Abstract: Protein-losing enteropathy，or protein-losing gastroenteropathy，refers to a group of diseases in which serum proteins are excessively lost from the gastrointestinal tract due to various causes. The pathogenesis mainly includes mucosal erosion and exudation，increased permeability and intestinal lymphatic obstruction. Clinical manifestations vary greatly with underlying diseases，and the common manifestations are diarrhea，edema and malnutrition. The main characteristics of laboratory examination are the decrease of serum albumin and globulin. Increase in 24-hour alpha-1 antitryptase clearance rate or lymphonuclide intestinal protein tracing image can be used as an evidence of intestinal protein loss. Treatment includes dietary therapy and treatment for underlying diseases.

Key words: protein-losing enteropathy, intestinal lympangiectasia, dietary therapy, child

摘要： 蛋白丢失性肠病或称蛋白丢失性胃肠病是指各种原因所致的血清蛋白质从胃肠道过量丢失的一组疾病。发病机制主要包括黏膜糜烂渗出、通透性增加及肠淋巴管阻塞。临床表现随基础疾病不同而有很大差异，常见的有慢性腹泻、水肿、营养不良等。实验室检查以血清白蛋白、球蛋白均减低为主要特征。24 h α1抗胰蛋白酶清除率升高或淋巴核素肠蛋白示踪显像可作为肠道蛋白丢失的依据。治疗包括膳食疗法及针对基础疾病的治疗。

关键词: 蛋白丢失性肠病, 小肠淋巴管扩张, 膳食疗法, 儿童