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丹麦东部儿童自身免疫性肝炎。

Autoimmune hepatitis in children in Eastern Denmark.

机构信息

Sarcoma Unit, Royal Marsden Hospital, London, UK

出版信息

J Pediatr Gastroenterol Nutr. 2012 Oct;55(4):376-9. doi: 10.1097/MPG.0b013e3182602b20.

Abstract

OBJECTIVES

Autoimmune hepatitis (AIH) in childhood is a progressive chronic inflammatory liver disease. The aim of this study was to compare the clinical and biochemical characteristics of 33 paediatric patients diagnosed as having AIH with earlier described cohorts, and to examine the effect of early treatment strategies on the course of disease.

METHODS

A population-based cohort of patients from January 1993 to September 2009 was identified prospectively, and the patient data were collected by a retrospective examination of the files.

RESULTS

Twenty-nine patients had type 1 AIH, 2 had type 2, and 2 could not be categorised. Among the 33 children, 16 (48.5%) were girls and 17 (51.5%) were boys. Twenty-three (69.7%) of the patients had symptoms at presentation indistinguishable from acute viral hepatitis, but in 16 (69.6%) of those the liver biopsy showed cirrhosis. Twenty (60.6%) patients were treated with prednisolone and azathioprine at the time of remission, whereas 8 (24.2%) were treated with prednisolone. One (3%) patient did not experience remission during the observation period.

CONCLUSIONS

The patients in our study appeared similar to previously published cohorts, although a female predominance was not observed. Our data suggest that early treatment including both prednisolone and azathioprine could be more effective than prednisolone alone, even if randomised controlled paediatric studies comparing these 2 different treatment regimens are needed.

摘要

目的

儿童自身免疫性肝炎(AIH)是一种进行性慢性炎症性肝病。本研究旨在比较 33 例诊断为 AIH 的儿科患者的临床和生化特征与先前描述的队列,并探讨早期治疗策略对疾病进程的影响。

方法

前瞻性地确定了 1993 年 1 月至 2009 年 9 月的基于人群的患者队列,并通过对病历的回顾性检查收集患者数据。

结果

29 例患者为 1 型 AIH,2 例为 2 型,2 例无法分类。在 33 名儿童中,16 名(48.5%)为女孩,17 名(51.5%)为男孩。23 例(69.7%)患者在出现症状时与急性病毒性肝炎无法区分,但在 16 例(69.6%)患者中,肝活检显示肝硬化。20 例(60.6%)患者在缓解时接受泼尼松龙和硫唑嘌呤治疗,而 8 例(24.2%)接受泼尼松龙治疗。1 例(3%)患者在观察期间未缓解。

结论

我们研究中的患者与先前发表的队列相似,尽管未观察到女性优势。我们的数据表明,包括泼尼松龙和硫唑嘌呤在内的早期治疗可能比单独使用泼尼松龙更有效,即使需要比较这两种不同治疗方案的随机对照儿科研究。

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