What is non-cirrhotic portal fibrosis?
Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by ‘obliterative portovenopathy’ leading to PHT, massive splenomegaly and well-tolerated episodes of variceal bleeding in young adults from low socioeconomic backgrounds, having near normal hepatic functions.
Is portal hypertension rare?
Introduction. Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized by of intrahepatic portal hypertension in the absence of cirrhosis, other causes of liver disease and splanchnic venous thrombosis [1–7].
What is Hepatoportal sclerosis?
Abstract. Background: Hepatoportal sclerosis (HPS) is a cause of noncirrhotic portal hypertension, with patients typically presenting with variceal bleeding. It is idiopathic in nature but is felt to be due to an abnormality of the intrahepatic vasculature.
Can portal hypertension occur without cirrhosis?
Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis.
What makes up hepatic portal system?
The hepatic portal vein is formed by the confluence of three main vessels, the gastric, pancreaticomesenteric, and lienomesenteric veins. They unite to form the hepatic portal vein near the anterior tip of the dorsal lobe of the pancreas.
Should hepatoportal sclerosis be retired and replaced with portal vein stenosis?
It has recently been suggested that the term hepatoportal sclerosis be retired and replaced with portal vein stenosis and herniated portal vein depending on the particular histologic findings ( Histopathology 2019;74:219 )
What is hepatoportal sclerosis in children?
Abstract Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS.
Should hepatoportal sclerosis be considered as the initial diagnosis for portal hypertension?
In conclusion, for pediatric age group with hepatoportal sclerosis findings in liver biopsy, who have not fulfilled the criteria for INCPH yet, it may be necessary to consider HPS as the initial diagnosis and once portal hypertension develops, INCPH diagnosis can be made.
Is there a case series for hepatocellular insufficiency (HPS)?
Isabel Fiel et al. (14) have reported a case series, consisting of 8 adult patients with HPS requiring liver transplantation. None of them was cirrhotic and 2 of them had portal vein thrombosis. Seven patients in this cohort had variceal bleeding as presenting symptom.
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