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R Azziz

Publications and source records attributed to R Azziz.

At least 19 recordsLinked to original sources
PubMed2006

Reproductive outcome of women with 21-hydroxylase-deficient nonclassic adrenal hyperplasia.

CONTEXT: Because many women with 21-hydroxylase (21-OH)-deficient nonclassic adrenal hyperplasia (NCAH) carry at least one allele affected by a severe mutation of CYP21, they are at risk for giving birth to infants with classic adrenal hyperplasia (CAH). OBJECTIVE: Our objective was to determine the frequency of CAH and NCAH infants born to mothers with 21-OH-deficient NCAH. DESIGN AND SETTING: We conducted an international multicenter retrospective/prospective study. PATIENTS AND METHODS: The outcome of 203 pregnancies among 101 women with 21-OH-deficient NCAH was reviewed. The diagnosis of 21-OH-deficient NCAH was established by a basal or post-ACTH-stimulation 17-hydroxyprogesterone level of more than 10 ng/ml (30.3 nmol/liter). When possible, genotype analyses were performed to confirm CAH or NCAH in the offspring. RESULTS: Of the 203 pregnancies, 138 (68%) occurred before the mother's diagnosis of NCAH and 65 (32%) after the diagnosis. Spontaneous miscarriages occurred in 35 of 138 pregnancies (25.4%) before the maternal diagnosis of NCAH, and in only four of 65 pregnancies (6.2%) after the diagnosis (P < 0.002). Four (2.5%; 95% confidence interval, 0.7-6.2%) of the 162 live births were diagnosed with CAH. To date, 24 (14.8%; 95% confidence interval, 9.0-20.6%) children, 13 girls and 11 boys, have been diagnosed with NCAH. The distribution of NCAH children and their mothers varied significantly by ethnicity (P < 0.0001, for both). CONCLUSIONS: The risk of a mother with 21-OH-deficient NCAH for giving birth to a child affected with CAH is 2.5%; at least 14.8% of children born to these mothers have NCAH.

Adrenal Hyperplasia, Congenital↗
PubMed2004

Androgen excess in women: experience with over 1000 consecutive patients.

The objective of the present study was to estimate the prevalence of the different pathological conditions causing clinically evident androgen excess and to document the degree of long-term success of suppressive and/or antiandrogen hormonal therapy in a large consecutive population of patients. All patients presenting for evaluation of symptoms potentially related to androgen excess between October 1987 and June 2002 were evaluated, and the data were maintained prospectively in a computerized database. For the assessment of therapeutic response, a retrospective review of the medical chart was performed, after the exclusion of those patients seeking fertility therapy only, or with inadequate follow-up or poor compliance. A total of 1281 consecutive patients were seen during the study period. Excluded from analysis were 408 patients in whom we were unable to evaluate hormonal status, determine ovulatory status, or find any evidence of androgen excess. In the remaining population of 873 patients, the unbiased prevalence of androgen-secreting neoplasms was 0.2%, 21-hydroxylase-deficient classic adrenal hyperplasia (CAH) was 0.6%, 21-hydroxylase-deficient nonclassic adrenal hyperplasia (NCAH) was 1.6%, hyperandrogenic insulin-resistant acanthosis nigricans (HAIRAN) syndrome was 3.1%, idiopathic hirsutism was 4.7%, and polycystic ovary syndrome (PCOS) was 82.0%. Fifty-nine (6.75%) patients had elevated androgen levels and hirsutism but normal ovulation. A total of 257 patients were included in the assessment of the response to hormonal therapy. The mean duration of follow-up was 33.5 months (range, 6-155). Hirsutism improved in 86%, menstrual dysfunction in 80%, acne in 81%, and hair loss in 33% of patients. The major side effects noted were irregular vaginal bleeding (16.1%), nausea (13.0%), and headaches (12.6%); only 36.6% of patients never complained of side effects. In this large study of consecutive patients presenting with clinically evident androgen excess, specific identifiable disorders (NCAH, CAH, HAIRAN syndrome, and androgen-secreting neoplasms) were observed in approximately 7% of subjects, whereas functional androgen excess, principally PCOS, was observed in the remainder. Hirsutism, menstrual dysfunction, or acne, but not alopecia, improved in the majority of patients treated with a combination suppressive therapy; although more than 60% experienced side effects.

Acanthosis Nigricans↗
PubMed2003

The effectiveness of the interview for predicting the presence of polycystic ovary syndrome.

