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An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis

Introduction Protein losing enteropathy is a symptom characterized by loss of protein in intestines resulting in low protein levels in serum and generalized edema. Several causes are reported for this condition. Hereby we report an as yet unreported cause of protein losing enteropathy that we named...

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Autores principales: Mahjoub, Fatemeh, Sani, Mehri Najafi, Tabari, Ahmad Khaleghnejad, Monajemzadeh, Maryam, Zandieh, Saeed
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Georg Thieme Verlag KG 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4336048/
https://www.ncbi.nlm.nih.gov/pubmed/25755965
http://dx.doi.org/10.1055/s-0033-1348040
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author Mahjoub, Fatemeh
Sani, Mehri Najafi
Tabari, Ahmad Khaleghnejad
Monajemzadeh, Maryam
Zandieh, Saeed
author_facet Mahjoub, Fatemeh
Sani, Mehri Najafi
Tabari, Ahmad Khaleghnejad
Monajemzadeh, Maryam
Zandieh, Saeed
author_sort Mahjoub, Fatemeh
collection PubMed
description Introduction Protein losing enteropathy is a symptom characterized by loss of protein in intestines resulting in low protein levels in serum and generalized edema. Several causes are reported for this condition. Hereby we report an as yet unreported cause of protein losing enteropathy that we named meso-intestinal fibrosis. Case Report A 2.5-year-old girl referred with features of partial intestinal obstruction and underwent laparotomy. She had history of protein losing enteropathy since 16 months of age with generalized edema and received albumin every other week. Workup of protein losing enteropathy was inconclusive and only a histology report denoted increase in eosinophils in lamina propria of small intestine and hypoallergenic diet was started for her, but no significant response was noted. Laparotomy revealed lace-like white areas in meso of small intestine and intestinal wall was firm in palpation in some areas. Biopsy was taken from these sites and histology revealed severe fibrosis of meso overlying muscularis propria and also patchy fibrosis of intestinal meso led to severe lymphangiectasis in submucosa of small intestine. Discussion Secondary lymphangiectasis due to obstruction of lymphatic flow is mentioned as cause of protein losing enteropathy. Meso-intestinal fibrosis seen in this case that led to secondary lymphangiectasis and also motility disorder has not been reported as yet.
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spelling pubmed-43360482015-03-09 An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis Mahjoub, Fatemeh Sani, Mehri Najafi Tabari, Ahmad Khaleghnejad Monajemzadeh, Maryam Zandieh, Saeed European J Pediatr Surg Rep Article Introduction Protein losing enteropathy is a symptom characterized by loss of protein in intestines resulting in low protein levels in serum and generalized edema. Several causes are reported for this condition. Hereby we report an as yet unreported cause of protein losing enteropathy that we named meso-intestinal fibrosis. Case Report A 2.5-year-old girl referred with features of partial intestinal obstruction and underwent laparotomy. She had history of protein losing enteropathy since 16 months of age with generalized edema and received albumin every other week. Workup of protein losing enteropathy was inconclusive and only a histology report denoted increase in eosinophils in lamina propria of small intestine and hypoallergenic diet was started for her, but no significant response was noted. Laparotomy revealed lace-like white areas in meso of small intestine and intestinal wall was firm in palpation in some areas. Biopsy was taken from these sites and histology revealed severe fibrosis of meso overlying muscularis propria and also patchy fibrosis of intestinal meso led to severe lymphangiectasis in submucosa of small intestine. Discussion Secondary lymphangiectasis due to obstruction of lymphatic flow is mentioned as cause of protein losing enteropathy. Meso-intestinal fibrosis seen in this case that led to secondary lymphangiectasis and also motility disorder has not been reported as yet. Georg Thieme Verlag KG 2013-05-31 2014-06 /pmc/articles/PMC4336048/ /pubmed/25755965 http://dx.doi.org/10.1055/s-0033-1348040 Text en © Thieme Medical Publishers
spellingShingle Article
Mahjoub, Fatemeh
Sani, Mehri Najafi
Tabari, Ahmad Khaleghnejad
Monajemzadeh, Maryam
Zandieh, Saeed
An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis
title An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis
title_full An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis
title_fullStr An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis
title_full_unstemmed An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis
title_short An Unusual Cause of Protein Losing Enteropathy in a 2.5-Year-Old Girl: Meso-Intestinal Fibrosis
title_sort unusual cause of protein losing enteropathy in a 2.5-year-old girl: meso-intestinal fibrosis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4336048/
https://www.ncbi.nlm.nih.gov/pubmed/25755965
http://dx.doi.org/10.1055/s-0033-1348040
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