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The prune belly syndrome of marked abdominal muscle deficiency and associated genitourinary anomalies has been found almost exclusively in male subjects. Of the few reported cases of female patients with this entity most are questionable or incomplete forms. The light microscopic histology and electron microscopic ureteral abnormalities in male subjects have been reported previously. A female newborn with marked abdominal muscle deficiency and severe bilateral ureteropyelocaliectasis is described. Chromosomal studies showed 46 XX karyotype. Histologic examination of the ureter by light microscopy was compatible with the diagnosis.

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