Contemporary epidemiology and characterization of newborn males with prune belly syndrome.

摘要: Objectives Prune belly syndrome (PBS) is a rare condition characterized by the congenital absence or deficiency of abdominal wall musculature, with associated abnormalities genitourinary tract, including hydronephrosis and cryptorchidism. Few population-based epidemiology mortality data are available. Methods We retrospectively reviewed Kids' Inpatient Database to evaluate PBS among newborn infants during their initial hospitalization in 2000, 2003, 2006. The International Classification Diseases, Ninth Revision, Clinical Modification codes were used identify patients determine comorbidity status. incidence, demographics, comorbid conditions, disposition assessed. Results A total 133 male diagnosed identified 1 420 991 live births, for weighted incidence estimate 3.8 cases/100 000 births. Of newborns PBS, 50% white, 31% black, 10% Hispanic. In-hospital was high (39 133, 29%). 55 (41%) discharged home 39 (29%) required inpatient transfer nursing care. Fifty-seven (43%) born premature; 56% deaths occurred premature infants. Mechanical ventilation 64 (48%), 33 (24%) had coexisting cardiovascular anomalies. Renal failure uncommon, occurring only 5 (4%); none dialysis. Only 13 (10%) underwent urinary diversion (vesicostomy ureterostomy). Conclusions Despite advances care children this continues be perinatal mortality, likely related prematurity pulmonary complications. rare, as immediate diversion.