title: [Peritoneal dialysis in adult patients with prune belly syndrome: an impossible challenge?].
abstract: Prune belly syndrome (PBS) is a rare congenital syndrome characterized by hypoplasia of the abdominal muscles, urinary tract malformations, and cryptorchidism in males. The estimated incidence is 1 in 35,000 to 50,000 live births. Chronic renal failure and end-stage renal disease (ESRD), due both to different degrees of renal hypoplasia or dysplasia and infectious complications, develops in 20-30% of patients who survive the neonatal period. No data are available on progression time to ESRD, owing to the variability of the phenotypic features of nephropathy. Nevertheless, PBS is primarily a pathology of pediatric interest as demonstrated, for example, by the reported average age at transplantation which usually does not exceed fifteen years of age. Therefore the need for renal replacement therapy (RRT) in adult patients with PBS is unusual. It is reasonable to suppose that the abdominal muscular defects may represent a limit for peritoneal dialysis (PD) utilization in PBS adult patients in many Kidney Units where, conversely, treatment with hemodialysis would be probably the easier choice. Here we describe the case of a 44 -year- old man with PBS who, at the age of 41, required RRT and was faced with the challenge of accepting PD. After more than three years of nocturnal automated peritoneal dialysis treatment we can safely say, as the following case illustrates, that PD is a feasible option in PBS adult patients.
authors.forename: Dario
authors.initials: D
authors.lastname: Musone
authors.forename: Valentina
authors.initials: V
authors.lastname: Nicosia
authors.forename: Riccardo
authors.initials: R
authors.lastname: D'Alessandro
authors.forename: Antonio
authors.initials: A
authors.lastname: Treglia
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Saltarelli
authors.forename: Maurizio
authors.initials: M
authors.lastname: Montella
authors.forename: Alessandro
authors.initials: A
authors.lastname: Sparagna
authors.forename: Francesco
authors.initials: F
authors.lastname: Amoroso
mesh_terms: D000328:Adult; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications* / Q000628:therapy*; D008297:Male; D010530:Peritoneal Dialysis; D011535:Prune Belly Syndrome / Q000150:complications*