Ohlendorf Johanna, Kiene Hella, Wiegandt Jessica, Karch André, Jaeger Veronika K, Laue Tobias, Junge Norman, Mutschler Frauke, Goldschmidt Imeke, Pfister Eva-Doreen, Leiskau Christoph, Petersen Claus, Madadi-Sanjani Omid, Kuebler Joachim Friedrich, Götz Juliane Katharina, Baumann Ulrich
Paediatric Gastroenterology, Hepatology and Liver Transplantation, Hanover Medical School, 30625 Hanover, Germany.
Institute of Epidemiology and Social Medicine, University of Muenster, 48149 Muenster, Germany.
J Clin Med. 2023 Mar 8;12(6):2108. doi: 10.3390/jcm12062108.
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.
流行病学证据表明，包括男性、非O血型、亚甲基四氢叶酸还原酶（MTHFR）nt677TT突变、凝血因子V莱顿（Factor V Leiden）G1691A突变以及凝血酶原G20210A多态性在内的血栓形成倾向因素，可能促使肝病患者的纤维化进展和门静脉血栓形成。我们进行了一项回顾性研究，以142名在2017年4月至2019年10月期间于汉诺威医学院接受胆道闭锁随访的儿童为队列，研究潜在血栓形成倾向因素对自体肝存活的影响，将其作为与疾病进展相关的患者终点指标。未发现显著关联。未发现凝血因子V莱顿G1691A突变（风险比[HR]=0.86，95%置信区间[CI]0.38 - 1.98，P = 0.73）、凝血酶原G20210A多态性（HR = 0.96，95%CI 0.24 - 3.65，P = 0.96）、非O血型（HR = 0.79，95%CI 0.51 - 1.21，P = 0.28）或MTHFR nt677TT突变（HR = 1.24，95%CI 0.60 - 2.56，P = 0.56）与自体肝存活存在相关差异的证据。不过，有一定（尽管不太显著）证据表明，在接受肝门空肠吻合术后的男性患者中，自体肝存活有所降低，尤其是在最初2000天内（降低42%；HR = 1.41，95%CI 0.92 - 2.18，P = 0.11）。所有移植前门静脉血栓形成的儿童（n = 7）均为非O血型。需要开展更大规模的多中心研究，以确定男性或其他血栓形成倾向因素是否可能与自体肝存活降低存在潜在关联。
