Prevalence of nonclassic adrenal hyperplasia (NCAH) in hyperandrogenic women. 2008

Michael Fanta, and David Cibula, and Jana Vrbíková
Dept. of Gynecology and Obstetrics, 1st Medical Faculty of Charles University and General Faculty Hospital, Praha 2, Czech Republic. michael.fanta@volny.cz

OBJECTIVE The clinical symptoms of nonclassic adrenal hyperplasia (NCAH) are identical with polycystic ovary syndrome (PCOS). The aim of our study was to determine the prevalence of nonclassic adrenal hyperplasia (21-hydroxylase-deficiency) in hyperandrogenic women, its biochemical, endocrine and clinical characteristics and to compare them with parameters of patients with ovarian hyperandrogenism. METHODS Since 1999, 298 patients with elevation of at least one androgen and manifestation of one of the clinical androgenic symptoms (oligo/amenorrhea, hirsutism or acne) have been identified in our database. A diagnosis of NCAH was considered when the basal or stimulated 17-hydroxyprogesterone was elevated. RESULTS Only eight patients were identified as having 21- hydroxylase deficient NCAH in the whole group of 298 hyperandrogenic women. Hirsutism and acne were found only in three, two patients, five of them had oligo/amenorrhea. Seven patients had both elevated basal and stimulated 17-hydroxyprogesterone, while in one case only elevation of stimulated level was found. All of the NCAH patients had elevated concentrations of testosterone, six DHEA, lower SHBG was found in four patients. Surprisingly, none of the NCAH patients had increased DHEAS. CONCLUSIONS In our study, the prevalence of NCAH in hyperandrogenic women was 2.68%. Their leading symptom was oligomenorrhea, skin androgenic disorders were a minor clinical problem. None of the NCAH patients had an elevated DHEAS, the androgen dominantly produced by the adrenal glands.

UI MeSH Term Description Entries
D011085 Polycystic Ovary Syndrome A complex disorder characterized by infertility, HIRSUTISM; OBESITY; and various menstrual disturbances such as OLIGOMENORRHEA; AMENORRHEA; ANOVULATION. Polycystic ovary syndrome is usually associated with bilateral enlarged ovaries studded with atretic follicles, not with cysts. The term, polycystic ovary, is misleading. Stein-Leventhal Syndrome,Polycystic Ovarian Syndrome,Polycystic Ovary Syndrome 1,Sclerocystic Ovarian Degeneration,Sclerocystic Ovaries,Sclerocystic Ovary Syndrome,Ovarian Degeneration, Sclerocystic,Ovarian Syndrome, Polycystic,Ovary Syndrome, Polycystic,Ovary, Sclerocystic,Sclerocystic Ovary,Stein Leventhal Syndrome,Syndrome, Polycystic Ovary,Syndrome, Stein-Leventhal
D003687 Dehydroepiandrosterone A major C19 steroid produced by the ADRENAL CORTEX. It is also produced in small quantities in the TESTIS and the OVARY. Dehydroepiandrosterone (DHEA) can be converted to TESTOSTERONE; ANDROSTENEDIONE; ESTRADIOL; and ESTRONE. Most of DHEA is sulfated (DEHYDROEPIANDROSTERONE SULFATE) before secretion. Dehydroisoandrosterone,Prasterone,5-Androsten-3-beta-hydroxy-17-one,5-Androsten-3-ol-17-one,Androstenolone,DHEA,Prasterone, 3 alpha-Isomer,5 Androsten 3 beta hydroxy 17 one,5 Androsten 3 ol 17 one,Prasterone, 3 alpha Isomer
D005260 Female Females
D006628 Hirsutism A condition observed in WOMEN and CHILDREN when there is excess coarse body hair of an adult male distribution pattern, such as facial and chest areas. It is the result of elevated ANDROGENS from the OVARIES, the ADRENAL GLANDS, or exogenous sources. The concept does not include HYPERTRICHOSIS, which is an androgen-independent excessive hair growth.
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000152 Acne Vulgaris A chronic disorder of the pilosebaceous apparatus associated with an increase in sebum secretion. It is characterized by open comedones (blackheads), closed comedones (whiteheads), and pustular nodules. The cause is unknown, but heredity and age are predisposing factors. Acne
D000312 Adrenal Hyperplasia, Congenital A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders. Congenital Adrenal Hyperplasia,Hyperplasia, Congenital Adrenal,Adrenal Hyperplasias, Congenital,Congenital Adrenal Hyperplasias,Hyperplasias, Congenital Adrenal
D000324 Adrenocorticotropic Hormone An anterior pituitary hormone that stimulates the ADRENAL CORTEX and its production of CORTICOSTEROIDS. ACTH is a 39-amino acid polypeptide of which the N-terminal 24-amino acid segment is identical in all species and contains the adrenocorticotrophic activity. Upon further tissue-specific processing, ACTH can yield ALPHA-MSH and corticotrophin-like intermediate lobe peptide (CLIP). ACTH,Adrenocorticotropin,Corticotropin,1-39 ACTH,ACTH (1-39),Adrenocorticotrophic Hormone,Corticotrophin,Corticotrophin (1-39),Corticotropin (1-39),Hormone, Adrenocorticotrophic,Hormone, Adrenocorticotropic
D012738 Sex Hormone-Binding Globulin A glycoprotein migrating as a beta-globulin. Its molecular weight, 52,000 or 95,000-115,000, indicates that it exists as a dimer. The protein binds testosterone, dihydrotestosterone, and estradiol in the plasma. Sex hormone-binding protein has the same amino acid sequence as ANDROGEN-BINDING PROTEIN. They differ by their sites of synthesis and post-translational oligosaccharide modifications. Sex Steroid-Binding Protein,Testosterone-Estradiol Binding Globulin,Binding Globulin, Testosterone-Estradiol,Globulin, Sex Hormone-Binding,Globulin, Testosterone-Estradiol Binding,Hormone-Binding Globulin, Sex,Sex Hormone Binding Globulin,Sex Steroid Binding Protein,Steroid-Binding Protein, Sex,Testosterone Estradiol Binding Globulin
D013739 Testosterone A potent androgenic steroid and major product secreted by the LEYDIG CELLS of the TESTIS. Its production is stimulated by LUTEINIZING HORMONE from the PITUITARY GLAND. In turn, testosterone exerts feedback control of the pituitary LH and FSH secretion. Depending on the tissues, testosterone can be further converted to DIHYDROTESTOSTERONE or ESTRADIOL. 17-beta-Hydroxy-4-Androsten-3-one,17-beta-Hydroxy-8 alpha-4-Androsten-3-one,8-Isotestosterone,AndroGel,Androderm,Andropatch,Androtop,Histerone,Sterotate,Sustanon,Testim,Testoderm,Testolin,Testopel,Testosterone Sulfate,17 beta Hydroxy 4 Androsten 3 one,17 beta Hydroxy 8 alpha 4 Androsten 3 one,8 Isotestosterone

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