Hypertensive crisis associated with renal cell carcinoma.
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Biomedical subjects
Publications and source records attributed to H R Reeve.
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This is the fifth family in the literature with siblings having the prune belly syndrome. It is the first with associated pulmonary stenosis, mental retardation, and hearing deficit. A genetic factor is suggested as the primary cause of the syndrome.
Two cases of megalourethra are presented, the ninth and tenth cases to be documented in the literature. Both patients exhibited mesodermal abnormalities which justify categorization among the minor forms of prune belly syndrome. One patient, the youngest to have undergone surgical correction, presented with azotemia, dilated posterior urethra, megacystis, and megaureters. The second patient with incomplete or scaphoid form of megalourethra also exhibited undescended testis, corrected by orchiopexy at the time of urethoplasty. In all instances the goal of treatment is preservation of renal function with subsequent functional and anatomic reconstruction of the urinary tract.