Prune-Belly syndrome; kidney injury in a rare disorder

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.

Keywords

• prune-belly syndrome
• end-stage renal disease
• renal replacement therapy
• cryp-torchidism