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A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl

Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion,...

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Autores principales: Najjar, Afaf Mohammad Maher, Babensi, Nour Khaled, Ghazal, Ahmad, Brimo Alsaman, Muhamad Zakaria, Ismail, Alaa aldin, Alnaeb, Hasnaa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10558647/
https://www.ncbi.nlm.nih.gov/pubmed/37808573
http://dx.doi.org/10.1002/ccr3.7980
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author Najjar, Afaf Mohammad Maher
Babensi, Nour Khaled
Ghazal, Ahmad
Brimo Alsaman, Muhamad Zakaria
Ismail, Alaa aldin
Alnaeb, Hasnaa
author_facet Najjar, Afaf Mohammad Maher
Babensi, Nour Khaled
Ghazal, Ahmad
Brimo Alsaman, Muhamad Zakaria
Ismail, Alaa aldin
Alnaeb, Hasnaa
author_sort Najjar, Afaf Mohammad Maher
collection PubMed
description Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion, or chylous ascites. The goal of the treatment is to restrict the consumption of long‐chain fats and administer a formula that includes protein and medium‐chain triglycerides. Here, we report 11‐year‐old Syrian girl presented to the hospital with 2 years history of diarrhea and generalized edema. Furthermore, investigation showed ascites and pleural effusion. The disease was detected by several biopsies from duodenum that showed dilation of the lymph vessels within the lamina propria without any evidence for inflammation. She was managed by modifying her diet and albumin transfusion, which caused significant improvement in the child's condition.
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spelling pubmed-105586472023-10-08 A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl Najjar, Afaf Mohammad Maher Babensi, Nour Khaled Ghazal, Ahmad Brimo Alsaman, Muhamad Zakaria Ismail, Alaa aldin Alnaeb, Hasnaa Clin Case Rep Case Report Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion, or chylous ascites. The goal of the treatment is to restrict the consumption of long‐chain fats and administer a formula that includes protein and medium‐chain triglycerides. Here, we report 11‐year‐old Syrian girl presented to the hospital with 2 years history of diarrhea and generalized edema. Furthermore, investigation showed ascites and pleural effusion. The disease was detected by several biopsies from duodenum that showed dilation of the lymph vessels within the lamina propria without any evidence for inflammation. She was managed by modifying her diet and albumin transfusion, which caused significant improvement in the child's condition. John Wiley and Sons Inc. 2023-10-06 /pmc/articles/PMC10558647/ /pubmed/37808573 http://dx.doi.org/10.1002/ccr3.7980 Text en © 2023 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Case Report
Najjar, Afaf Mohammad Maher
Babensi, Nour Khaled
Ghazal, Ahmad
Brimo Alsaman, Muhamad Zakaria
Ismail, Alaa aldin
Alnaeb, Hasnaa
A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl
title A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl
title_full A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl
title_fullStr A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl
title_full_unstemmed A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl
title_short A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl
title_sort late diagnosis of primary intestinal lymphangiectasia in a syrian girl
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10558647/
https://www.ncbi.nlm.nih.gov/pubmed/37808573
http://dx.doi.org/10.1002/ccr3.7980
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