Ten years experience with masculinizing genitoplasty in male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase deficiency.
An extensive survey in a highly inbred Arab community documented over 60 cases of male pseudohermaphroditism due to 17 beta hydroxysteroid dehydrogenase deficiency. During a period of 10 years (1981-1991), 16 such patients underwent masculinating genitoplasty. Two children have had a one stage corrective genitoplasty while all others (2 adults, 3 adolescents and 9 children) had staged operations. Cosmetic and functional results are very encouraging in children and adolescents but very poor among adults. Based on our experience and follow-up, we advocate early sex assignment and early masculinizing genitoplasty with preoperative hormonal therapy to male pseudohermaphrodites due to 17 beta hydroxysteroid dehydrogenase deficiency.
Department of Urology, Shaare Zedek Medical Center, Jerusalem, Israel.
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Farkas
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Rosler
D015067:17-Hydroxysteroid Dehydrogenases / Q000172:deficiency*; D000293:Adolescent; D000328:Adult; D002675:Child, Preschool; D003131:Combined Modality Therapy; D012734:Disorders of Sex Development / Q000188:drug therapy / Q000378:metabolism / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012086:Reoperation; D013518:Surgery, Plastic / Q000379:methods; D013514:Surgical Procedures, Operative / Q000379:methods; D013739:Testosterone / Q000627:therapeutic use
D013739:Testosterone; D015067:17-Hydroxysteroid Dehydrogenases