report data from several retrospective cohorts.

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