[Partial deficiency in 21-hydroxylase in certain forms of hirsutism].
The ACTH test is important when hirsutism occurs in women with a slight 21-hydroxylase deficiency, and normal basal 17-OH Progesterone (17-OH-P/plasma levels). Extensive hormonal assays: LH, FSH, Prolactin, 17 beta-estradiol (E2), Estrone, 17OH-P, Androstenedione, Testosterone, Cortisol (C), Dehydroepiandrosterone-S (DEA-S) were carried out in 36 hirsute women. 13 of these presented hormone levels as found in polycystic ovary syndrome (PCOS), 6 women presented a slight 21-hydroxylase deficiency (increased plasma 17-OH-P and decreased C after ACTH test with significant, p less than 0.01, increase of 17-OH-P/C and 17 women presented idiopathic hirsutism (IH). The hormonal pattern, in the basal condition, is not different in IH or in slight 21-hydroxylase deficiency. The ACTH test is able to differentiate between IH and adrenal hirsutism.
Lucisano
A
A
Istituto di Clinica Ostetrica e Ginecologia U. C. S. C., Roma.
Satta
M A
MA
Tripodi
R
R
Mobili
L
L
De Cicco
F
F
Monaco
F
F
Dell'Acqua
S
S
Roche
J
J
D019326:17-alpha-Hydroxyprogesterone; D000312:Adrenal Hyperplasia, Congenital; D000324:Adrenocorticotropic Hormone; D000328:Adult; D000728:Androgens / Q000097:blood; D004967:Estrogens / Q000097:blood; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D006628:Hirsutism / Q000201:enzymology*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D006908:Hydroxyprogesterones / Q000097:blood; D007986:Luteinizing Hormone / Q000097:blood; D011085:Polycystic Ovary Syndrome / Q000097:blood; D013250:Steroid Hydroxylases / Q000172:deficiency*
D000728:Androgens; D004967:Estrogens; D006908:Hydroxyprogesterones; D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D013250:Steroid Hydroxylases; D006854:Hydrocortisone
A propos du déficit partiel en 21-hydroxylase dans certaines formes d'hirsutisme.