ArticleJournal of pediatric gastroenterology and nutrition2026
Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study.
Article in Journal of pediatric gastroenterology and nutrition, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study.Journal of pediatric gastroenterology and nutrition · 2026Article
- Successful Early Therapy With Sirolimus for an Infant With Primary Intestinal Lymphangiectasia.Case reports in pediatrics · 2026Article
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Authors and funding
13 authors.
Funding
Abstract
objectivesPrimary intestinal lymphangiectasia (PIL) is a very rare disease responsible for protein-losing enteropathy. There is little published data about treatments efficacy and outcomes. Our main objective was to describe the clinical profile, response to therapy, and outcomes of children with PIL.
methodsWe conducted a national retrospective study including children with PIL followed in French university hospitals between 2010 and 2022. Response to treatment was defined as clinical remission and no need for albumin infusion. Response was considered complete if albuminemia was normal (>35 g/l) during follow-up and partial if less than 35 g/l.
resultsThirty-four children (22 males) were included; median age at diagnosis was 7 (3-29,5) months. The median follow-up in our cohort was 4.5 years. Edema (79%), chronic diarrhea (50%), and ascites (35%) were the main symptoms. Thirty-one patients received a low long-chain triglycerides dietary therapy and 25 (81%) responded: 15 had a partial response and 10 a complete response. Four patients (12%) required a second-line drug treatment. The presence of lymphedema or an identified genetic variant were associated with a partial response to diet (p < 0.05). A normal diet could be reintroduced in 14 patients (45%) without relapse during the follow-up. Among them, nine children (26%) were considered cured with a complete and prolonged remission under a normal diet.
conclusionsMost children with PIL responded to diet therapy and about a quarter of the cohort had a good prognosis with complete remission even after discontinuation of the diet. Presence of lymphedema or a genetic variant was associated with a chronic condition.
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