Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients

Background: Non-cirrhotic portal hypertension of unknown cause is a poorly understood condition attributed to obstructive portal venopathy. Aim: To reassess the manifestations, course, and causes, with special attention to thrombosis. Methods: Analysis of a cohort of 28 patients. Results: Gastrointe...

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Published in:	Gut 2002-08, Vol.51 (2), p.275-280
Main Authors:	Hillaire, S, Bonte, E, Denninger, M-H, Casadevall, N, Cadranel, J-F, Lebrec, D, Valla, D, Degott, C
Format:	Article
Language:	eng
Subjects:	Adult anti-HBc anti-HBs antibodies against hepatitis B core antigen antibodies against hepatitis B surface antigen Anticoagulants Arsenic Biological and medical sciences Biopsy Blood clot Causes of Complications and side effects Female Follow-Up Studies Gastroenterology. Liver. Pancreas. Abdomen Gastrointestinal bleeding Gastrointestinal Hemorrhage - etiology HBsAg Hepatitis hepatitis B surface antigen hepatoportal sclerosis Humans Hypertension Hypertension, Portal - diagnostic imaging Hypertension, Portal - pathology Liver - diagnostic imaging Liver - pathology Liver Cirrhosis Liver Disease Liver diseases liver failure Liver. Biliary tract. Portal circulation. Exocrine pancreas Male Medical sciences Methods NASH NCIPH non-alcoholic steatohepatitis non-cirrhotic intrahepatic portal hypertension Other diseases. Semiology Patients perisinusoidal fibrosis Physiological aspects Portal hypertension Portal Vein portal vein thrombosis portal venules prothrombotic disorder Radiography Rodents Sclerosis Thrombosis Thrombosis - pathology Ultrasonography Veins & arteries Venous pressure
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Summary:	Background: Non-cirrhotic portal hypertension of unknown cause is a poorly understood condition attributed to obstructive portal venopathy. Aim: To reassess the manifestations, course, and causes, with special attention to thrombosis. Methods: Analysis of a cohort of 28 patients. Results: Gastrointestinal bleeding occurred in 11 patients. Liver failure developed at the time of concurrent disease in eight patients, including all four patients who died. Portal vein thrombosis developed in 13 patients. A prothrombotic disorder was found in 12 of 23 fully investigated patients. Hepatoportal sclerosis was observed in 11 patients (with associated perisinusoidal fibrosis and/or nodular regenerative hyperplasia in six); periportal fibrosis, perisinusoidal fibrosis, nodular regenerative hyperplasia, or a combination thereof were observed in other patients. A morphometric evaluation showed an increased number of portal vessels in patients with hepatoportal sclerosis. There was no relation between pathological results and haemodynamic findings or prothrombotic disorders. Conclusions: Outcome was related to associated conditions. Overlap in pathological, haemodynamic, and causal features suggests a single entity, with prothrombotic disorders as major causal factors, and injury to sinusoids as well as to portal venules as the primary mechanism. Activated coagulation could mediate vascular injury in the absence of thrombosis. Anticoagulation should be considered.
ISSN:	0017-5749 1468-3288