Polycystic ovary syndrome (PCOS), a leading cause of female infertility, occurs in approximately 4% of women of reproductive age. Multifamily studies have established that PCOS has strong inherited traits. Although diagnosis of PCOS in the relatives of affected women can readily be made by clinical and biochemical evaluations, these methods are costly and laborious. The aim of this investigation was to determine whether clinically evident PCOS could be detected by a written questionnaire, which is a significantly less expensive means of detection than direct determination. A questionnaire about the history of possible androgenic symptoms of PCOS was presented to patients and their first-degree female relatives, who were also evaluated by physical and laboratory examinations. The sensitivity, specificity, and positive predictive value (PPV) and negative predictive value (NPV) for the detection of PCOS by interview were calculated. The NPV of the proband interview was significantly lower for sisters than for mothers (82% vs. 100%, respectively; p < 0.05). When the family member completed the written questionnaire directly, the specificity and NPV of self-reporting were equally high (> 90%) for both mothers and sisters. Thus direct interviewing of PCOS patients or their mothers and sisters reliably predicts affected status, but patient interview alone will not predict PCOS in almost 50% of the affected sisters.

Adult↗
PubMed2003

Polycystic ovary syndrome and ovulation induction.

Polycystic ovary syndrome (PCOS) is likely the most common cause of anovulatory infertility. Although many options are available for ovulation induction in these patients, there is currently no evidence-based algorithm to guide the initial and subsequent choices of ovulation induction methods. In obese women with PCOS, mild to moderate weight loss results in improvement of ovulatory dysfunction, and should be advocated at the onset of the evaluation. Clomiphene citrate is currently the 1st line medical therapy for ovulation induction. Glucocorticoids do not result in consistent ovulation and have significant side effects. Exogenous pulsatile GnRH treatment has low ovulation and pregnancy rates with a high risk of miscarriage. The most commonly used medical agents for ovulation induction in clomiphene-resistant women with PCOS are parenteral gonadotropins. Various gonadotropin preparations and different protocols are available; however the risk of multiple pregnancy and ovarian hyperstimulation is high with gonadotropin therapy. The frequent association between PCOS and insulin resistance has prompted recent studies on the effect of insulin-sensitizing agents on spontaneous and as an adjuvant to conventional ovulation induction therapies. Overall, the improvement in ovulation with insulin sensitizing drugs is modest, and unresolved issues such as variability in ovarian response remain to be addressed in future studies. Nevertheless, these agents may be beneficial in a subset of PCOS patients. Surgical ovulation induction methods such as ovarian diathermy have been reported to be moderately effective. However, due to the inherent associated risks and unknown effect on long-term reproductive potential, this modality should be reserved for patients who are clomiphene-resistant and unable or unwilling to proceed to gonadotropin therapy.

Female↗
PubMed2001

Prevalence of polycystic ovary syndrome (PCOS) in first-degree relatives of patients with PCOS.

OBJECTIVE: To determine the rate of clinically evident polycystic ovary syndrome (PCOS) among first-degree female relatives within families with a proband affected by PCOS. DESIGN: Clinical and biochemical evaluation of the mothers and sisters of 93 patients with PCOS. The diagnosis of PCOS was established by: [1] a history of oligomenorrhea, [2] clinical evidence (i.e., hirsutism) or biochemical evidence (i.e., elevated total or free T) of hyperandrogenism, and [3] the exclusion of related disorders. SETTING: Tertiary care university. PATIENT(S): Patients with PCOS and their mothers and sisters. INTERVENTION(S): Interview, physical examination, and hormonal testing on blood samples were performed for all subjects. MAIN OUTCOME MEASURE(S): The presence of hirsutism and hyperandrogenemia was determined in the mothers and sisters of the patients with PCOS. RESULT(S): Of the 78 mothers and 50 sisters evaluated clinically, 19 (24%) and 16 (32%) were affected with PCOS, respectively. A higher rate of PCOS was observed when only premenopausal women not taking hormones (i.e., untreated) were considered (i.e., 35% of mothers and 40% of sisters), consistent with amelioration of symptoms with hormonal therapy or aging. These rates of PCOS are significantly higher than that observed in our general population (approximately 4%) and suggest the involvement of a major genetic component in the disorder. CONCLUSION(S): The rates of PCOS in mothers and sisters of patients with PCOS were 24% and 32%, respectively, although the risk was higher when considering untreated premenopausal women only.

