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Hepatic encephalopathy in non-cirrhotic portal hypertension.
Metab Brain Dis
January 2025
Institute of Liver and Biliary Sciences, New Delhi, India.
Hepatic encephalopathy (HE) is traditionally associated with hepatic parenchymal diseases, such as acute liver failure and cirrhosis. Its prevalence in non-cirrhotic portal hypertension (NCPH) patients, extrahepatic portal vein obstruction (EHPVO), and non-cirrhotic portal fibrosis (NCPF) is less well described. HE in NCPH allows one to study the effect of portosystemic shunting and ammonia without significant hepatic parenchymal injury.
View Article and Find Full Text PDFCommon Variable Immunodeficiency Associated With Noninfectious Pulmonary Complications and Its Treatment: Beyond Immunoglobulin Therapy.
Pulm Circ
January 2025
Division of Pulmonary Medicine, Henry Ford Hospital Detroit Michigan USA.
Common variable immunodeficiency (CVID) is a type of primary immunodeficiency that presents as a heterogenous disorder characterized by hypogammaglobinemia, poor response to vaccines, recurrent sinopulmonary infections, and can have noninfectious systemic manifestations. We performed a single-center, retrospective, observational study of five patients with noninfectious complications of CVID. All patients had CVID as defined by the European Society of Immunodeficiencies criteria and had received intravenous immunoglobulin therapy.
View Article and Find Full Text PDFIdiopathic 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.
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 PDFPorto-Sinusoidal Vascular Disease: A New Nomenclature Different from Idiopathic Non-Cirrhotic Portal Hypertension.
Diagnostics (Basel)
September 2024
Department of Hepatology, Tianjin Second People's Hospital, Tianjin 300192, China.
Background And Aims: Porto-sinusoidal vascular disease (PSVD) as a novel clinical conception was modified on the basis of idiopathic non-cirrhotic portal hypertension (INCPH). This study aimed to compare the clinical, biochemical histological features and prognosis between the diagnostic criteria for PSVD and that of INCPH.
Methods: A total of 65 patients who underwent liver biopsies were analyzed retrospectively.
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