García Romero R, Martín de Carpi J, Bernal Cuartas C, Pinillos Pisón S, Varea Calderón V
Sección de Gastroenterología, Hepatología y Nutrición Pediátrica, Hospital Sant Joan de Déu, Barcelona.
Rev Esp Enferm Dig. 2007 May;99(5):255-8. doi: 10.4321/s1130-01082007000500002.
Autoimmune hepatitis (AIH) is an inflammatory disease of unknown origin that is responsible for progressive liver necrosis and ultimately cirrhosis.
Our aim was to evaluate the characteristics of autoimmune hepatitis presenting in the pediatric age.
We conducted a retrospective study of all patients diagnosed with AIH in our hospital department during the last 10 years. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology, treatment, need for transplant, and clinical evolution. According to the positive level of auto-antibodies, AIH patients were classified as type I AIH (ANA and/or smooth-muscle antibodies) and type II (anti-LKM-1).
Seven patients were diagnosed in this period -5 girls (71.5%) and 2 boys (28.5%). Five patients presented with type-I serological markers, and two with type-II markers. Age range at diagnosis was from 21 months to 12 years. In the type-I group, 3 patients presented with acute hepatitis while 2 other patients were diagnosed from laboratory findings while asymptomatic. Elevated aminotransferase (10 times the normal level) was observed in 71.5%, and 85% had elevated immunoglobulins. Treatment with azathioprine and prednisone was started after diagnosis with an average time to remission of 14 months. Two patients relapsed following steroid withdrawal.
AIH can have different forms of clinical presentation, and is sometimes indistinguishable from viral hepatitis. AIH must be ruled out in patients presenting with concomitant elevation of aminotransferases and immunoglobulins. The commonly accepted treatment is a combination of azathioprine and corticosteroids. A high percentage of patients experience a relapse of disease after steroids are withdrawn. Therefore, some patients will need to stay on combined therapy with minimal doses of steroids.
自身免疫性肝炎（AIH）是一种病因不明的炎症性疾病，可导致进行性肝坏死并最终发展为肝硬化。
我们的目的是评估儿童期自身免疫性肝炎的特征。
我们对过去10年在我院科室诊断为AIH的所有患者进行了回顾性研究。分析的变量包括年龄、性别、临床表现、肝功能、免疫球蛋白、自身免疫标志物、组织学、治疗、移植需求及临床进展。根据自身抗体阳性水平，AIH患者被分为I型AIH（抗核抗体和/或平滑肌抗体）和II型（抗肝肾微粒体-1抗体）。
在此期间共诊断出7例患者，其中5例为女孩（71.5%），2例为男孩（28.5%）。5例患者呈现I型血清学标志物，2例呈现II型标志物。诊断时的年龄范围为21个月至12岁。在I型组中，3例患者表现为急性肝炎，另外2例患者在无症状时通过实验室检查确诊。71.5%的患者转氨酶升高（高于正常水平10倍），85%的患者免疫球蛋白升高。诊断后开始使用硫唑嘌呤和泼尼松治疗，平均缓解时间为14个月。2例患者在停用类固醇后复发。
AIH可有不同的临床表现形式，有时与病毒性肝炎难以区分。转氨酶和免疫球蛋白同时升高的患者必须排除AIH。普遍接受的治疗方法是硫唑嘌呤和皮质类固醇联合使用。很大一部分患者在停用类固醇后疾病复发。因此，一些患者需要持续接受最小剂量类固醇的联合治疗。
