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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
Descripción
Sumario: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.