儿童自身免疫性肝炎11例临床分析

Clinical characteristics of autoimmune hepatitis in 11 children

目的 了解儿童自身免疫性肝炎（AIH）的临床特点,以提高对该病的认识.方法 提取2004至2008年临床诊断为AIH的12例病史及随访资料,参照AIH国际诊断评分标准对每个病例重新予以治疗前和治疗后评分,符合AIH诊断标准的资料进行回顾性分析.结果 11例符合确诊标准,临床表现为急性肝炎者7例（63.6%）,其中2例进展为亚急性重症肝炎,隐匿起病3例（27.3%）,肝硬化并发症起病1例（9.1%）.10例为Ⅰ型AIH,1例未分型.平均确诊时间（7.5±7.4）个月.血清球蛋白和IgG升高10例（90.9%）,抗核抗体阳性10例,其中1例合并平滑肌抗体阳性,肝肾微粒体抗体、抗线粒体抗体均阴性.11例病理符合慢性和急性炎症型者分别为6例和5例,肝细胞亚大块坏死等重度炎症表现3例,肝硬化3例,淋巴细胞浆细胞浸润9例（81.8%）,界面性肝炎4例（36.4%）,玫瑰花环2例（18.2%）.11例均经糖皮质激素或联合免疫抑制剂治疗,3例死于继发感染,2例恶化后失访,1例部分缓解,1例缓解,4例复发.除死亡和失访者外平均随访2.25年（1.5～3.5年）.结论 儿童AIH临床表现多样;部分进展快、病情重;病理表现典型者少;早期易误诊;需要长期随访治疗.

Objective To investigate the clinical features of autoimmune hepatitis （AIH） in children so as to improve the awareness of the disease. Method The medical records of 12 children who were clinically diagnosed as AIH between 2004 and 2008 were reviewed. The scoring system of the International Autoimmune Hepatitis Group （IAIHG） for diagnosis of AIH was used to confirm the diagnoses.Clinical manifestations, laboratory examinations, liver pathology results and prognosis were retrospectively analyzed. Result Eleven patients were diagnosed as AIH by the scoring system and 10 of them were type Ⅰ , one had not been typed. The average time from onset to diagnosis was （7.5 ± 7.4） months. Seven patients （63.6%） had acute onset, among them 2 cases progressed to subacute severe hepatitis,3 （27.3% ） had delitescence onset and 1 （9.1% ）was complicated with hepatic cirrhosis. Levels of serum globulin and IgG were tested and were higher in 10 cases （ 90.9% ） with average （ 39.4 ± 7.4） g/L and （31 ± 12）g/L respectively. Antinuclear antibodies （ANA） were measured positive in 10 cases, and 1 was anti-smooth muscle antibody （SMA） positive. Liver-kidney microsomal antibody （LKM-1） and anti-mitochondrial antibody （AMA） were detected in none of them. The liver pathology of 11 cases could be divided into acute and chronic hepatitis in 5 and 6 cases, respectively. Severe submassive liver necrosis and severe fibrosis were identified in 3 cases respectively. Lymphocytes infiltration, interfaces hepatitis and rosette-like annulation of hepatocytes were found in 81.8%, 36.4%, and 18.2% of cases on liver pathology. Eleven patients were followed up with therapy of single glucocorticoids or glucocorticoids combined with immunosuppressive agents. The disease of 2 cases deteriorated and 3 cases died. One case was still under therapy,1 case was stabilized and 4 cases had recurrence. Conclusion The children with AIH had diverse symptoms, signs, onsets and laboratory test results. The liver pathological changes were less typical. Rate of misdiagnosis was high in early stage. Prognosis was poor in most cases even though properly treated. Therefore close attention needs to be paid to children with AIH.