Adolescent↗
PubMed2001

Altered autophosphorylation of the insulin receptor in the ovary of a woman with polycystic ovary syndrome.

OBJECTIVE: To determine whether the tyrosine autophosphorylation of the insulin receptor (IR) in the ovary of a woman with polycystic ovary syndrome (PCOS) was reduced compared to normal. DESIGN: Experimental study. SETTING: Tertiary care medical center. PATIENT(S): One woman with PCOS and one healthy control, both of whom underwent a hysterectomy and bilateral salpingo-oophorectomy. INTERVENTION(S): Plasma membrane fraction of ovarian tissue was isolated, and the IR was purified and concentrated. MAIN OUTCOME MEASURE(S): Western blots of the IR, which had been incubated with and without insulin, were prepared. Colorimetric and chemiluminescent methods were used to detect the presence of the IR beta-subunit and IR tyrosine autophosphorylation, respectively. RESULT(S): The presence of the beta-subunit of the IR was identified in both ovarian samples. The degree of insulin-stimulated IR tyrosine autophosphorylation, reflected by the mean (+/-SD) relative optical density of the 95 kd band, was 4.3-fold higher in the normal ovary compared to the PCOS ovary (0.56 +/- 0.18 optical density vs. 0.13 +/- 0.10 optical density, respectively). CONCLUSION(S): Tyrosine autophosphorylation of the IR may be decreased in the ovaries of women with PCOS, similar to the findings in other tissues. The mechanisms through which insulin acts to produce an excess in ovarian androgen production in the face of a decrease in ovarian IR autophosphorylation remain to be determined.

Adult↗
PubMed2001

Hyperandrogenemia in patients presenting with acne.

OBJECTIVE: To determine whether acne is associated with hyperandrogenemia, regardless of age of presentation. DESIGN: Prospective controlled study. SETTING: Tertiary-care medical center. PATIENT(S): Thirty consecutive unselected women presenting with acne and no hirsutism and 24 eumenorrheic healthy controls. INTERVENTION(S): Serum samples was taken in all patients, and an acute 60-minute ACTH-(1-24) test was performed in 19 patients. MAIN OUTCOME MEASURE(S): Total and free T, sex hormone-binding globulin (SHBG), and DHEAS levels in basal samples, and ACTH-stimulated 17-hydroxyprogesterone (17-HP) response to exclude 21-hydroxylase (21-OH)-deficient nonclassic adrenal hyperplasia (NCAH) were determined. RESULT(S): Nonhirsute patients with acne demonstrated significantly lower levels of SHBG and higher free-T and DHEAS levels than controls. Nineteen (63%) acneic patients had at least one androgen value above the 95% of controls. In patients aged 12-18 years, 7/8 (88%) had at least one increased androgen value, compared with 12/22 (55%) patients aged 19-43 years. One patient (5.3%) was found to have 21-OH-deficient NCAH. CONCLUSION(S): Hyperandrogenemia was evident in a majority of nonhirsute acneic patients studied, regardless of age. These data suggest that androgen suppression may be useful in treating acne in many of these patients.

17-alpha-Hydroxyprogesterone↗
PubMed2001

Repeated freezing and thawing does not generally alter assay results for several commonly studied reproductive hormones.

OBJECTIVE: To determine the effect of repeated freezing and thawing, and storage temperature, on the assay results of commonly measured reproductive hormones, and sex hormone-binding globulin (SHBG) in human serum. DESIGN: Prospective laboratory study. SETTING: Academic medical center. PATIENT(S): Four men and three pregnant women in the third trimester. INTERVENTION(S): Pooled serum from men and pregnant females were frozen at either -20 degrees C or -70 degrees C. Aliquots were then subjected to repeat freeze/thaw cycles, from 1 to 10 times, and assays were performed after the final freeze/thaw cycle. MAIN OUTCOME MEASURE(S): Assay results for eight hormones (FSH, LH, PRL, androstenedione (A), 17alpha-hydroxyprogesterone, P, insulin, and SHBG, as a function of the number of freeze/thaw cycles and storage temperature. RESULT(S): Only SHBG in male serum at -20 degrees C and P in pregnant serum at -70 degrees C showed statistically significant decreases in assay results with repeated freeze/thaw cycles (3.3% and 1.1% per cycle, respectively). All other analytes did not show significant changes as a function of freeze/thaw cycles or storage temperature. CONCLUSION(S): There is no consistent or predictable alteration in the results of SHBG, or the glycoprotein and steroid hormones evaluated, as a function of repeated freeze/thaw or the storage temperature of human serum.

