Posthouwer Dirk, Fischer Kathelijn, van Erpecum Karel J, Mauser-Bunschoten Eveline P
Van Creveldkliniek, the Julius Center for Health Sciences and Primary Care, and the Department of Gastroenterology, University Medical Center, Utrecht, the Netherlands.
Transfusion. 2006 Aug;46(8):1360-6. doi: 10.1111/j.1537-2995.2006.00903.x.
Although many patients with inherited bleeding disorders have been infected with hepatitis C in early childhood, the natural history of infection in this patient group remains poorly defined.
A total of 212 patients with inherited bleeding disorders born between 1976 and 1992 were evaluated for hepatitis C virus (HCV) infection, spontaneous clearance, and (by noninvasive tests) progressive liver disease.
A total of 120 of 212 patients had been exposed to non-HCV-inactivated clotting products, and 68 of these 120 patients (57%) were anti-HCV-positive. Of these patients, 44 (65%) had chronic hepatitis C (HCV RNA-positive) and 24 (35%) showed spontaneous clearance (HCV RNA-negative). Five patients with hepatitis C were coinfected with hepatitis B virus and/or human immunodeficiency virus (HIV). Multivariate analysis indicated that hepatitis C infection was independently associated with longer treatment period (odds ratio [OR], 1.6; 95% confidence interval [CI], 1.3-1.9) and exposure to a larger number of donors (OR, 2.1; 95% CI, 1.1-3.9). Spontaneous HCV clearance was associated with a younger age at first exposure to clotting product (p = 0.02). After a mean infection period of 21 years, evidence of cirrhosis was present in 2 patients (5%), both of whom were coinfected with HIV.
Spontaneous HCV clearance is associated with young age at infection. Despite frequent childhood-acquired hepatitis C infection among patients with inherited bleeding disorders, progression to cirrhosis after 21 years of infection is rare. The diagnosis of cirrhosis without biopsy, however, remains challenging in this population, and new, noninvasive means must be developed to accurately identify cirrhotic patients.
尽管许多遗传性出血性疾病患者在儿童早期就感染了丙型肝炎，但该患者群体中感染的自然病程仍不清楚。
对1976年至1992年间出生的212例遗传性出血性疾病患者进行了丙型肝炎病毒（HCV）感染、自发清除以及（通过非侵入性检测）进展性肝病的评估。
212例患者中有120例曾接触过未灭活HCV的凝血制品，其中68例（57%）抗HCV阳性。在这些患者中，44例（65%）患有慢性丙型肝炎（HCV RNA阳性），24例（35%）表现为自发清除（HCV RNA阴性）。5例丙型肝炎患者同时感染了乙型肝炎病毒和/或人类免疫缺陷病毒（HIV）。多变量分析表明，丙型肝炎感染与较长的治疗期独立相关（比值比[OR]，1.6；95%置信区间[CI]，1.3 - 1.9）以及接触更多的献血者（OR，2.1；95%CI，1.1 - 3.9）。HCV自发清除与首次接触凝血制品时年龄较小有关（p = 0.02）。经过平均21年的感染期后，2例患者（5%）出现肝硬化迹象，这2例患者均同时感染了HIV。
HCV自发清除与感染时年龄较小有关。尽管遗传性出血性疾病患者儿童期获得性丙型肝炎感染频繁，但感染21年后进展为肝硬化的情况罕见。然而，在该人群中，未经活检诊断肝硬化仍然具有挑战性，必须开发新的非侵入性方法来准确识别肝硬化患者。
