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

Publication ,  Journal Article
Routh, JC; Huang, L; Retik, AB; Nelson, CP
Published in: Urology
July 2010

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

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Published In

Urology

DOI

EISSN

1527-9995

Publication Date

July 2010

Volume

76

Issue

1

Start / End Page

44 / 48

Location

United States

Related Subject Headings

  • Urology & Nephrology
  • Retrospective Studies
  • Prune Belly Syndrome
  • Male
  • Infant, Newborn
  • Incidence
  • Humans
  • 3202 Clinical sciences
  • 1103 Clinical Sciences
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Routh, J. C., Huang, L., Retik, A. B., & Nelson, C. P. (2010). Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology, 76(1), 44–48. https://doi.org/10.1016/j.urology.2009.12.072
Routh, Jonathan C., Lin Huang, Alan B. Retik, and Caleb P. Nelson. “Contemporary epidemiology and characterization of newborn males with prune belly syndrome.Urology 76, no. 1 (July 2010): 44–48. https://doi.org/10.1016/j.urology.2009.12.072.
Routh JC, Huang L, Retik AB, Nelson CP. Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 2010 Jul;76(1):44–8.
Routh, Jonathan C., et al. “Contemporary epidemiology and characterization of newborn males with prune belly syndrome.Urology, vol. 76, no. 1, July 2010, pp. 44–48. Pubmed, doi:10.1016/j.urology.2009.12.072.
Routh JC, Huang L, Retik AB, Nelson CP. Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 2010 Jul;76(1):44–48.
Journal cover image

Published In

Urology

DOI

EISSN

1527-9995

Publication Date

July 2010

Volume

76

Issue

1

Start / End Page

44 / 48

Location

United States

Related Subject Headings

  • Urology & Nephrology
  • Retrospective Studies
  • Prune Belly Syndrome
  • Male
  • Infant, Newborn
  • Incidence
  • Humans
  • 3202 Clinical sciences
  • 1103 Clinical Sciences