Prognostic factors for long-term renal function in boys with the prune-belly syndrome.
OBJECTIVE
Renal failure develops in 25 to 30% of patients with the prune-belly syndrome. The clinical parameters associated with renal failure in this condition are not well understood. We determined which factors predict renal failure in children with the prune-belly syndrome.


METHODS
We reviewed the records of 35 patients with the prune-belly syndrome who were treated at our hospital during a 37-year period. Nadir serum creatinine, time to nadir creatinine, presence or absence of a patent urachus, presence and severity of urinary tract infection, and renal failure were analyzed.


RESULTS
Mean followup was 14 years 2 months. In 2 of the 17 patients with a nadir serum creatinine of less than 0.7 mg./dl. renal failure developed. Each of these 2 children also had clinical pyelonephritis and urosepsis. In 12 of the 13 patients nadir serum creatinine greater than 0.7 mg./dl. strongly predicted renal failure. Nonfebrile urinary tract infection within the first year of life was not predictive of outcome (p = 0.8) but a history of clinical pyelonephritis correlated with eventual renal failure (p<0.001). Urosepsis also correlated with renal failure (p = 0.008). Children with at least 1 normal kidney on renal ultrasound or renal scan had a significantly lower chance of renal failure (p<0.001).


CONCLUSIONS
Bilaterally abnormal kidneys on ultrasound or renal scan, a nadir serum creatinine of greater than 0.7 mg./dl. and clinical pyelonephritis are prognostic for renal failure.
Noh
P H
PH
Division of Urology, Children's Hospital of Philadelphia, Pennsylvania, USA.
Cooper
C S
CS
Winkler
A C
AC
Zderic
S A
SA
Snyder
H M
HM
Canning
D A
DA
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000503:physiopathology*; D008297:Male; D011379:Prognosis; D011535:Prune Belly Syndrome / Q000150:complications* / Q000503:physiopathology*; D051437:Renal Insufficiency / Q000209:etiology*; D013997:Time Factors