Journal of Shandong University (Health Sciences) ›› 2023, Vol. 61 ›› Issue (9): 118-124.doi: 10.6040/j.issn.1671-7554.0.2023.0217

• Clinical Medicine • Previous Articles    

Autoimmune enteropathy in children: a case report and literature review

FU Xiaoli, WEI Xuxia, XU Junjie, XUE Ning, ZHANG Le, CHEN Hongling   

  1. Department of Digestion, Childrens Hospital Affiliated to Shandong University (Jinan Childrens Hospital), Jinan 250022, Shandong, China
  • Received:2023-02-17 Published:2023-10-10

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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

CLC Number: 

  • R725.7
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