cortodoxone and Polycystic-Ovary-Syndrome

To determine if the (tttta)(n) repeat polymorphism in the promoter region of CYP11a gene is associated with hirsutism and hyperandrogenism in women from Spain.. Controlled clinical study.. Tertiary-care institutional hospital.. Ninety-two hirsute women and 33 healthy control women.. Basal and adrenocorticotropin-stimulated serum samples and genomic DNA extracted and purified from whole-blood samples were obtained during the follicular phase of the menstrual cycle.. CYP11a (tttta)(n) repeat-polymorphism genotype and serum ovarian and adrenal androgen levels.. None of the CYP11a (tttta)(n) polymorphic alleles was associated with hirsutism. The absence of the four-repeat-units allele (4R-- genotype), which has been reported by other authors to be associated with polycystic ovary syndrome (PCOS), was found in 22.4% of the women studied here and was equally distributed among patients and controls, independently of the presence of PCOS and/or ovarian or adrenal hyperandrogenism. No differences were observed in serum hormone concentrations in 4R-- individuals as compared with subjects with at least one four-repeat-units allele.. The (tttta)(n) repeat polymorphism in the promoter region of CYP11a does not appear to play any significant role in the pathogenesis of hirsutism and hyperandrogenism in women from Spain.

Topics: Adult; Base Sequence; Cholesterol Side-Chain Cleavage Enzyme; Cortodoxone; Cosyntropin; Dehydroepiandrosterone Sulfate; Dexamethasone; Estradiol; Female; Follicle Stimulating Hormone; Genotype; Hirsutism; Humans; Hyperandrogenism; Luteinizing Hormone; Menstrual Cycle; Microsatellite Repeats; Polycystic Ovary Syndrome; Polymorphism, Genetic; Progesterone; Promoter Regions, Genetic; Reference Values; Sex Hormone-Binding Globulin; Testosterone

Do naturally occurring, hyperandrogenic (≥1 SD of population mean testosterone, T) female rhesus monkeys exhibit traits typical of women with polycystic ovary syndrome (PCOS)?. Hyperandrogenic female monkeys exhibited significantly increased serum levels of androstenedione (A4), 17-hydroxyprogesterone (17-OHP), estradiol (E2), LH, antimullerian hormone (AMH), cortisol, 11-deoxycortisol and corticosterone, as well as increased uterine endometrial thickness and evidence of reduced fertility, all traits associated with PCOS.. Progress in treating women with PCOS is limited by incomplete knowledge of its pathogenesis and the absence of naturally occurring PCOS in animal models. A female macaque monkey, however, with naturally occurring hyperandrogenism, anovulation and polyfollicular ovaries, accompanied by insulin resistance, increased adiposity and endometrial hyperplasia, suggests naturally occurring origins for PCOS in nonhuman primates.. As part of a larger study, circulating serum concentrations of selected pituitary, ovarian and adrenal hormones, together with fasted insulin and glucose levels, were determined in a single, morning blood sample obtained from 120 apparently healthy, ovary-intact, adult female rhesus monkeys (Macaca mulatta) while not pregnant or nursing. The monkeys were then sedated for somatometric and ultrasonographic measurements.. Female monkeys were of prime reproductive age (7.2 ± 0.1 years, mean ± SEM) and represented a typical spectrum of adult body weight (7.4 ± 0.2 kg; maximum 12.5, minimum 4.6 kg). Females were defined as having normal (n = 99) or high T levels (n = 21; ≥1 SD above the overall mean, 0.31 ng/ml). Electronic health records provided menstrual and fecundity histories. Steroid hormones were determined by tandem LC-MS-MS; AMH was measured by enzymeimmunoassay; LH, FSH and insulin were determined by radioimmunoassay; and glucose was read by glucose meter. Most analyses were limited to 80 females (60 normal T, 20 high T) in the follicular phase of a menstrual cycle or anovulatory period (serum progesterone <1 ng/ml).. Of 80 monkeys, 15% (n = 12) exhibited classifiable PCOS-like phenotypes. High T females demonstrated elevations in serum levels of LH (P < 0.036), AMH (P < 0.021), A4 (P < 0.0001), 17-OHP (P < 0.008), E2 (P < 0.023), glucocorticoids (P < 0.02-0.0001), the serum T/E2 ratio (P < 0.03) and uterine endometrial thickness (P < 0.014) compared to normal T females. Within the high T group alone, anogenital distance, a biomarker for fetal T exposure, positively correlated (P < 0.015) with serum A4 levels, while clitoral volume, a biomarker for prior T exposure, positively correlated (P < 0.002) with postnatal age. Only high T females demonstrated positive correlations between serum LH, and both T and A4. Five of six (83%) high T females with serum T ≥2 SD above T mean (0.41 ng/ml) did not produce live offspring.. N/A.. This is an initial study of a single laboratory population in a single nonhuman primate species. While two biomarkers suggest lifelong hyperandrogenism, phenotypic expression during gestation, prepuberty, adolescence, mid-to-late reproductive years and postmenopause has yet to be determined.. Characterizing adult female monkeys with naturally occurring hyperandrogenism has identified individuals with high LH and AMH combined with infertility, suggesting developmental linkage among traits with endemic origins beyond humans. PCOS may thus be an ancient phenotype, as previously proposed, with a definable pathogenic mechanism(s).. Funded by competitive supplement to P51 OD011106 (PI: Mallick), by P50 HD028934 (PI: Marshall) and by P50 HD044405 (PI: Dunaif). The authors have no potential conflicts of interest.

