{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T19:58:46Z","timestamp":1791489526549,"version":"4.3.4"},"reference-count":24,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,11,23]],"date-time":"2025-11-23T00:00:00Z","timestamp":1763856000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,11,23]],"date-time":"2025-11-23T00:00:00Z","timestamp":1763856000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Primary intestinal lymphangiectasia (PIL) is a very rare disease responsible for protein\u2010losing 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We 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 (\n                      <jats:styled-content>&gt;<\/jats:styled-content>\n                      35\u2009g\/l) during follow\u2010up and partial if less than 35\u2009g\/l.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Thirty\u2010four children (22 males) were included; median age at diagnosis was 7 (3\u201329,5) months. The median follow\u2010up in our cohort was 4.5 years. Edema (79%), chronic diarrhea (50%), and ascites (35%) were the main symptoms. Thirty\u2010one patients received a low long\u2010chain triglycerides dietary therapy and 25 (81%) responded: 15 had a partial response and 10 a complete response. Four patients (12%) required a second\u2010line drug treatment. The presence of lymphedema or an identified genetic variant were associated with a partial response to diet (\n                      <jats:italic>p<\/jats:italic>\n                      <jats:styled-content>\u2009&lt;<\/jats:styled-content>\n                      \u20090.05). A normal diet could be reintroduced in 14 patients (45%) without relapse during the follow\u2010up. Among them, nine children (26%) were considered cured with a complete and prolonged remission under a normal diet.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Most 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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.70266","type":"journal-article","created":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T07:31:26Z","timestamp":1763969486000},"page":"398-406","source":"Crossref","is-referenced-by-count":2,"title":["Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study"],"prefix":"10.1002","volume":"82","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-1223-5314","authenticated-orcid":false,"given":"No\u00e9mie","family":"Goret","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine P\u00e9diatrique, CHU de Tours Tours France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9cile","family":"Lambe","sequence":"additional","affiliation":[{"name":"Service de Gastroent\u00e9rologie, H\u00e9patologie et Nutrition P\u00e9diatrique, H\u00f4pital Necker \u2013 Enfants Malades Universit\u00e9 Paris Cit\u00e9 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Ecochard\u2010Dugelay","sequence":"additional","affiliation":[{"name":"Service de Gastroent\u00e9rologie, H\u00e9patologie et Nutrition P\u00e9diatrique, APHP Robert Debr\u00e9 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Fabre","sequence":"additional","affiliation":[{"name":"APHM, H\u00f4pital de la Timone Enfant, Service de P\u00e9diatrie Multidisciplinaire Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Dabadie","sequence":"additional","affiliation":[{"name":"P\u00e9diatrie, CHU Rennes Rennes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lie","family":"Comte","sequence":"additional","affiliation":[{"name":"Service de P\u00e9diatrie, CHU Besan\u00e7on Besan\u00e7on France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Rebeuh","sequence":"additional","affiliation":[{"name":"Service de P\u00e9diatrie H\u00f4pitaux Universitaires de Strasbourg Strasbourg France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Poinsot","sequence":"additional","affiliation":[{"name":"Service de Gastroent\u00e9rologie, H\u00e9patologie et Nutrition P\u00e9diatrique, Hospices Civil de Lyon, H\u00f4pital Femme M\u00e8re Enfant Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilie","family":"Chaillou","sequence":"additional","affiliation":[{"name":"D\u00e9partement de P\u00e9diatrie m\u00e9dicale, CHU Angers Angers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haude","family":"Clouzeau","sequence":"additional","affiliation":[{"name":"Service de Gastrop\u00e9diatrie, CHU de Bordeaux, H\u00f4pital Pellegrin\u2010Enfants Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lie","family":"Sabard","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine P\u00e9diatrique, CHU de Tours Tours France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9atrice","family":"Dubern","sequence":"additional","affiliation":[{"name":"Service de Nutrition et Gastroent\u00e9rologie P\u00e9diatrique, APHP Trousseau Sorbonne Universit\u00e9 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phanie","family":"Willot","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine P\u00e9diatrique, CHU de Tours Tours France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,11,23]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.revmed.2017.07.009"},{"key":"e_1_2_7_3_1","unstructured":"Minist\u00e8re de la Sant\u00e9. National Diagnostic and Care Protocol (PNDS) \u2013 Primary intestinal lymphangiectasia (Waldmann disease). Paris: Minist\u00e8re de la Sant\u00e9.2022.https:\/\/www.has\u2010sante.fr\/upload\/docs\/application\/pdf\/2022\u201011\/pdns_waldmann_vignes.pdf"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1746.2007.05225.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.25259\/IJDVL_287_19"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0029-1216389"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.revmed.2016.07.005"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-2562"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e3181d1b162"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12887-020-02447-5"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2009.06.034"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.3349\/ymj.2021.62.5.470"},{"key":"e_1_2_7_13_1","first-page":"23","article-title":"Pediatric localized intestinal lymphangiectasia treated with resection","volume":"12","author":"Mari J","year":"2019","journal-title":"Int Med Case Rep J"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1067\/mob.2001.106756"},{"key":"e_1_2_7_15_1","first-page":"OD17","article-title":"Primary intestinal lymphangiectasia (Waldmann's Disease) presenting with chylous effusions in a 15\u2010year\u2010Old","volume":"11","author":"Surampalli V","year":"2017","journal-title":"J Clin Diagn Res"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.12998\/wjcc.v10.i18.6234"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12876-022-02318-6"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.12659\/AJCR.899636"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0929-693X(01)00799-0"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10620-010-1161-1"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.revmed.2006.11.019"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-007-0445-8"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1159\/000499450"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-3257"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2301594"}],"container-title":["Journal of Pediatric Gastroenterology and Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpn3.70266","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jpn3.70266","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpn3.70266","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T03:50:33Z","timestamp":1776397833000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jpn3.70266"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,23]]},"references-count":24,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,2]]}},"alternative-id":["10.1002\/jpn3.70266"],"URL":"https:\/\/doi.org\/10.1002\/jpn3.70266","archive":["Portico"],"relation":{},"ISSN":["0277-2116","1536-4801"],"issn-type":[{"value":"0277-2116","type":"print"},{"value":"1536-4801","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,23]]}}}