Etiology and pathogenesis of the prune belly syndrome

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Abstract

The hypothesis explaining the characteristics of the prune belly syndrome (congenital partial absense of the abdominal wall musculature, severe urinary tract abnormalities, and cryptorchidism) as sequelae of a primary obstructive uropathy is supported neither by evidence from the literature nor by our own observations of 12 males and 2 females. Critical analysis of the known facts favors the concept of a developmental field complex in which the various manifestations of the prune belly syndrome are produced by an early mesodermal defect.

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Straub, E., & Spranger, J. (1981). Etiology and pathogenesis of the prune belly syndrome. Kidney International, 20(6), 695–699. https://doi.org/10.1038/ki.1981.198

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