Lu Yi, Wang Xiao-hong, Wang Jian-she
Department of Liver Diseases, Children's Hospital of Fudan University, Shanghai 201102, China.
Zhonghua Er Ke Za Zhi. 2010 Oct;48(10):758-63.
To investigate the clinical features of autoimmune hepatitis (AIH) in children so as to improve the awareness of the disease.
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.
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 deliquescence 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.
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.
探讨儿童自身免疫性肝炎（AIH）的临床特点，以提高对该疾病的认识。
回顾性分析2004年至2008年间临床诊断为AIH的12例儿童的病历资料。采用国际自身免疫性肝炎小组（IAIHG）的AIH诊断评分系统来确诊。对临床表现、实验室检查、肝脏病理结果及预后进行回顾性分析。
11例患者经评分系统确诊为AIH，其中10例为Ⅰ型，1例未分型。从发病到确诊的平均时间为（7.5±7.4）个月。7例（63.6%）急性起病，其中2例进展为亚急性重型肝炎，3例（27.3%）隐匿起病，1例（9.1%）合并肝硬化。检测10例（90.9%）患者血清球蛋白和IgG水平升高，平均分别为（39.4±7.4）g/L和（31±12）g/L。10例抗核抗体（ANA）检测阳性，1例抗平滑肌抗体（SMA）阳性。均未检测到肝肾微粒体抗体（LKM-1）和抗线粒体抗体（AMA）。11例患者肝脏病理检查，5例为急性肝炎，6例为慢性肝炎。分别有3例出现严重的亚大块肝坏死和严重纤维化。肝脏病理检查发现81.8%的病例有淋巴细胞浸润，36.4%的病例有界面性肝炎，18.2%的病例有肝细胞玫瑰花结样环状排列。11例患者采用单一糖皮质激素或糖皮质激素联合免疫抑制剂治疗并进行随访。2例病情恶化，3例死亡。1例仍在治疗中，1例病情稳定，4例复发。
儿童AIH症状、体征、起病方式及实验室检查结果多样。肝脏病理改变不太典型。早期误诊率高。即使经过适当治疗，多数病例预后仍较差。因此，需密切关注AIH患儿。
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