A broader spectrum of abnormalities in the prune belly syndrome.
The clinical course of 25 children with the prune belly syndrome was reviewed retrospectively to assess the over-all morbidity associated with this disorder. There were 3 neonatal deaths of renal or pulmonary disease. Chronic renal insufficiency or end stage renal disease developed in 5 survivors, all of whom had impaired kidney function in early infancy. An additional 17 patients survived with only mild renal insufficiency. Growth retardation, which correlated poorly with renal function, was present in a third of the patients. Clinically significant pulmonary and orthopedic problems were noted in 55 per cent of the survivors. Chronic constipation was another common, although less serious, feature. This report emphasizes the severity of the extrarenal problems associated with the prune belly syndrome.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005767:Gastrointestinal Diseases / Q000151:congenital / Q000175:diagnosis; D006130:Growth Disorders / Q000151:congenital / Q000175:diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000151:congenital / Q000175:diagnosis / Q000401:mortality; D007866:Leg / Q000002:abnormalities; D008297:Male; D011535:Prune Belly Syndrome / Q000175:diagnosis* / Q000401:mortality; D012120:Respiration Disorders / Q000151:congenital / Q000175:diagnosis / Q000401:mortality; D012189:Retrospective Studies