A 43-year old male patient with hyponatremic hypertensive syndrome was diagnosed as catastrophic primary antiphospholipid syndrome (PAPS). He subsequently developed hepatosplenomegaly. The patient also carried thrombophilia- and haemochromatosis-associated gene mutations. Further investigations upon persistence of splenomegaly indicated development of idiopathic portal hypertension.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Idiopathic non-cirrhotic portal hypertension: A case report.
Medicine (Baltimore)
December 2024
Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China.
Rationale: Idiopathic noncirrhotic portal hypertension (INCPH) is a rare liver disorder with elevated portal pressure without cirrhosis, making diagnosis challenging. This case report presents a 46-year-old Chinese male with INCPH, highlighting the crucial role of liver biopsy.
Patient Concerns: A 46-year-old male presented with persistent fatigue that lasted for 2 months and significantly worsened over the last 3 days.
A rare case of idiopathic portal hypertension with portopulmonary hypertension occurred following splenectomy with a change in portal hemodynamics.
Clin J Gastroenterol
December 2024
Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, 514-8507, Japan.
Article Synopsis
- * She had a splenectomy to address her thrombocytopenia caused by an enlarged spleen (hypersplenism), but nine months later, she experienced exertional dyspnea and was diagnosed with portopulmonary hypertension (PoPH).
- * Treatment with the medication macitentan improved her symptoms and right heart pressure readings, marking a rare case of PoPH arising after splenectomy.
Poor long-term outcome in patients with porto-sinusoidal vascular disease (PSVD): fact or disease misclassification?
J Hepatol
November 2024
Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas). Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver). Departament de Medicina i Ciències de la Salut. Universitat de Barcelona. Electronic address:
Racial Disparities in Inpatient Hospital Outcomes of Primary Sclerosing Cholangitis in United States: Nationwide Analysis.
Diagnostics (Basel)
November 2024
Parkview Cancer Institute, Advanced Interventional Endoscopy & Endoscopic Oncology (IOSE) Division, GI Oncology Program, 11104 Parkview Circle, Suite 310, Fort Wayne, IN 46845, USA.
Article Synopsis
- Primary sclerosing cholangitis (PSC) is a liver disease linked to inflammatory bowel disease and can cause serious liver complications; this study investigates trends in healthcare usage and mortality related to PSC in the U.S. from 2008 to 2017.
- The analysis of the Nationwide Inpatient Sample reveals that while hospital charges for PSC patients rose by 32.2%, the average length of hospital stays decreased during the study period, indicating changes in healthcare practices.
- Additionally, there was a notable increase in the severity of illness and risk of death among PSC patients, with a rise in cases of hepatocellular carcinoma (HCC), while cases of cholangiocarcinoma (CCA) saw a decline.
Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management.
Hepatol Int
December 2024
Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!