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Kusuma P. A
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Health Professions Medicine & Pharmacology

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Prune-belly syndrome Yenny Yenny; Kusuma P. A; Damanik M. P.
Paediatrica Indonesiana Vol 49 No 5 (2009): September 2009
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (129.485 KB) | DOI: 10.14238/pi49.5.2009.304-8

Abstract

Prune-belly syndrome, also known as Eagle-Barretsyndrome, is a congenital anomaly comprisingthree clinical findings: deficient abdominalmusculature, urinary tract anomalies, andbilateral cryptorchidism. Other clinical findings involvingrespiratory, skeletal, digestion and cardiovascular systemmay also accompany the syndrome. The incidence isapproximately 1 : 30,000 to 40,000 live births and 95%of cases occur in boys. Pulmonary hypoplasia and kidneyfailure are important prognostic factors that contributeto 60% of mortality rate. Treatment includes surgicalcorrection of the abdominal wall and urinary tract,orchidopexy and other supportive managements.l-4 Wereport 4 cases on typical Prune-belly syndrome, togetherwith other clinical variants.
Co-Authors Damanik M. P. Yenny Yenny