Topics: Androstenedione; Animals; Anti-Mullerian Hormone; Corticosterone; Cortodoxone; Endometrium; Estradiol; Female; Fertility; Hydrocortisone; Hydroxyprogesterones; Hyperandrogenism; Macaca mulatta; Phenotype; Polycystic Ovary Syndrome

Are there abnormalities in gonadotrophin secretion, adrenal steroidogenesis and/or testicular steroidogenesis in brothers of women with polycystic ovary syndrome (PCOS)?. Brothers of women with PCOS have increased gonadotrophin responses to gonadotrophin releasing hormone (GnRH) agonist stimulation and alterations in adrenal and gonadal steroidogenesis.. PCOS is a complex genetic disease. Male as well as female first-degree relatives have reproductive features of the syndrome. We previously reported that brothers of affected women have elevated circulating dehydroepiandrosterone sulfate levels.. This was a case-control study performed in 29 non-Hispanic white brothers of 22 women with PCOS and 18 control men.. PCOS brothers and control men were of comparable age, weight and ethnicity. Adrenocorticotrophic hormone (ACTH) and GnRH agonist stimulation tests were performed. Gonadotrophin responses to GnRH agonist as well as changes in precursor-product steroid pairs (delta, Δ) across steroidogenic pathways in response to ACTH and GnRH agonist were examined.. Basal total (T) levels did not differ, but dehydroepiandrosterone (DHEA) levels (0.13 ± 0.08 brothers versus 0.22 ± 0.09 controls, nmol/l, P = 0.03) were lower in brothers compared with control men. ACTH-stimulated Δ17-hydroxypregnenolone (17Preg)/Δ17-hydroxyprogesterone (17Prog) (7.8 ± 24.2 brothers versus 18.9 ± 21.3 controls, P = 0.04) and ΔDHEA/Δandrostenedione (AD) (0.10 ± 0.05 brothers versus 0.14 ± 0.08 controls, P = 0.04) were lower in brothers than in the controls. GnRH agonist-stimulated Δ17Prog/ΔAD (0.28 ± 8.47 brothers versus 4.79 ± 10.28 controls, P = 0.003) was decreased and luteinizing hormone (38.6 ± 20.6 brothers versus 26.0 ± 9.8 controls, IU/l, P = 0.02), follicle-stimulating hormone (10.2 ± 7.5 brothers versus 4.8 ± 4.1 controls, IU/l P = 0.002), AD (1.7 ± 1.4 brothers versus 0.9 ± 1.5 controls, nmol/l, P = 0.02) and ΔAD/ΔT (0.16 ± 0.14 brothers versus 0.08 ± 0.12 controls, P = 0.005) responses were increased in brothers compared with controls.. The modest sample size may have limited our ability to observe other possible differences in steroidogenesis between PCOS brothers and control men.. Decreased ACTH-stimulated Δ17Preg/Δ17Prog and ΔDHEA/ΔAD responses suggested increased adrenal 3β-hydroxysteroid dehydrogenase activity in the brothers. Decreased Δ17Prog/ΔAD and increased ΔAD/ΔT responses to GnRH agonist stimulation suggested increased gonadal 17,20-lyase and decreased gonadal 17β-hydroxysteroid dehydrogenase activity in the brothers. Increased LH and FSH responses to GnRH agonist stimulation suggested neuroendocrine alterations in the regulation of gonadotrophin secretion similar to those in their proband sisters. These changes in PCOS brothers may reflect the impact of PCOS susceptibility genes and/or programming effects of the intrauterine environment.. This research was supported by P50 HD044405 (A.D.), K12 HD055884 (L.C.T.), U54 HD034449 (A.D., R.S.L.) from the National Institute of Child Health and Development. Some hormone assays were performed at the University of Virginia Center for Research in Reproduction Ligand Assay and Analysis Core that is supported by U54 HD28934 from the Eunice Kennedy Shriver National Institute of Child Health and Human Development. Partial support for some of the clinical studies was provided by UL1 RR025741 and UL1 TR000150 (Northwestern University Clinical and Translational Sciences Institute) from the National Center for Research Resources, National Institutes of Health, which is now the National Center for Advancing Translational Sciences. The authors have no conflict of interest to declare.

