[Effect of prepuberal gonadectomy in partial androgen resistance syndrome].
The androgen resistance syndrome, is a dominant and recessive linked to X disorder, with clinically heterogenous manifestations. Reported case. A 17-years patient with primary amenorrhoea and genital ambiguity. Bilateral inguinal herniplastia and probable gonadectomy were performed at 14-months-old. His cariotipe was male 46,XY and the hormonal determinations showed the presence of hypergonadotropic hypogonadismo. The congenital adrenal hyperplasia (deficiency of 21-hidroxilase) was discarded. In the partial androgen resistance syndrome, the prepuberal gonadectomy avoid a progresive virilization of the external genitals. However, the estrogenic hormonal replacement is mandatory.
Garcia Morales
M A
MA
Médicos Adscritos del Departamento de Ginecología y Obstetrica.
Barron Vallejo
J
J
Limon Luque
L
L
D000293:Adolescent; D000367:Age Factors; D012734:Disorders of Sex Development / Q000378:metabolism / Q000503:physiopathology*; D015914:Estrogen Replacement Therapy; D005260:Female; D006066:Gonads / Q000601:surgery; D006801:Humans; D007006:Hypogonadism / Q000209:etiology; D007223:Infant; D007621:Karyotyping; D008297:Male; D011944:Receptors, Androgen; D013577:Syndrome; D014960:X Chromosome; D014998:Y Chromosome
D011944:Receptors, Androgen
Efecto de la gonadectomía prepuberal en el Síndrome de Resistencia Parcial a los Andrógenos.