Abu Faddan Nagla H, Abdel-Baky Laila, Aly Sherin A, Rashed Hebat-Allah G
Department of Pediatrics, Assuit University, Assuit, Egypt.
Arab J Gastroenterol. 2011 Dec;12(4):178-83. doi: 10.1016/j.ajg.2011.11.003. Epub 2011 Dec 27.
Auto-immune hepatitis (AIH) in children is a rare chronic progressive liver disorder. It is characterised serologically by high aminotransferase levels, elevated immunoglobulin G (IgG) and the presence of autoantibodies. AIH is divided into two types according to the autoantibody profile. This study aims to assess frequency, clinical manifestations, biochemical features and outcome of AIH in children attending Assuit University Hospitals in Upper Egypt with acute icteric hepatitis and seronegative viral markers (anti-hepatitis A virus (HAV) IgM, HbsAg, anti-hepatitis C virus (anti-HCV) Ab).
The study includes 34 children with AIH, diagnosed on the basis of the International Scoring Criteria of Auto-immune Hepatitis, recruited from Assuit University Hospitals, during the period from January 2005 to December 2009. All patients received prednisolone 2mgkg(-1)day(-1). Follow-up was done for 1year.
Among 34 children diagnosed as AIH, 24 were females (70.5%) and 10 were males (29.5%). Jaundice represented the most consistent finding in all patients. According to the autoantibody profile, 25 children were classified as type 1 and nine children were classified as type 2. Corticosteroid therapy was started. Complete remission was observed in 67.6% of patients and partial remission in 17.6%. There was no significant statistical difference in clinical and biochemical features of AIH in patients regarding the response to treatment. Mild side effects of steroid therapy were encountered in 48.2% of patients. After complete withdrawal of corticosteroids, six patients (20.7%) developed relapse.
AIH type 1 was the main form of AIH in children referred to Assiut University Hospitals. Girls were more affected than boys. AIH type 1 exhibited a more active, ongoing immunologic process. Steroid alone can be used successfully in most cases. Children with AIH type 2 had a higher frequency of relapse after corticosteroid withdrawal. Further studies on a larger number of cases and long-term follow-up are recommended.
儿童自身免疫性肝炎（AIH）是一种罕见的慢性进行性肝脏疾病。其血清学特征为转氨酶水平升高、免疫球蛋白G（IgG）升高以及自身抗体的存在。根据自身抗体谱，AIH分为两型。本研究旨在评估埃及上埃及阿斯尤特大学医院收治的急性黄疸型肝炎且病毒血清学标志物阴性（抗甲型肝炎病毒（HAV）IgM、乙肝表面抗原（HbsAg）、抗丙型肝炎病毒（抗-HCV）抗体）患儿中AIH的发病率、临床表现、生化特征及预后。
本研究纳入了2005年1月至2009年12月期间从阿斯尤特大学医院招募的34例根据自身免疫性肝炎国际评分标准确诊为AIH的患儿。所有患者均接受泼尼松龙2mg·kg⁻¹·d⁻¹治疗。随访1年。
在34例确诊为AIH的患儿中，24例为女性（70.5%），10例为男性（29.5%）。黄疸是所有患者中最常见的表现。根据自身抗体谱，25例患儿被分类为1型，9例患儿被分类为2型。开始使用皮质类固醇治疗。67.6%的患者实现完全缓解，17.6%的患者部分缓解。在患者对治疗的反应方面，AIH的临床和生化特征无显著统计学差异。48.2%的患者出现了类固醇治疗的轻度副作用。在完全停用皮质类固醇后，6例患者（20.7%）复发。
1型AIH是转诊至阿斯尤特大学医院的儿童AIH的主要形式。女孩比男孩受影响更严重。1型AIH表现出更活跃、持续的免疫过程。大多数情况下单独使用类固醇即可成功治疗。2型AIH患儿在停用皮质类固醇后复发频率更高。建议对更多病例进行进一步研究并进行长期随访。