Topics: 17-alpha-Hydroxypregnenolone; 17-alpha-Hydroxyprogesterone; Adolescent; Adult; Androstenedione; Case-Control Studies; Cortodoxone; Dehydroepiandrosterone Sulfate; Female; Gonadotropins; Humans; Male; Middle Aged; Polycystic Ovary Syndrome; Siblings; Steroids

Androgen excess carries varied clinical manifestations in women. Although testosterone and dehydroepiandrostendionesulfate (DHEAS) determination is considered useful in diagnostic workup, there is no laboratory definition that sufficiently describes androgen excess.. We studied 464 hirsute women with a Ferriman and Gallwey score of at least 8 between 2000 and 2005. Our examination included clinical data, total testosterone (T), sex hormone-binding globulin (SHBG), the free androgen index (FAI), and DHEAS. Additionally, androstendione, 17alpha-hydroxyprogesterone (17OHP), dehydroepiandrostendione (DHEA), and 11-deoxycortisol were determined at baseline and 60min after corticotropin challenge (250microg synacthen).. Of 464 women, 77.6% fulfilled the clinical criteria for hyperandrogenemia. Of these 360 women, 78.1% had hyperandrogenic hirsutism. Of these 281 women, 43.4% showed increased stimulation of 17OHP to 250microg of synacthen. Another 37.4% showed adrenal steroid biosynthesis defects other than 21alpha-hydroxylase deficiency, such as defective 11beta-hydroxylation or 3beta-hydroxysteroid dehydrogenase malfunction. The diagnosis of polycystic ovary syndrome was applicable to 12.4%. In addition, our results show that 72% of 281 patients with secondary hirsutism had normal T concentrations, and 55% had a normal FAI. Only 5% of hirsute patients with a normal FAI had elevated DHEAS values. However, 40% showed elevated DHEA levels, while 26% of the women with normal FAI showed androstendione values over the maximal levels in the 79 controls.. Our data suggest that in addition to testosterone and FAI, androstendione and DHEA are significantly helpful parameters in diagnosing hyperandrogenemia in hirsute women. DHEAS was not found to be helpful.

Topics: 17-alpha-Hydroxyprogesterone; Adolescent; Adult; Aged; Androstenedione; Biomarkers; Cortodoxone; Dehydroepiandrosterone; Female; Follicle Stimulating Hormone; Hirsutism; Humans; Hyperandrogenism; Insulin-Like Growth Factor I; Luteinizing Hormone; Middle Aged; Polycystic Ovary Syndrome; Radioimmunoassay; Steroid 21-Hydroxylase