Blood Preservation↗
PubMed2001

Influence of insulin and testosterone on adrenocortical steroidogenesis in vitro: preliminary studies.

OBJECTIVE: The mechanisms underlying the adrenal androgen (AA) excess of polycystic ovary syndrome (PCOS) remain unclear, although it is possible that the adrenocortical dysfunction may be a response to other, extraadrenal factors. Consistent with the pathophysiology of PCOS and with in vivo data in normal and PCOS women, we have hypothesized that insulin inhibits and that T stimulates AA secretion in vitro. DESIGN: In vitro experimental study. SETTING: University medical center. PATIENT(S): Normal human adrenals (n = 4 women, ages 25-57 years) were obtained with consent at the time of organ donation. INTERVENTION(S): Fresh adrenal tissue minces were incubated in serum-free medium with 10-microM pregnenolone substrate and 1-microM ACTH-(1-24). Challenge doses of 0.2, 1, 5, 20, and 100 nM of insulin and 1, 10, 100, 1,000, and 10,000 nM of T were added, and the media were sampled after 8 hours of incubation at 37 degrees C, 4% CO2. Dehydroepiandrosterone (DHEA), DHEA-sulfate (DHEAS), and cortisol (F) were measured by radioimmunoassay (significant effects compared with the case of zero-dose control). MAIN OUTCOME MEASURE(S): The production of DHEA, DHEAS, and F in the media of the adrenal minces was compared between different subjects and at different concentrations of T and insulin. RESULT(S): Analysis of the combined data from all donors indicated that insulin stimulated DHEAS and suppressed DHEA production but had no consistent effect on F. Similar analyses of the combined data indicated that T had no significant predictable effect on the production of DHEAS, DHEA, or F. When examining donor data individually, insulin and T did elicit significant increases and/or decreases in steroid production within subjects, although no consistent trends were observed. CONCLUSION(S): On the basis of these data, it is clear that extra-adrenal factors such as insulin and T have some adrenal regulatory capacity. In general, insulin stimulated DHEAS and decreased DHEA production, suggesting that it increases adrenocortical sulfotransferase activity. However, although in the individual subjects studied, both insulin and T frequently altered the production of DHEAS, DHEA or F, these effects did not appear to be uniform or consistent from subject to subject. Expanded studies are required to confirm these results.

Adrenal Cortex↗
PubMed2001

The role of the adrenal cortex in polycystic ovary syndrome.

Adrenal androgen excess affects approximately 25% of PCOS patients. The exact etiology of this excess in PCOS patients is unclear. Some evidence that adrenal androgen excess may be a genetic trait. The adrenal androgen response to ACTH is highly individualized, and the relative response seems to be constant over time. In addition, there is a strong familial component to adrenal androgen levels in normal individuals and PCOS patients. It is possible that the tendency to overproduce adrenal androgens is an inherited risk factor for the development of PCOS. Overall, few hyperandrogenic patients actually have isolated deficiencies of 3 beta-hydroxysteroid dehydrogenase, 21-hydroxylase, and 11-hydroxylase. The ovarian hormonal secretion in PCOS can affect adrenal androgen secretion and metabolism, although this factor accounts for only part of this abnormality. More likely, the adrenal androgen excess results from a generalized hyperresponsiveness of the adrenal cortex to ACTH, but without an increase in CRH or ACTH sensitivity. Although glucocorticoid administration may improve the ovulatory function of these patients, the results are modest and cannot be predicted by the circulating androgen levels.

Adrenal Cortex Diseases↗
PubMed2001

Facial and abdominal hair growth in hirsutism: a computerized evaluation.

