Testicular feminization syndrome: current clinical considerations.
The testicular feminization syndrome (TFS) in its complete form results in total feminization due to a nuclear inaction of androgens, and the female role should be supported with postpubertal orchiectomy to avoid the risk of malignancy. Incomplete forms of the syndrome (ITFS) include Type I n which some degree of masculinization may be observed, prompting earlier gonadectomy, and Type II or pseudovaginal perineoscrotal hypospadias (PPSH) which is always characterized by pubertal masculinization, necessitating management and support of these patients as males. Other intersex abnormalities which must be differentiated include true hermaphroditism, the Swyer syndrome, males with 17-ketosteroid reductase deficiency, and Reifenstein's syndrome.
D015068:17-Ketosteroids; D000293:Adolescent; D000328:Adult; D013734:Androgen-Insensitivity Syndrome / Q000145:classification / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D000728:Androgens / Q000096:biosynthesis; D000818:Animals; D002648:Child; D003937:Diagnosis, Differential; D012734:Disorders of Sex Development / Q000175:diagnosis / Q000235:genetics; D005260:Female; D005640:Follicle Stimulating Hormone / Q000652:urine; D006801:Humans; D006913:Hydroxysteroid Dehydrogenases / Q000172:deficiency; D007021:Hypospadias / Q000175:diagnosis; D007248:Infertility, Male / Q000175:diagnosis; D008297:Male; D051379:Mice; D051381:Rats
D015068:17-Ketosteroids; D000728:Androgens; D005640:Follicle Stimulating Hormone; D006913:Hydroxysteroid Dehydrogenases