The diagnosis of the polycystic ovary syndrome requires the exclusion of nonclassical congenital adrenal hyperplasia (NCAH).. Our objective was to evaluate the actual prevalences of 21-hydroxylase and 11beta-hydroxylase deficiencies among women presenting with hyperandrogenic complaints.. This study was performed at an academic hospital.. A total of 270 consecutive unselected women presenting with hyperandrogenic symptoms were prospectively recruited.. Basal and ACTH-stimulated 11-deoxycortisol and 17-hydroxyprogesterone concentrations were measured.. The prevalences of 21-hydroxylase and 11beta-hydroxylase deficiencies were calculated, and the diagnostic performance of basal serum 17-hydroxyprogesterone levels for the screening of NCAH was evaluated by receiver operating characteristic curve analysis.. Six of the 270 patients had 21-hydroxylase-deficient NCAH that was confirmed by CYP21 genotyping, whereas no patient was diagnosed with 11beta-hydroxylase deficiency, for an overall NCAH prevalence of 2.2% (95% confidence limits 0.5-3.9%). According to receiver operating characteristic analysis, a single basal serum 17-hydroxyprogesterone determination has a 0.97 (95% confidence interval: 0.934-1.008) chance of detecting NCAH in hyperandrogenic women. In our experience, the most appropriate cutoff value for the detection of NCAH is a 17-hydroxyprogesterone above 1.7 ng/ml, showing a 100% sensitivity and a 88.6% specificity. Five of the six 21-hydroxylase-deficient NCAH patients carried a severe CYP21 allele requiring genetic counseling and highlighting the importance of excluding this disorder among hyperandrogenic patients.. The prevalence of NCAH among hyperandrogenic patients from Spain is 2.2%. Basal serum 17-hydroxyprogesterone measurements have an excellent diagnostic performance, yet the cutoff value should be established in each laboratory to avoid false-negative results.

Topics: 17-alpha-Hydroxyprogesterone; Adolescent; Adrenal Hyperplasia, Congenital; Adult; Cortodoxone; Diagnosis, Differential; Female; Humans; Hyperandrogenism; Polycystic Ovary Syndrome; Prevalence; Prospective Studies; ROC Curve; Sensitivity and Specificity; Spain; Steroid 11-beta-Hydroxylase; Steroid 21-Hydroxylase

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.

Topics: 17-alpha-Hydroxyprogesterone; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Age Distribution; Age of Onset; Body Mass Index; Case-Control Studies; Cortodoxone; Dehydroepiandrosterone Sulfate; Female; Hirsutism; Humans; Hydrocortisone; Polycystic Ovary Syndrome; Prevalence; Steroid 21-Hydroxylase; Turkey

To assess the activity of the enzyme cytochrome P450c17alpha in patients with polycystic ovary syndrome (PCOS).. Prospective clinical study.. Outpatients at Erciyes University Medical School, Kayseri, Turkey.. Twenty-eight women with PCOS aged 25.44 +/- 4.37 years (mean +/- SD) and 18 normal women aged 26.94 +/- 3.17 years.. Serum levels of 11-deoxycortisol, androstenedione (A), and 17alpha-hydroxy progesterone were measured before and 30 and 60 minutes after ACTH (0.25 mg i.v.) injection.. There was a statistically significant correlation between basal levels of 11-deoxycortisol (4.05 +/-1.16 ng/mL) and A (3.36 +/- 0.97 ng/mL) (r = 0.539). The peak level of 11-deoxycortisol (7.82 +/- 2.36 ng/mL) was also significantly correlated with the peak level of A (6.66 +/- 1.32 ng/mL) (r = 0.570) in women with PCOS. There was no statistically significant correlation between basal A (2.33 +/- 0.50 ng/mL) and basal 11-deoxycortisol (2.71 +/- 0.59 ng/mL) or between peak A (3.38 +/- 0.50 ng/mL) and peak 11 -deoxycortisol (3.68 +/- 0.48 ng/mL) levels in control subjects.. We believe that PCOS is characterized by enhanced activity of 17,20-lyase enzyme in an alternate pathway between 11-deoxycortisol and A in the adrenal glands.

