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目的探讨儿童自身免疫性肝炎(autoimmune hepatitis,AIH)的临床特征、肝脏病理及治疗转归。方法回顾性分析2007年6月—2012年6月在我中心住院的36例儿童AIH的临床、肝脏病理、治疗及随访资料,进行统计学分析。结果①患者年龄中位数11岁(1.3~18.0岁),女性占69.44%。②8.33%的患者急性起病,16.67%亚急性起病,75.00%慢性起病,30.56%就诊时已发展为肝硬化。36例ALT、AST、TBIL、球蛋白(GLO)和免疫球蛋白(IgG)分别为(223.88±218.11)μ/L、(352.92±247.19)μ/L、(90.20±79.09)μmol/L、(34.51±11.06)g/L和(31.43±15.07)g/L。77.78%的患者IgG高于正常,41.67%血沉快。32例行肝脏病理检查,其中65.63%肝脏炎症≥3级,68.75%肝纤维化≥3期。③分组:Ⅰ型占77.78%,Ⅱ型占19.44%,2.78%自身抗体全阴性。Ⅰ型患者GLO和IgG平均值明显高于Ⅱ型(P=0.003、0.000)。④32例接受治疗,其中75.00%激素单药或联合免疫抑制剂治疗有效,12.50%疗效不佳。治疗有效患者中11例治疗中出现转氨酶波动,5例停药后转氨酶波动需再次治疗,1例停药6个月后肝功能维持正常。⑤7例治疗前后行2次肝脏穿刺均提示肝脏炎症明显减轻,纤维化程度改善。结论①儿童AIH发病以慢性起病多见,Ⅰ型为主;②2/3的儿童AIH患者肝脏炎症和纤维化分期较重,均≥3级/3期;③75.00%的AIH患者激素单药或联合硫唑嘌呤治疗有效,激素治疗可明显改善炎症和纤维化程度,但需较长期治疗。
Objective To investigate the clinical features, autoimmune hepatitis (AIH) in children and the prognosis of liver disease. Methods The clinical data, liver pathology, treatment and follow-up data of 36 children with AIH admitted to our center from June 2007 to June 2012 were analyzed retrospectively for statistical analysis. Results ① The median age of patients was 11 years (1.3 ~ 18.0 years) and female was 69.44%. ② 8.33% of patients had acute onset, 16.67% subacute onset, 75.00% chronic onset, and 30.56% developed cirrhosis at diagnosis. The levels of ALT, AST, TBIL, GLO and IgG were (223.88 ± 218.11) μL, (352.92 ± 247.19) μL, (90.20 ± 79.09) μmol / L, 34.51 ± 11.06) g / L and (31.43 ± 15.07) g / L respectively. 77.78% patients with IgG higher than normal, 41.67% ESR fast. 32 routine liver biopsy, of which 65.63% of liver inflammation ≥ 3, 68.75% of liver fibrosis ≥ 3. ③ grouping: Ⅰ type accounted for 77.78%, Ⅱ type accounted for 19.44%, 2.78% of all autoantibodies negative. Type I patients GLO and IgG average was significantly higher than type Ⅱ (P = 0.003,0.000). ④32 cases received treatment, of which 75.00% hormone single agent or combined immunosuppressive therapy, 12.50% poor efficacy. In the treatment-effective patients, 11 cases showed transaminase fluctuation during the treatment, 5 cases were treated again after the withdrawal of aminotransferase volatility, and 1 case remained normal after 6 months withdrawal. ⑤ 7 cases before and after the line 2 times the liver puncture are significantly reduced liver inflammation, fibrosis improved. Conclusions ① The onset of AIH is more common in children with chronic onset, and type I is predominant. ② Two thirds of children with AIH have severe liver inflammation and fibrosis stages, all of which are grade ≥3 / 3; ③75.00% Drugs or combined azathioprine treatment is effective, hormone therapy can significantly improve the degree of inflammation and fibrosis, but more long-term treatment.