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A rare case report of prune belly syndrome with malnutrition

Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by a triad of deficient abdominal wall musculature, undescended testicles and urinary tract malformations. Most of the patients have pulmonary, cardiac, skeletal and gastrointestinal tract anomalies. Lack...

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Detalles Bibliográficos
Autores principales: Hakimi, Turyalai, Ibrahimi, Mohammad Akbar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8081022/
https://www.ncbi.nlm.nih.gov/pubmed/33948190
http://dx.doi.org/10.1093/omcr/omab017
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author Hakimi, Turyalai
Ibrahimi, Mohammad Akbar
author_facet Hakimi, Turyalai
Ibrahimi, Mohammad Akbar
author_sort Hakimi, Turyalai
collection PubMed
description Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by a triad of deficient abdominal wall musculature, undescended testicles and urinary tract malformations. Most of the patients have pulmonary, cardiac, skeletal and gastrointestinal tract anomalies. Lack of abdominal muscles leads to constipation due to inability to perform Valsalva maneuver, which helps push the stool out of the rectum during the defecation. Additionally, frequent respiratory tract infections, persisting constipation and urinary tract infections lead to the development of malnutrition in children. We report this case to raise the awareness of low socioeconomic and low-resource medical settings that malnutrition could be existed or caused by PBS. We also encourage the expansion of pediatric surgery and family medicine training to increase the number of specialist (family medicine) to report and refer PBS in earlier phase, while working in rural areas and remote provinces.
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spelling pubmed-80810222021-05-03 A rare case report of prune belly syndrome with malnutrition Hakimi, Turyalai Ibrahimi, Mohammad Akbar Oxf Med Case Reports Case Report Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by a triad of deficient abdominal wall musculature, undescended testicles and urinary tract malformations. Most of the patients have pulmonary, cardiac, skeletal and gastrointestinal tract anomalies. Lack of abdominal muscles leads to constipation due to inability to perform Valsalva maneuver, which helps push the stool out of the rectum during the defecation. Additionally, frequent respiratory tract infections, persisting constipation and urinary tract infections lead to the development of malnutrition in children. We report this case to raise the awareness of low socioeconomic and low-resource medical settings that malnutrition could be existed or caused by PBS. We also encourage the expansion of pediatric surgery and family medicine training to increase the number of specialist (family medicine) to report and refer PBS in earlier phase, while working in rural areas and remote provinces. Oxford University Press 2021-04-28 /pmc/articles/PMC8081022/ /pubmed/33948190 http://dx.doi.org/10.1093/omcr/omab017 Text en © The Author(s) 2021. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Hakimi, Turyalai
Ibrahimi, Mohammad Akbar
A rare case report of prune belly syndrome with malnutrition
title A rare case report of prune belly syndrome with malnutrition
title_full A rare case report of prune belly syndrome with malnutrition
title_fullStr A rare case report of prune belly syndrome with malnutrition
title_full_unstemmed A rare case report of prune belly syndrome with malnutrition
title_short A rare case report of prune belly syndrome with malnutrition
title_sort rare case report of prune belly syndrome with malnutrition
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8081022/
https://www.ncbi.nlm.nih.gov/pubmed/33948190
http://dx.doi.org/10.1093/omcr/omab017
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