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.
Farkas
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Department of Urology, Shaare Zedek Medical Center, Jerusalem, Israel.
Rosler
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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