儿童自身免疫性肠病1例并文献复习

doi:10.6040/j.issn.1671-7554.0.2023.0217

摘要/Abstract

摘要： 目的探讨儿童自身免疫性肠病(AIE)的临床特点、病理组织学特征、诊断、治疗及预后。方法回顾性分析2021年12月收治于山东大学附属儿童医院消化科的1例AIE患儿的临床资料。分别以“儿童自身免疫性肠病”“自身免疫性肠病”“autoimmune enteropathy in children”“autoimmune enteropathy”为关键词检索中国知网、万方数据知识服务平台、pubmed数据库自建库至2023年3月的相关文献,总结儿童AIE的临床特点、病理组织学特征、诊疗及预后。结果患儿,女,3岁10个月,因腹泻18 d就诊,根据患儿难治性水样泻、电解质紊乱、低蛋白血症、营养不良等临床症状及典型病理改变,排除感染性腹泻、乳糜泻、极早发型炎症性肠病、先天性遗传代谢病及免疫缺陷病,确诊为儿童AIE。予甲泼尼龙治疗8个月余,停药复发,遂予他克莫司治疗,随访至2023年3月(他克莫司治疗6个月余),病情好转。检索符合条件的国外儿童AIE病例共66例,中国儿童AIE仅5例。结论儿童AIE发病率低,临床主要以难治性腹泻为主要表现,常规治疗及回避饮食无效,病理组织学特征性改变为明确诊断提供了重要依据,早期诊断并及时治疗有助于改善预后。

关键词: 儿童, 自身免疫性肠病, 腹泻, 小肠黏膜萎缩

Abstract: Objective To investigate the clinical and histopathological characteristics, diagnosis, treatment and prognosis of autoimmune enteropathy(AIE)in children. Methods The clinical data of a child with AIE treated at Department of Digestion, Childrens Hospital Affiliated to Shandong University in Dec. 2021 were retrospectively analyzed. With "childrens autoimmune enteropathy" "autoimmune enteropathy in children" "autoimmune enteropathy" as the key words, relevant literature in CNKI, Wanfang Data Knowledge Service Platform and PubMed database were retrieved from the inception to March 2023 to summarize the clinicopathological characteristics, diagnosis and treatment and prognosis of AIE. Results The girl, aged 3 years and 10 months, was admitted due to diarrhea for 18 days. According to the clinical symptoms and typical pathological changes, including refractory watery diarrhea, electrolyte disorder, hypoproteinemia and malnutrition, the diagnosis was AIE, after excluding infectious diarrhea, chylous diarrhea, extremely early inflammatory bowel disease, congenital genetic metabolic disease, and immunodeficiency disease. The child received methylprednisolone for more than 8 months, relapsed after the medication stopped, and then received tacrolimus. After tacrolimus use for more than six months, up to March 2023, the childs condition was improved. Literature retrieval collected 66 eligible cases of AIE in foreign children and 5 in Chinese children. Conclusion The incidence of AIE in children is low, and the main clinical manifestation is refractory diarrhea. Conventional treatment and avoidance diet are ineffective. Characteristic pathological histological changes provide an important basis for a clear diagnosis, and early diagnosis and timely treatment can help improve the prognosis.

Key words: Children, Autoimmune enteropathy, Diarrhea, Atrophy of small intestinal mucosa

中图分类号:

R725.7

符晓莉,魏绪霞,徐俊杰,薛宁,张乐,陈红苓. 儿童自身免疫性肠病1例并文献复习[J]. 山东大学学报 (医学版), 2023, 61(9): 118-124.

FU Xiaoli, WEI Xuxia, XU Junjie, XUE Ning, ZHANG Le, CHEN Hongling. Autoimmune enteropathy in children: a case report and literature review[J]. Journal of Shandong University (Health Sciences), 2023, 61(9): 118-124.

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