title: Renal transplantation in prune-belly syndrome.
abstract: We assess the effect of the prune-belly syndrome (PBS) on renal transplantation outcome. Six renal transplantations were performed in five boys affected by PBS (median age 5.8+/-2.1 years, median weight 13.6+/-2.4kg). Renal graft survival, graft function, lower urinary tract dysfunction, urinary tract infections (UTIs), associated complications and patients' survival after 1 and 5 years of follow-up were analysed. The rate for 1-5-year graft survival was 66.7% (mean serum creatinine 98-103 micromol/l). The surgical treatment of the documented bladder obstruction (two patients) and the severe abdominal wall deficit (one patient) led to a reduction of UTI: the patients maintained their native urinary tract and none received prophylactic antibiotics. The lack of abdominal wall musculature led to severe mechanical complication in one patient, but Monfort's abdominal wall reconstruction was able to restore the graft's function. The outcome of patients with PBS who undergo renal transplantation is good. Before the transplant, an accurate assessment of urinary tract anomalies and deficiency of the abdominal wall musculature is mandatory, to program the appropriate treatment and obtain a good long-term prognosis for the renal graft.
authors.affiliation: Division of Pediatric Surgery, University of Padova, via Giustiniani 3, 35128, Padova, Italy. fabiofusaro@libero.it
authors.forename: Fabio
authors.initials: F
authors.lastname: Fusaro
authors.forename: Giovanni F
authors.initials: GF
authors.lastname: Zanon
authors.forename: Anna M
authors.initials: AM
authors.lastname: Ferreli
authors.forename: Stefano
authors.initials: S
authors.lastname: Giuliani
authors.forename: Graziella
authors.initials: G
authors.lastname: Zacchello
authors.forename: Giacomo
authors.initials: G
authors.lastname: Passerini-Glazel
authors.forename: Waifro
authors.initials: W
authors.lastname: Rigamonti
mesh_terms: D034861:Abdominal Wall / Q000002:abnormalities / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006085:Graft Survival; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D011535:Prune Belly Syndrome / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome; D001748:Urinary Bladder Neck Obstruction / Q000150:complications / Q000601:surgery; D014551:Urinary Tract / Q000503:physiopathology; D014552:Urinary Tract Infections / Q000209:etiology / Q000517:prevention & control