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.
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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)
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