{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T08:03:48Z","timestamp":1792569828968,"version":"4.5.0"},"reference-count":0,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2019,1,1]],"date-time":"2019-01-01T00:00:00Z","timestamp":1546300800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2019,1,1]],"date-time":"2019-01-01T00:00:00Z","timestamp":1546300800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dig Dis"],"published-print":{"date-parts":[[2019]]},"abstract":"<b><i>Objective:<\/i><\/b> Intestinal lymphangiectasia (IL; primary or secondary) is an important cause of protein-losing enteropathy. We evaluated the clinicolaboratory profile, response to therapy, complications, and outcome of children with primary IL (PIL). <b><i>Methods:<\/i><\/b> Consecutive children (\u226418 years) diagnosed with PIL (clinical setting, typical small bowel histopathology, and exclusion of secondary causes) from 2007 to 2017 were evaluated. <b><i>Results:<\/i><\/b> Twenty-eight children with PIL (16 boys, age at symptom onset-12 [1\u2013192] months and at diagnosis 8 [1\u201318] years) were studied. Pedal edema (93%), chronic diarrhea (78.6%), and recurrent anasarca (64%) were the common presentations. Ascites, pleural, and pericardial effusion were seen in 64 (n-18; chylous-5, non-chylous-13), 18, and 18% cases, respectively. Hypoproteinemia, hypoalbuminemia, hypocalcemia, and lymphopenia were present in 82, 82, 75 and 39% cases, respectively. Duodenal biopsy established the diagnosis in 86% cases, while 14% required distal small bowel biopsies. Dietary therapy was given in all and 6 cases required additional therapy (octreotide-6, tranexamic acid-3, and total parenteral nutrition-1). Lymphedema (3\/5 vs. 1\/23), pleural effusion (4\/5 vs. 1\/23), and the need for additional therapy (4\/5 vs. 2\/23) were significantly more in patients with chylous ascites (<i>n<\/i> = 5) than those without chylous ascites (<i>n<\/i> = 23). Twenty-four cases in follow-up (39 [6\u2013120] months) showed improvement; however, 8 required readmission (symptom recurrence-6 [25%], complication-2 [8.3%], Budd Chiari Syndrome-1, and abdominal B cell lymphoma-1). <b><i>Conclusion:<\/i><\/b> Presence of chylous ascites suggests severe disease in children with PIL. Majority of PIL children respond to dietary therapy; only 20% need additional therapy. Long-term follow-up is essential to monitor for symptoms relapse and complications.<\/jats:p>","DOI":"10.1159\/000499450","type":"journal-article","created":{"date-parts":[[2019,4,26]],"date-time":"2019-04-26T21:00:40Z","timestamp":1556312440000},"page":"458-466","source":"Crossref","is-referenced-by-count":30,"title":["Clinical Profile, Response to Therapy, and Outcome of Children with Primary Intestinal Lymphangiectasia"],"prefix":"10.1159","volume":"37","author":[{"given":"Durga","family":"Prasad","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anshu","family":"Srivastava","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anil","family":"Tambe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Surender\u00a0Kumar","family":"Yachha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moinak\u00a0Sen","family":"Sarma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ujjal","family":"Poddar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2019,4,26]]},"container-title":["Digestive Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/499450","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/499450","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T16:27:46Z","timestamp":1745425666000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000499450"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019]]},"references-count":0,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2019,10,16]]},"published-print":{"date-parts":[[2019,10,24]]}},"URL":"https:\/\/doi.org\/10.1159\/000499450","archive":["Portico"],"relation":{},"ISSN":["0257-2753","1421-9875"],"issn-type":[{"value":"0257-2753","type":"print"},{"value":"1421-9875","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019]]}}}