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Non-Cirrhotic Portal Hypertension: an Overview

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Abstract

Purpose of Review

Non-cirrhotic portal hypertension (NCPH) is a broad term, which includes a heterogeneous group of disorders characterized by elevated portal pressure in the absence of cirrhosis. Despite a better understanding, the diagnosis and treatment remains challenging. This review gives new insights into some of the recent advances.

Recent Findings

Although current guidelines for varices screening/surveillance are inadequate for NCPH, discovery of new genetic mutations helps us better understand its pathogenesis. Endohepatology is a promising emerging technique for an accurate diagnosis of NCPH. Pregnancy is not contraindicated however; multidisciplinary team approach is needed for an optimal outcome.

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SJ wrote the initial draft of the manuscript. AKS supervised and reviewed the first draft to make edits. All the authors reviewed and approved the manuscript.

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Correspondence to Ashwani K. Singal.

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Take Away Points

A good liver imaging should be performed to diagnose extrahepatic portal or splenic vein thrombosis as a cause for portal hypertension.

A liver biopsy is critical in the diagnosis of NCPH and excludes cirrhosis as cause of portal hypertension.

A comprehensive clinical evaluation is important for etiological diagnosis. Immunological and thrombophilia screen should be performed for patients without an obvious cause.

Endohepatology with endoscopic ultrasound-guided liver biopsy with simultaneous measurement of portal pressure gradient is an emerging technique to expedite the diagnostic process.

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Jalil, S., Singal, A.K. Non-Cirrhotic Portal Hypertension: an Overview. Curr Hepatology Rep 23, 153–161 (2024). https://doi.org/10.1007/s11901-024-00642-x

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