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FEMALE PSEUDOHERMAPHRODITISM DUE TO ADRENOGENITAL SYNDROME
Journal of the Korean Society of Plastic and Reconstructive Surgeons ; : 186-198, 1997.
Artigo em Coreano | WPRIM | ID: wpr-80250
ABSTRACT
Female pseudohermaphroditism due to adrenogenital syndrome is a condition in which individuals with a 46XX karyotype, negative H-Y antigen, normal mullerian duct derivatives, and a lack of development of w lffian duct structures differentiate partially as phenotypic males. They usually manifest masculinization of the external genitalia as a result of excess endogenous androgens. Most female pseudohermaphrodities have one of the types of congenital virilizing adrenal hyperplasia. Adrenogenital syndrome is inborn errors transmitted by autosomal recessive genes and may be due to defects in any of the enzymic steps in the biosynthesis of cortisol. Most affected individuals have a failure of 21-hydroxylation which prevents the conversion of 17 alpha-hydroxyprogesterone to 11-deoxycortisol. Such a defect in 21-hydroxylase leads to excessive production of adrenal androgens causing virilization. The treatment is early endocrinologic support and surgical reconstruction. There are some considerations in surgical repairs including normal sized clitoris with adequate erogenous sensation, sufficiently wide vaginal introitus and normal aesthetic appearance of the external genitalia for her normalized life as a female. We have experienced four cases of female pseudohermaphroditism due to adrenogenital syndrome. In two cases, we performed clitoroplasty with nerve sparing technique, vulvoplasty with mons pubis augmentation, vaginoplasty with posterior perineal flap and urethral reconstruction. In the other cases, we performed clitoroplasty with nerve sparing technique, vulvoplasty and vaginoplasty There was no specific operative complication and the result of the correction was satisfactory.
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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Sensação / Virilismo / Hidrocortisona / Esteroide 21-Hidroxilase / Antígeno H-Y / Clitóris / 17-alfa-Hidroxiprogesterona / Cortodoxona / Síndrome Adrenogenital / Transtornos 46, XX do Desenvolvimento Sexual Limite: Feminino / Humanos / Masculino Idioma: Coreano Revista: Journal of the Korean Society of Plastic and Reconstructive Surgeons Ano de publicação: 1997 Tipo de documento: Artigo

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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Sensação / Virilismo / Hidrocortisona / Esteroide 21-Hidroxilase / Antígeno H-Y / Clitóris / 17-alfa-Hidroxiprogesterona / Cortodoxona / Síndrome Adrenogenital / Transtornos 46, XX do Desenvolvimento Sexual Limite: Feminino / Humanos / Masculino Idioma: Coreano Revista: Journal of the Korean Society of Plastic and Reconstructive Surgeons Ano de publicação: 1997 Tipo de documento: Artigo