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儿童自身免疫性肝炎的长期治疗减少和类固醇停药:55 例儿童单中心经验。

Long-term treatment reduction and steroids withdrawal in children with autoimmune hepatitis: a single centre experience on 55 children.

机构信息

Fédération des Spécialités Digestives bUnité d'Hépatologie, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.

出版信息

Eur J Gastroenterol Hepatol. 2009 Dec;21(12):1413-8. doi: 10.1097/MEG.0b013e32832ad5f7.

DOI:10.1097/MEG.0b013e32832ad5f7
PMID:19907227
Abstract

BACKGROUND

The combination of corticosteroids and azathioprine is the standard therapy for autoimmune hepatitis. The aim of this study was to describe our experience on long-term corticosteroid doses reducing and withdrawal in a large cohort of children with autoimmune hepatitis (AIH).

METHODS

All children presenting with AIH in our institution, from 1990 to 2006, were retrospectively included.

RESULTS

The study population included 55 children [38 females, 17 males, median age 8 years (ranging from 0.8 to 15)] with type 1 (74.5%), type 2 (20%) or seronegative (5.5%) AIH. The diagnosis was made in 41 of them at the time of acute hepatitis (75%); the other 14 were diagnosed as chronic liver disease (25%). Treatment consisted of corticosteroids and azathioprine in 45 patients or corticosteroids alone in five patients. Complete remission was obtained within 1 year in 31 (69%) patients. The median initial dose of corticosteroids was 1.6 mg/kg/day, and the dose was progressively reduced to 0.32 mg/kg/day at 1 year, 0.24 mg/kg/day at 3 years, 0.11 mg/kg/day at 5 years and 0.05 mg/kg/day at 10 years. Corticosteroids withdrawal was possible in 0% of patients at 1 year, 75% at 3 years, 78% at 5 years and 90% at 10 years. At the end of follow-up, azathioprine was maintained in 36 patients (80%). Total treatment withdrawal was obtained in four patients.

CONCLUSION

Our results strongly confirm that long-term corticosteroids withdrawal is possible in a large majority of children with autoimmune hepatitis.

摘要

背景

皮质类固醇和硫唑嘌呤的联合治疗是自身免疫性肝炎的标准疗法。本研究旨在描述我们在大量自身免疫性肝炎(AIH)患儿中使用长期皮质类固醇剂量减少和停药的经验。

方法

回顾性纳入我院 1990 年至 2006 年期间出现 AIH 的所有患儿。

结果

研究人群包括 55 例患儿[38 例女性,17 例男性,中位年龄 8 岁(范围 0.8-15 岁)],其中 1 型(74.5%)、2 型(20%)或血清阴性(5.5%)AIH。其中 41 例在急性肝炎时诊断(75%);其余 14 例诊断为慢性肝病(25%)。45 例患儿接受皮质类固醇和硫唑嘌呤治疗,5 例患儿仅接受皮质类固醇治疗。31 例(69%)患儿在 1 年内获得完全缓解。皮质类固醇的初始剂量中位数为 1.6mg/kg/天,1 年后剂量逐渐减少至 0.32mg/kg/天,3 年后减少至 0.24mg/kg/天,5 年后减少至 0.11mg/kg/天,10 年后减少至 0.05mg/kg/天。1 年内有 0%的患儿可停用皮质类固醇,3 年内有 75%的患儿可停用皮质类固醇,5 年内有 78%的患儿可停用皮质类固醇,10 年内有 90%的患儿可停用皮质类固醇。在随访结束时,36 例患儿(80%)继续使用硫唑嘌呤。4 例患儿完全停药。

结论

我们的结果强烈证实,在大多数自身免疫性肝炎患儿中,长期皮质类固醇停药是可行的。

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