Digestive Diseases

Small and Large Bowel: Research Article

Clinical Profile, Response to Therapy, and Outcome of Children with Primary Intestinal Lymphangiectasia

Prasad D. · Srivastava A. · Tambe A. · Yachha S.K. · Sarma M.S. · Poddar U.

Author affiliations

Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

Keywords: Intestinal lymphangiectasiaChildrenPrimaryProtein-losing enteropathy

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Dig Dis 2019;37:458–466
https://doi.org/10.1159/000499450

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Article / Publication Details

First-Page Preview
Abstract of Small and Large Bowel: Research Article

Received: August 27, 2018
Accepted: March 06, 2019
Published online: April 26, 2019
Issue release date: October 2019

Number of Print Pages: 9
Number of Figures: 3
Number of Tables: 3

ISSN: 0257-2753 (Print)
eISSN: 1421-9875 (Online)

For additional information: https://www.karger.com/DDI

Abstract

Objective: 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). Methods: Consecutive children (≤18 years) diagnosed with PIL (clinical setting, typical small bowel histopathology, and exclusion of secondary causes) from 2007 to 2017 were evaluated. Results: Twenty-eight children with PIL (16 boys, age at symptom onset-12 [1–192] months and at diagnosis 8 [1–18] 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 (n = 5) than those without chylous ascites (n = 23). Twenty-four cases in follow-up (39 [6–120] 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). Conclusion: 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.

© 2019 S. Karger AG, Basel



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Article / Publication Details

First-Page Preview
Abstract of Small and Large Bowel: Research Article

Received: August 27, 2018
Accepted: March 06, 2019
Published online: April 26, 2019
Issue release date: October 2019

Number of Print Pages: 9
Number of Figures: 3
Number of Tables: 3

ISSN: 0257-2753 (Print)
eISSN: 1421-9875 (Online)

For additional information: https://www.karger.com/DDI

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2019, Vol.37, No. 6

October 2019

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