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Prune belly syndrome: current perspectives

Prune belly syndrome (PBS) is a rare but morbid congenital disease, classically defined by a triad of cardinal features that includes cryptorchidism, urinary tract dilation and laxity of the abdominal wall musculature. Children often require numerous surgical interventions including bilateral orchid...

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Detalles Bibliográficos
Autores principales: Arlen, Angela M, Nawaf, Cayce, Kirsch, Andrew J
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6689549/
https://www.ncbi.nlm.nih.gov/pubmed/31496864
http://dx.doi.org/10.2147/PHMT.S188014
Descripción
Sumario:Prune belly syndrome (PBS) is a rare but morbid congenital disease, classically defined by a triad of cardinal features that includes cryptorchidism, urinary tract dilation and laxity of the abdominal wall musculature. Children often require numerous surgical interventions including bilateral orchidopexy as well as individually tailored urinary tract and abdominal wall reconstruction. Along with the classic features, patients with PBS often experience gastrointestinal, orthopedic, and cardiopulmonary comorbidities.