title: The prevalence of late onset congenital adrenal hyperplasia in hirsute women from Central Anatolia.
abstract: Late onset congenital adrenal hyperplasia (LO CAH) can be seen in association with polycystic ovary syndrome (PCOS) or idiopathic hirsutism (IH). The study aimed to find out the prevalence of LO CAH in Central Anatolia among hirsute women. Sixty-three patients with hirsutism were evaluated to determine the frequency of LO CAH by comparing them with their age and body mass index matched 28 healthy controls. Of those 63 hirsute women, 43 were diagnosed as PCOS, and 20 were diagnosed as IH. Following basal hormonal evaluation, all subjects underwent ACTH stimulation test and ACTH stimulated 17-hydroxyprogesterone (17-OH P), 11-desoxycortisol (11-DOC), cortisol (F), and dehydroepiandrosterone sulfate (DHEA-S) levels were determined in all subjects. ACTH stimulated 17-OH P, 11-DOC, and DHEA-S levels did not differ between groups. However, stimulated F levels were found to be higher in hirsute women (p<0.001). Six out of 63 (9.52%) patients with hirsutism met the criterion for 21 hydroxylase deficiency. We found no subject presumed to have 11-beta hydroxylase deficiency, but one subject in control group (3.57%) and two patients among PCOS subjects (4.65%) had exaggerated DHEA-S response which was suggestive of mild 3-beta hydroxysteroid dehydrogenase deficiency. In conclusion, the most frequent form of LO CAH seems to be due to 21 OH deficiency among women with PCOS and IH in Central Anatolia. Mild 3-beta HSD deficiency may also be an underlying cause for hirsutism and it may be seen without any clinical presentation. Adrenal hyperactivity is likely to be the main reason of hyperandrogenemia in women with hirsutism.
authors.affiliation: Department of Endocrinology and Metabolic Diseases, Ankara University, School of Medicine, Ankara, Turkey.
authors.forename: Nuri
authors.initials: N
authors.lastname: Kamel
authors.forename: Vedia
authors.initials: V
authors.lastname: Tonyukuk
authors.forename: Rifat
authors.initials: R
authors.lastname: Emral
authors.forename: Demet
authors.initials: D
authors.lastname: Corapçioğlu
authors.forename: Mehmet
authors.initials: M
authors.lastname: Baştemir
authors.forename: Sevim
authors.initials: S
authors.lastname: Güllü
mesh_terms: D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology*; D000324:Adrenocorticotropic Hormone; D017677:Age Distribution; D017668:Age of Onset; D015992:Body Mass Index; D016022:Case-Control Studies; D003350:Cortodoxone / Q000097:blood; D019314:Dehydroepiandrosterone Sulfate / Q000097:blood; D005260:Female; D006628:Hirsutism / Q000097:blood / Q000150:complications*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D011085:Polycystic Ovary Syndrome / Q000150:complications; D015995:Prevalence; D013255:Steroid 21-Hydroxylase / Q000097:blood; D014421:Turkey / Q000453:epidemiology
chemical_list: D019314:Dehydroepiandrosterone Sulfate; D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D013255:Steroid 21-Hydroxylase; D003350:Cortodoxone; D006854:Hydrocortisone