Lopez Robert N, Day Andrew S
Department of Gastroenterology, Hepatology and Liver Transplantation, Queensland Children's Hospital, Brisbane, Queensland, Australia.
Department of Paediatrics, University of Otago, Christchurch, New Zealand.
J Paediatr Child Health. 2020 Nov;56(11):1719-1723. doi: 10.1111/jpc.14837. Epub 2020 May 28.
Primary intestinal lymphangiectasia is an uncommon condition that usually presents early in childhood. This incurable condition is consequent to underlying lymphatic abnormalities that lead to loss of lymphatic contents into the intestinal lumen. This article outlines an approach to the assessment of children presenting with characteristic features and consideration of other conditions that could lead to enteric protein loss. An overview of the management of primary intestinal lymphangiectasia is outlined.
原发性肠淋巴管扩张症是一种罕见的疾病，通常在儿童早期出现。这种无法治愈的疾病是由潜在的淋巴管异常导致淋巴内容物进入肠腔所致。本文概述了对具有特征性表现的儿童进行评估的方法，以及对其他可能导致肠道蛋白质丢失的疾病的考虑。同时概述了原发性肠淋巴管扩张症的治疗概况。
