Ten years experience with masculinizing genitoplasty in male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase deficiency.

Abstract

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.

• Authors: A Farkas, A Rosler
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 152 Suppl 2 Pg. S88-90 ( 1993) ISSN: 0340-6199 [Print] Germany
• PMID: 8393406 (Publication Type: Journal Article)

Chemical References

• Testosterone
• 17-Hydroxysteroid Dehydrogenases

Topics

• 17-Hydroxysteroid Dehydrogenases (deficiency)
• Adolescent
• Adult
• Child, Preschool
• Combined Modality Therapy
• Disorders of Sex Development (drug therapy, metabolism, surgery)
• Follow-Up Studies
• Humans
• Infant
• Male
• Reoperation
• Surgery, Plastic (methods)
• Surgical Procedures, Operative (methods)
• Testosterone (therapeutic use)