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Idiopathic noncirrhotic portal hypertension

Definition
INCPH is a rare liver disorder characterized by an increased portal venous pressure gradient in the absence of a known cause of liver disease and portal vein thrombosis.
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Pathophysiology
The pathophysiology of INCPH is complex and not fully understood. However, three main mechanisms have been proposed: increased resistance to blood flow in the portal vein, abnormal angiogenesis leading to the formation of portosystemic collaterals, and sinusoidal endothelial dysfunction.
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Epidemiology
INCPH is a rare disease in the Western world, with a higher prevalence in India.
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Disease course
Clinical manifestations of INCPH can include recurrent ascites, splenomegaly, variceal bleeding, and portal vein thrombosis. It can also lead to serious complications such as liver failure, HCC, and portal hypertensive gastropathy.
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Prognosis and risk of recurrence
The prognosis of INCPH is generally favorable as it does not typically lead to liver failure.
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Key sources
The following summarized guidelines for the evaluation and management of idiopathic noncirrhotic portal hypertension are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2016).
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Guidelines

1.Screening and diagnosis

Diagnostic criteria: consider the diagnosis of INCPH in any patient with portal hypertension, particularly when there is no other cause for liver disease.
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2.Diagnostic investigations

Initial evaluation: exclude cirrhosis and other causes of non-cirrhotic portal hypertension prior to diagnosing INCPH.
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3.Diagnostic procedures

Liver biopsy: obtain a liver biopsy to diagnose INCPH.
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4.Medical management

Management of portal hypertension: manage portal hypertension according to guidelines elaborated for patients with cirrhosis.
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5.Surgical interventions

Liver transplantation: consider liver transplantation in patients with INCPH who develop liver failure or unmanageable portal hypertension-related complications.
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6.Follow-up and surveillance

Monitoring for portal vein thrombosis: screen patients with INCPH at least every 6 months for the occurrence of portal vein thrombosis.
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