Non-Cirrhotic Portal Hypertension (NCPH) is a rare cause of hematemesis and melena. Like in cirrhotic patient, hematemesis in NCPH patient was caused by rupture of esophageal varices. But unlike in cirrhotic patient, in NCPH there are no sign of liver failure, because liver physiology is still normal. We reported case of male patient with NCPH that had hematemesis because of rupture of esophageal varices.
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J Med Case Rep
January 2025
Department of Hepatic Biliary Pancreatic Medicine, First Hospital of Jilin University, 1 Xinmin Avenue, Changchun, 130021, China.
Background: Dyskeratosis congenita is a rare genetic disease due to telomere biology disorder and characterized by heterogeneous clinical manifestations and severe complications. "Porto-sinusoidal vascular disease" has been recently proposed, according to new diagnostic criteria, to replace the term "idiopathic non-cirrhotic portal hypertension." TERT plays an important role in telomeric DNA repair and replication.
View Article and Find Full Text PDFTrends Cardiovasc Med
January 2025
Department of Cardiology, Euroclinic Hospital, Athens, Greece; First Department of Cardiology, Athens University School of Medicine, Athens, Greece. Electronic address:
Metabolic dysfunction-associated steatotic liver disease (MASLD), previously termed nonalcoholic fatty-liver disease, is an important and rising health issue with a link with atherosclerotic cardiovascular (CV) disease (CVD), affecting ∼25-30% of the adults in the general population; in patients with diabetes, its prevalence culminates to ∼70%; its evolutive form, nonalcoholic steatohepatitis, is estimated to be the main cause of liver transplantation in the future. MASLD is a multisystem disease that affects, besides the liver, extra-hepatic organs and regulatory pathways; it raises the risk of type 2 diabetes mellitus (T2D), CVD, and chronic kidney disease; the disease may also progress to hepatocellular carcinoma. Its diagnosis requires hepatic steatosis and at least one cardiometabolic risk factor and the exclusion of both significant alcohol consumption and other competing causes of chronic liver disease.
View Article and Find Full Text PDFCureus
December 2024
Department of Clinical Immunology and Allergology, Iuliu Hatieganu University of Medicine and Pharmacy of Cluj, Cluj-Napoca, ROU.
Macrotrabecular-massive hepatocellular carcinoma (MTM-HCC) is a rare and aggressive molecular subtype of hepatocellular carcinoma (HCC) associated with a poor prognosis. Unlike typical HCC, which commonly arises in the context of cirrhosis, MTM-HCC can develop in non-cirrhotic livers, presenting unique diagnostic and therapeutic challenges. This case report describes a 35-year-old male who presented with persistent epigastric pain, fatigue, and loss of appetite.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Rheumatology and Immunology, the Second Affiliated Hospital of Xiamen Medical College, Xiamen, China.
Introduction: Systemic lupus erythematosus (SLE) complicated by thrombotic microangiopathy (TMA) and non-cirrhotic portal hypertension (NCPH) is rare. We present a case of a female patient with SLE who developed TMA and NCPH and responded positively to rituximab and plasma exchange treatment.
Case Description: A 53-year-old woman was admitted with 6 h of confusion.
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.
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