Department of Pediatric Gastroenterology, Cleveland Clinic, 9500 Euclid Avenue, A 111, Cleveland, OH 44195, United States.
Dig Liver Dis. 2010 Oct;42(10):724-8. doi: 10.1016/j.dld.2010.01.002. Epub 2010 Feb 16.
Little is known regarding the natural history of autoimmune hepatitis in children. The aims of this longitudinal cohort study were to determine the long-term prognosis of children with autoimmune hepatitis and to determine the effect of cirrhosis at presentation on survival.
Thirty-three children with autoimmune hepatitis who were seen at our institution over a 25-year period were studied retrospectively.
The median age of diagnosis was 12.9 years (2.7-18.1) with a female predominance of 3:1. Liver biopsies showed cirrhosis in 18 (55%) patients at time of diagnosis. Patients with cirrhosis at baseline had a similar 10-year survival 85% (70-100%) to those without cirrhosis 75% (49-100%) (p=0.97). The overall survival was significantly lower than the expected in the age- and gender-matched U.S. population (log-rank test; p<0.001). In Cox regression models, weight loss (p=0.037), baseline elevated bilirubin (p=0.028), prolonged International Normalized Ratio (INR) (p=0.013), and positive LKM-1 antibodies (p=0.007) were associated with shorter survival.
AIH in children is associated with a significant shorter survival rate than the expected in the general population. Cirrhosis on initial liver biopsy does not seem to impact long-term survival in children with AIH.
本纵向队列研究旨在确定儿童自身免疫性肝炎的长期预后，并确定发病时肝硬化对生存的影响。
我们回顾性研究了在本机构就诊的 25 年间的 33 例自身免疫性肝炎患儿。
诊断中位年龄为 12.9 岁（2.7-18.1），女性占 3:1。基线时的肝活检显示 18 例（55%）患者存在肝硬化。基线时有肝硬化的患者 10 年生存率为 85%（70-100%）与无肝硬化的患者 75%（49-100%）相似（p=0.97）。总生存率明显低于同龄和同性别美国人群的预期（对数秩检验；p<0.001）。在 Cox 回归模型中，体重减轻（p=0.037）、基线胆红素升高（p=0.028）、国际标准化比值（INR）延长（p=0.013）和 LKM-1 抗体阳性（p=0.007）与生存时间缩短相关。
儿童自身免疫性肝炎的生存率明显低于普通人群的预期。初始肝活检时的肝硬化似乎不会影响儿童自身免疫性肝炎的长期生存。
