Features Of Non-cirrhotic Portal Hypertension Research Articles

Clinical features of non-cirrhotic portal hypertension in patients with common variable immunodeficiency

We wished to summarize the clinical features of common variable immunodeficiency (CVID) complicated by non-cirrhotic portal hypertension (NCPH) and to deepen our understanding of it. The case data of CVID complicated with NCPH admitted to Peking Union Medical College Hospital from January 1983 to May 2021 were analyzed retrospectively to summarize their clinical characteristics. Six patients with CVID combined with NCPH (three of each sex; 16-45 years) were assessed. Four patients had portal hypertension. All patients had anemia, splenomegaly, a normal serum level of albumin and transaminases, and possibly increased levels of alkaline phosphatase and gamma-glutamyl transpeptidase. Two patients were diagnosed with esophagogastric fundic varices by gastroscopy. Two patients underwent splenectomy (which improved hematologic abnormalities partially). Four patients had autoimmune disease. Two cases were diagnosed with nodular regenerative hyperplasia (NRH) upon liver biopsy. Six patients were administered intravenous immunoglobulin-G (0.4-0.6 g/kg bodyweight) once every 3-4 weeks as basic therapy. Often, CVID complicated with NCPH has: (1) The manifestations of portal hypertension as the primary symptom. (2) Autoimmune-related manifestations. Imaging can provide important diagnostic clues. The etiology may be related to hepatic NRH and splenomegaly due to recurrent infections.

Ayurvedic treatment induced severe alcoholic hepatitis and non-cirrhotic portal hypertension in a 14-year-old girl

ABSTRACTWe report a novel and as yet undescribed clinical scenario in a young girl with liver failure, in whom, the liver histopathology was suggestive of alcoholic hepatitis in the background of hepatoportal sclerosis and incomplete septal cirrhosis. An extensive clinical and investigational evaluation revealed chronic consumption of multiple Ayurvedic herbal medications for seizure disease. Six months after stopping herbal medicines, the repeat liver biopsy demonstrated resolution of alcohol-related changes but persistence of classical features of non-cirrhotic portal hypertension. Analysis of the retrieved agents, including state of the art chemical and toxicology analysis, using gas chromatography and mass spectroscopy methods demonstrated multiple organic and inorganic toxins associated with acute alcohol and arsenic poisoning related hepatoportal sclerosis/incomplete septal cirrhosis in the young girl.

Pathology of non-cirrhotic portal hypertension and incomplete septal cirrhosis

This review will provide an overview of the pathological features of non-cirrhotic portal hypertension (NCPH) and incomplete septal cirrhosis (ISC), focussing on practical diagnostic approaches and problems. Although many liver diseases may be associated with portal hypertension in the absence of advanced fibrosis, the term NCPH is most widely used to describe a spectrum of changes resulting from occlusion of small portal vein branches, also referred to as “hepato-portal sclerosis” or “obliterative portal venopathy”. Other histological manifestations of NCPH include parenchymal atrophy, nodular regenerative hyperplasia, formation of shunt vessels and foci of sinusoidal dilatation. In more severe cases, foci of parenchymal collapse may be associated with formation of delicate non-linking fibrous septa, corresponding to incomplete septal cirrhosis. Although ISC is generally considered to be part of the morphological spectrum of NCPH, it can also be regarded as part of the spectrum of true cirrhosis – either reflecting cirrhosis that is incompletely developed or partially regressed.

Successful stenting of a complex inferior vena cava stenosis using a modified sharp recanulization technique.

We describe a case of a 30-year-old male who presented with features of noncirrhotic portal hypertension, who was diagnosed to have inferior vena cava (IVC) obstruction. IVC angiogram and ultrasound study revealed a long-segment (36 mm long), chronic total thrombotic occlusion that was dilated and stented with a satisfactory end result. The unique feature of this case is a modified sharp recanulization technique involving the use of Brokenborough (septal puncture) needle and Mullin dilator to create a track in such a long, chronic total occlusion under simultaneous ultrasound and fluoroscopic guidance.