Catarina Isabel Ribeiro
1* 
1 Nephrology Department, Vila Nova de Gaia-Espinho Hospital Center, Vila Nova de Gaia, Porto, Portugal
Abstract
Prune-Belly syndrome (PBS) is a rare congenital disease characterized by a clinical triad; abdominal muscle’s hypoplasia, severe urinary tract abnormalities and cryptorchid-ism. PBS consists of a multisystem disease, which includes cardiopulmonary, gastrointestinal, musculoskeletal and urinary anomalies in varying degree. The cause and pathogenesis of PBS is unknown and the severity of symptoms can diverge greatly. On one hand, this condition may cause severe renal and pulmonary disorders, sometimes incompatible with life; on the other hand, it may origin few and tenuous urological abnormalities. Treatment is variable; it usually includes surgical management of symptoms. Renal replacement therapy for those patients with end-stage kidney disease may be necessary and it includes dialysis and kidney transplantation. The kidney failure is the main cause of postnatal death.
Implication for health policy/practice/research/medical education:
The PBS is a rare congenital disease. It is a multisystem disease, which includes cardiopulmonary, gastrointestinal, musculoskeletal and urinary anomalies. The severity of renal disease is the primary factor that affects the clinical manifestations and the kidney failure is the main cause of postnatal death.
Please cite this paper as: Ribeiro CI. Prune-Belly syndrome; kidney injury in a rare disorder. J Nephropathol. 2021;10(3):e22. DOI: 10.34172/ jnp.2021.22.