Topics: 17-alpha-Hydroxyprogesterone; Adrenal Glands; Adrenocorticotropic Hormone; Adult; Androstenedione; Cortodoxone; Female; Humans; Kinetics; Polycystic Ovary Syndrome; Prospective Studies; Steroid 17-alpha-Hydroxylase

To investigate the presence of a dysregulation in steroid biosynthesis in women from southern Italy.. Controlled clinical study.. Normal and hyperandrogenic women referred to the Endocrinology Unit of Federico II University Medical School of Naples.. One hundred fifty untreated young hyperandrogenic women and 50 normal age-matched women.. Morning (basal) blood samples obtained in the early follicular phase and after a long (360 minute) ACTH stimulation test.. The adrenal maximal response was calculated as stimulus under curve areas (AUCa), and all steroids were assayed using RIA methods.. A dysregulation of 21-hydroxylase was found in 22 patients (14.7%), with a prevalent increase of 17 alpha-hydroxyprogesterone AUC, whereas in 9 hirsute women (6%), there was a prevalent significant increase in 11-deoxycortisol AUC. In 5 women (3.3%), DHEA and DHEAS basal and AUCs plasma levels were increased, suggesting an impaired 3 beta-olo-dehydrogenase activity. The remaining 114 hyperandrogenic women (76%) compose the nonadrenal group, with a probable diagnosis of primitive functional ovarian hyperandrogenism.. Considering the high prevalence of hirsutism and oligomenorrhea in our female hyperandrogenic population, we suggest an adrenal hyperresponsiveness likely due to a dysregulation in enzymes related to androgen adrenal steroidogenesis.

Topics: 17-alpha-Hydroxyprogesterone; Adolescent; Adrenal Cortex Hormones; Adrenocorticotropic Hormone; Adult; Cortodoxone; Dehydroepiandrosterone; Dehydroepiandrosterone Sulfate; Desoxycorticosterone; Female; Hirsutism; Homeostasis; Humans; Hyperandrogenism; Italy; Polycystic Ovary Syndrome; Steroid 21-Hydroxylase

We report the features of a new syndrome of aromatase deficiency due to molecular defects in the CYP19 (P450arom) gene in a 46,XX female. At birth, the patient presented with a nonadrenal form of female pseudohermaphrodism. At 17 months of age, laparotomy revealed normal female internal genital structures; the histological appearance of the ovaries was normal. FSH concentrations were markedly elevated at 9.4 ng/mL LER 869, and estrone and estradiol levels were undetectable (< 37 pmol/L). By 14 yr of age, she had failed to exhibit breast development. The clitoris had enlarged to 4 x 2 cm, and pubic hair was Tanner stage IV. The plasma concentration of testosterone was elevated at 3294 pmol/L, as was androstenedione at 9951 pmol/L. Plasma estradiol levels were below 37 pmol/L. ACTH and dexamethasone tests indicated a nonadrenal source of testosterone and androstenedione. Plasma gonadotropin levels were in the castrate range. Pelvic sonography and magnetic resonance imaging showed multiple 4- to 6-cm ovarian cysts bilaterally. Despite increased circulating androgens and clitoral growth, the bone age was 10 yr at chronologic age 14 2/12 yr. Estrogen replacement therapy resulted in a growth spurt, breast development, menarche, suppression of gonadotropin levels, and resolution of the cysts. The clinical findings suggested the diagnosis of P450arom deficiency. Analyses of genomic DNA from ovarian fibroblasts demonstrated two single base changes in the coding region of the P450arom gene, one at 1303 basepairs (C-T), R435C, and the other at 1310 basepairs (G-A), C437Y, in exon 10. The molecular genetic studies indicate that the patient is a compound heterozygote for these mutations. Expression of these mutations showed that the R435C mutation had 1.1% the activity of the wild-type P450arom enzyme, whereas the C437Y mutation demonstrated no activity. The cardinal features of this syndrome are a consequence of P450arom deficiency: 1) the fetal masculinization in this syndrome can be ascribed to defective placental conversion of C19 steroids to estrogens, leading to exposure of the female fetus to excessive amounts of testosterone; 2) the pubertal failure, mild virilization, multicystic ovaries, and hyperstimulation of the ovaries by FSH and LH are the result of the inability of the ovary to aromatize testosterone and androstenedione to estrogens; and 3) the striking delay in bone age at 14 2/12 yr supports the notion that estrogens, in contrast to androgens, are th

