Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge

Introduction. Primary intestinal lymphangiectasia is a rare disorder, characterized by abnormal dilation of intestinal lymphatic vessels and extensive enteric loss of lymph rich in plasma proteins, lymphocytes and chylomicrons. The main characteristics of the disease are hypoalbuminemia, hypogammagl...

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Main Authors: Jocić Tatiana, Latinović-Bošnjak Olgica, Živojinov Mirjana, Stojšić Mirjana, Ilić-Sabo Jelena
Format: Article
Language:English
Published: Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade 2018-01-01
Series:Vojnosanitetski Pregled
Subjects:
Online Access:http://www.doiserbia.nb.rs/img/doi/0042-8450/2018/0042-84501600320J.pdf
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author Jocić Tatiana
Latinović-Bošnjak Olgica
Živojinov Mirjana
Stojšić Mirjana
Ilić-Sabo Jelena
author_facet Jocić Tatiana
Latinović-Bošnjak Olgica
Živojinov Mirjana
Stojšić Mirjana
Ilić-Sabo Jelena
author_sort Jocić Tatiana
collection DOAJ
description Introduction. Primary intestinal lymphangiectasia is a rare disorder, characterized by abnormal dilation of intestinal lymphatic vessels and extensive enteric loss of lymph rich in plasma proteins, lymphocytes and chylomicrons. The main characteristics of the disease are hypoalbuminemia, hypogammaglobulinemia, lymphocytopenia, and more rarely, the deficit of liposoluble vitamins and anemia. Except for primary, there are secondary lymphangiectasia, associated with celiac disease, malignant, infective and inflammatory diseases of the small intestine, fibrosis, liver and cardiovascular diseases. Case report. A male, 33 years of age, presented for his medical examination suffering from diarrhea and edema. The diagnosis was established upon the histological examination of a small intestine biopsy during double balloon enteroscopy, which revealed changes only in one segment of the intestine examined. Such a finding was later confirmed by the video endoscopy capsule. Conclusion. The diagnosis of intestinal lymphangiectasia is usually established before the age of 3, but it can also be diagnosed in adults. The diagnosis is based on the histological analysis of the intestinal mucosa biopsy, obtained by endoscopic procedures. The diagnosis of primary intestinal lymphangiectasia is also made upon the exclusion of secondary causes.
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publishDate 2018-01-01
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spelling doaj-art-e8e3b050d0a140809695b3df576d83b12025-08-20T03:24:38ZengMinistry of Defence of the Republic of Serbia, University of Defence, BelgradeVojnosanitetski Pregled0042-84502406-07202018-01-01751959910.2298/VSP160210320J0042-84501600320JPrimary intestinal lymphangiectasia in adults - diagnostic and therapeutic challengeJocić Tatiana0Latinović-Bošnjak Olgica1Živojinov Mirjana2Stojšić Mirjana3Ilić-Sabo Jelena4Clinical Center of Vojvodina, Clinic of Gastroenterology and Hepatology, Novi SadClinical Center of Vojvodina, Clinic of Gastroenterology and Hepatology, Novi SadClinical Center of Vojvodina, Center for Pathology and Histology, Novi SadClinical Center of Vojvodina, Institute of Child and Youth Health Care, Department of Gastroenterohepatology and Nutrition, Novi Sad Clinical Center of Vojvodina, Center for Pathology and Histology, Novi SadIntroduction. Primary intestinal lymphangiectasia is a rare disorder, characterized by abnormal dilation of intestinal lymphatic vessels and extensive enteric loss of lymph rich in plasma proteins, lymphocytes and chylomicrons. The main characteristics of the disease are hypoalbuminemia, hypogammaglobulinemia, lymphocytopenia, and more rarely, the deficit of liposoluble vitamins and anemia. Except for primary, there are secondary lymphangiectasia, associated with celiac disease, malignant, infective and inflammatory diseases of the small intestine, fibrosis, liver and cardiovascular diseases. Case report. A male, 33 years of age, presented for his medical examination suffering from diarrhea and edema. The diagnosis was established upon the histological examination of a small intestine biopsy during double balloon enteroscopy, which revealed changes only in one segment of the intestine examined. Such a finding was later confirmed by the video endoscopy capsule. Conclusion. The diagnosis of intestinal lymphangiectasia is usually established before the age of 3, but it can also be diagnosed in adults. The diagnosis is based on the histological analysis of the intestinal mucosa biopsy, obtained by endoscopic procedures. The diagnosis of primary intestinal lymphangiectasia is also made upon the exclusion of secondary causes.http://www.doiserbia.nb.rs/img/doi/0042-8450/2018/0042-84501600320J.pdfdiarrhealympangiectasishypoalbuminemialymphopeniaprotein-losing enteropathies
spellingShingle Jocić Tatiana
Latinović-Bošnjak Olgica
Živojinov Mirjana
Stojšić Mirjana
Ilić-Sabo Jelena
Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge
Vojnosanitetski Pregled
diarrhea
lympangiectasis
hypoalbuminemia
lymphopenia
protein-losing enteropathies
title Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge
title_full Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge
title_fullStr Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge
title_full_unstemmed Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge
title_short Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge
title_sort primary intestinal lymphangiectasia in adults diagnostic and therapeutic challenge
topic diarrhea
lympangiectasis
hypoalbuminemia
lymphopenia
protein-losing enteropathies
url http://www.doiserbia.nb.rs/img/doi/0042-8450/2018/0042-84501600320J.pdf
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