Prune-belly syndrome
Prune-belly syndrome, also known as Eagle-Barret syndrome, is a congenital anomaly comprising three clinical findings: deficient abdominal musculature, urinary tract anomalies, and bilateral cryptorchidism. Other clinical findings involving respiratory, skeletal, digestion and cardiovascular system...
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| Main Authors: | , , |
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| Format: | Book |
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Indonesian Pediatric Society Publishing House,
2009-10-01T00:00:00Z.
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| Summary: | Prune-belly syndrome, also known as Eagle-Barret syndrome, is a congenital anomaly comprising three clinical findings: deficient abdominal musculature, urinary tract anomalies, and bilateral cryptorchidism. Other clinical findings involving respiratory, skeletal, digestion and cardiovascular system may also accompany the syndrome. The incidence is approximately 1 : 30,000 to 40,000 live births and 95% of cases occur in boys. Pulmonary hypoplasia and kidney failure are important prognostic factors that contribute to 60% of mortality rate. Treatment includes surgical correction of the abdominal wall and urinary tract, orchidopexy and other supportive managements.l-4 We report 4 cases on typical Prune-belly syndrome, together with other clinical variants. |
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| Item Description: | 0030-9311 2338-476X 10.14238/pi49.5.2009.304-8 |