The Impact of Thrombophilic Factors on Disease Progression in Children with Biliary Atresia-A Single-Centre Cohort Study

Overview

Journal J Clin Med

Specialty General Medicine

Date 2023 Mar 29

PMID 36983111

Authors

Johanna Ohlendorf

Hella Kiene

Jessica Wiegandt

Andre Karch

Veronika K Jaeger

Tobias Laue

Norman Junge

Frauke Mutschler

Imeke Goldschmidt

Eva-Doreen Pfister

Christoph Leiskau

Claus Petersen

Omid Madadi-Sanjani

Joachim Friedrich Kuebler

Juliane Katharina Gotz

Ulrich Baumann

Affiliations

Soon will be listed here.

Abstract

Epidemiological evidence suggests that thrombophilic factors, including male sex, non-O blood type, MTHFRnt677TT mutation, factor V Leiden G1691A mutation, and prothrombin G20210A polymorphism, may contribute to the progression of fibrosis and occurrence of portal vein thrombosis in liver disease. We retrospectively investigated the effect of potentially thrombophilic factors on native liver survival as a patient-relevant endpoint of disease progression in a cohort of 142 children being followed up for biliary atresia at Hannover Medical School from April 2017 to October 2019. No significant association could be determined. There was no evidence for relevant differences in native liver survival for the Factor V Leiden G1691A mutation (hazard ratio [HR] = 0.86, 95% confidence interval [CI] 0.38-1.98, = 0.73), prothrombin G20210A polymorphism (HR = 0.96, 95%CI 0.24-3.65, = 0.96), non-O blood type (HR = 0.79, 95%CI 0.51-1.21, = 0.28) or MTHFRnt677TT mutation (HR = 1.24, 95%CI 0.60-2.56, = 0.56). A certain, albeit not strong, evidence of reduced native liver survival in male patients after Kasai hepatoportoenterostomy, particularly during the first 2000 days (42%; HR = 1.41, 95%CI 0.92-2.18, = 0.11) was found. All children with pre-transplant portal vein thrombosis ( = 7) had non-O blood types. Larger multi-centre studies are necessary to show if the male sex or other thrombophilic factors could be potentially associated with reduced native liver survival.

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