Prospective evaluation of patients with non‐cirrhotic portal hypertension: A single centre study

医学 门脉高压 肝硬化 失代偿 胃肠病学 内科学 前瞻性队列研究 门静脉压 肝病 静脉曲张 食管静脉曲张 门脉高压性胃病 肝活检 活检
作者
Maria Mironova,Harish Gopalakrishna,Gerardo Rodrı́guez,Nehna Abdul Majeed,Asif Hitawala,Ivan J. Fuss,Jenna Bergerson,Alison Faust,Jacqueline Laurin,Jaha Norman‐Wheeler,S. M. Scott,Julian Hercun,Bernadette Redd,David E. Kleiner,Christopher Koh,Theo Heller
出处
期刊:Alimentary Pharmacology & Therapeutics [Wiley]
标识
DOI:10.1111/apt.17987
摘要

Non-cirrhotic portal hypertension (NCPH) is a spectrum of liver diseases, including porto-sinusoidal vascular disorder, with portal hypertension (PH) in the absence of cirrhosis. The natural history and diagnostic approach to NCPH are not well understood.We aimed to evaluate disease progression and outcomes in NCPH.Patients with or at risk for NCPH were enrolled in a single centre prospective study; two groups were formed based on the presence of specific features of PH, such as varices, collaterals, portal hypertensive gastropathy or portal hypertensive bleeding. All participants underwent a baseline liver biopsy. Liver stiffness measurement (LSM), and imaging were repeated every 6-12 months.Fifteen patients without specific features of PH (Group I), and 35 patients with specific features (Group II) were enrolled. The median follow-up time was 50 months. Group II had higher hepatic venous pressure gradients, non-invasive measures of PH and a lower platelet count (PLT) when compared to Group I. Rates of survival and decompensation were similar in both groups. Patients with PLT ≤100 K/mcL had lower survival compared to those with PLT >100 K/mcL. Patients with LSM ≥10 kPa had lower survival and survival without decompensation when compared to patients with LSM <10 kPa.Patients irrespective of specific features of PH had similar survival or survival without decompensation. Patients without specific features are at risk for disease progression and should be monitored closely. Thrombocytopenia and increased LSM are associated with severe forms of liver disease, which are strongly associated with outcomes.
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