Topics: 17-alpha-Hydroxypregnenolone; 17-alpha-Hydroxyprogesterone; Adolescent; Adrenocorticotropic Hormone; Androgens; Aromatase; Cortodoxone; Disorders of Sex Development; Estradiol; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Hydroxyprogesterones; Hypogonadism; Luteinizing Hormone; Ovary; Point Mutation; Polycystic Ovary Syndrome; Syndrome

Sixty-three women with ultrasonically detected polycystic ovaries (PCO) were investigated for a disorder of adrenal steroid biosynthesis. Serum was obtained before, and at 30 and 60 min after, the administration of 250 micrograms tetracosactrin, and assayed for 17 alpha-OH-progesterone, 21-deoxycortisol, 17 alpha-OH-pregnenolone and dehydroepiandrosterone by radioimmunoassay following paper chromatography. Results were compared with those in 11 women with normal ovaries, seven adult females with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD), and 15 women heterozygous for this defect. Although the basal-peak steroid concentration differences were significantly greater when ACTH tests were conducted between 1400 and 1700 h than between 0900 and 1000 h, absolute peak steroid concentrations were not different at either time of day. Four of 63 (6.4%) women with PCO had responses to ACTH characteristic of non-classical (late onset) 21OHD CAH, and about half the remainder had responses characteristic of 21OHD heterozygotes. There was no clear cut evidence for a deficiency in 3 beta-hydroxysteroid dehydrogenase activity in women with PCO.

Topics: 17-alpha-Hydroxypregnenolone; 17-alpha-Hydroxyprogesterone; Adrenal Hyperplasia, Congenital; Adult; Cortodoxone; Cosyntropin; Dehydroepiandrosterone; Female; Heterozygote; Humans; Hydroxyprogesterones; Middle Aged; Polycystic Ovary Syndrome; Steroids

Based upon the use of a specific radioimmunoassay method, the plasma levels of 21-deoxycortisol (21-DF) as well as of 17-hydroxyprogesterone (17- OHP) were examined in women with polycystic ovary disease - in normal conditions and after stimulation with corticotropin and chorionic gonadotrophin. It was shown that there was an increased level of 21-DF in women with PCOD in comparison with the control group, both in normal conditions and after ACTH stimulation. Besides there was a pathological increase in the level of 21-DF after HCG stimulation, which was not observed in the control group. These results suggest an enzymatic defect ("11-aberrant hydroxylation") in PCOD, which is due to a disturbance of steroidogenesis. Based upon the positive experiences with a defined spleen dialysate ("DSD", Solcosplen) in the treatment of climacteric discomforts and its known influence on steroidogenesis, we examined possible effects on the levels of 21-DF in females with PCOD. The decrease in the level of this steroid was associated with bleeding in women with amenorrhea. This shows the importance of 21-DF in the pathogenesis of PCOD and proves the efficacy of the spleen dialysate in women suffering from this disease.

Topics: 17-alpha-Hydroxyprogesterone; 17-Hydroxycorticosteroids; Adrenocorticotropic Hormone; Adult; Chorionic Gonadotropin; Cortodoxone; Dialysis; Female; Humans; Hydroxyprogesterones; Polycystic Ovary Syndrome; Spleen

Topics: 17-Hydroxycorticosteroids; Adrenocorticotropic Hormone; Cortodoxone; Female; Hirsutism; Humans; Hydrocortisone; Hydroxylation; Hydroxyprogesterones; Polycystic Ovary Syndrome

When ovarian mitochondria from a patient with polycystic ovary syndrome (POS) were incubated with [7-3H]17alpha-hydroxypregnenolone and [4-14C]17alpha-hydroxyprogesterone, 11beta-hydroxylated metabolites were obtained. When the same mitochondrial preparation was incubated with [7-3H]17alpha-hydroxypregnenolone and [4-14C]11-deoxycortisol no 11beta-hydroxylated derivatives of 11-deoxycortisol were found. These results are compatible with and support the conclusion that the ovary of POS patients who excrete pregnanetriolone contains an 11beta-hydroxylase capable of hydroxylating C-21-deoxysteroids but not C-21-hydroxysteroids.

Topics: 17-alpha-Hydroxypregnenolone; Cortodoxone; Female; Humans; Hydroxyprogesterones; Hydroxysteroids; In Vitro Techniques; Mitochondria; Ovary; Polycystic Ovary Syndrome; Steroid Hydroxylases