Methods of objectively assessing the growth rate of hairs in hirsute women have generally required some form of shaving and have focused on studying hairs affecting the face, which has reduced the number of patients willing or able to participate in such studies. A possible solution is to assess the terminal hairs on the lower abdomen (ie, the male escutcheon) because these two body areas are the most frequently affected with excess hair growth in hirsute patients. Nonetheless, it is unclear how the growth characteristics (density, diameter, and growth rate) of the hairs on the abdomen and face differ in these patients. We hypothesize that the growth characteristics of terminal hairs on the abdomen and face are similar and that evaluation of either area may be sufficient in assessing the hair growth rate of these patients. To objectively evaluate hair growth in the face and abdomen in hirsute patients, we developed a computer-aided image analysis system capable of measuring several growth parameters. Twenty hirsute women (12 white and 8 black), aged 31.2 +/- 6.1 years, were studied. Facial and abdominal skin areas were shaved, and 3 to 5 days later the areas were photographed through a calibrated glass plate and 5 terminal hairs were plucked from each area. The daily hair growth rate (assessed by photography and by direct measurement of the plucked hair), the density of hairs (number of hairs per surface area assessed by photography), and hair diameter (of the plucked hairs) were determined. The extent of hirsutism was also measured, albeit subjectively, by a modification of the Ferriman-Gallwey method, with each area given a score of 0 (no terminal hairs seen) to 4 (terminal hairs in a pattern similar to that of a very hirsute man). Facial, abdominal, and total Ferriman-Gallwey scores were 1.3 +/- 0.6, 1.8 +/- 0.9, and 12.5 +/- 5.4, respectively. Our results indicated that facial hairs were distributed in greater density and had a greater diameter than abdominal hairs (15.6 +/- 14.2 hairs/cm(2) vs 5.4 +/- 1.9 hairs/cm(2), and 84.5 +/- 19.5 microm and 66.2 +/- 17.5 microm, respectively, P <.005). Alternatively, the growth rates of facial and abdominal hairs were similar, whether determined photographically (0.36 +/- 0.18 mm/day vs 0.43 +/- 0.19 mm/day, respectively) or from plucked hairs (1.2 +/- +0.2 mm/d vs 1.4 + 0.4 mm/d, respectively). We conclude that although the density and diameter of facial hairs are greater than that of lower abdominal hairs, these areas have very similar growth rates. Hence evaluation of either of the body areas, using an objective method of assessing hair growth, should provide equivalent results.

Abdomen↗
PubMed2001

Steroidogenic acute regulatory protein (StAR) in the ovaries of healthy women and those with polycystic ovary syndrome.

OBJECTIVE: Polycystic ovary syndrome is the most common cause of oligo-ovulation, affecting approximately 4% of women. A primary defect of steroidogenesis resulting in increased ovarian and adrenal androgen production may be responsible for polycystic ovary syndrome, at least in some patients. Because the action of the steroidogenic acute regulatory protein (StAR) initiates the process of steroidogenesis, we proceeded to test the hypothesis that increased production or concentration of StAR may result in the abnormality of steroidogenesis found in polycystic ovary syndrome. STUDY DESIGN: We examined the ovaries from 10 healthy women and 7 women with polycystic ovary syndrome, determining the relative concentration of StAR in total protein extracts by use of Western blotting, and the overall distribution and staining intensity of StAR in prepared tissue sections. RESULTS: Overall the ovaries of healthy women and women with polycystic ovary syndrome demonstrated a similar prevalence and size of follicular cysts, although the ovaries of women with polycystic ovary syndrome had a greater mean number of follicular cysts. In general, the distribution of StAR immunoreactivity within most of the ovarian structures was not different in the ovaries of women with polycystic ovary syndrome compared to those of the healthy ovaries. However, the ovaries from the cases demonstrated a significantly greater number of follicular cysts with staining for StAR immunoreactivity in the thecal cells than did the ovaries from healthy women (100% vs 38%, P <.05). CONCLUSION: These data suggest that the exaggeration in androgen biosynthesis in the ovaries of patients with polycystic ovary syndrome may be occurring at its earliest step (ie, that involving StAR), such that an increased amount of cholesterol is made available for androgen biosynthesis in the polycystic ovary.

Adult↗
PubMed2001

Troglitazone improves ovulation and hirsutism in the polycystic ovary syndrome: a multicenter, double blind, placebo-controlled trial.

We hypothesized that the administration of troglitazone, an insulin-sensitizing agent of the thiazolidinedione class, would improve the ovulatory dysfunction, hirsutism, hyperandrogenemia, and hyperinsulinemia of polycystic ovary syndrome (PCOS) patients. Four hundred and ten premenopausal women with PCOS in a multicenter, double blind trial were randomly assigned to 44 weeks of treatment with placebo (PBO) or troglitazone [150 mg/day (TGZ-150), 300 mg/day (TGZ-300), or 600 mg/day (TGZ-600)]. We compared changes in ovulatory function (by monitoring the urinary level of pregnanediol-3-glucuronide daily), hirsutism (by a modified Ferriman-Gallwey scoring method), hormonal levels (total and free testosterone, androstenedione, sex hormone-binding globulin, LH, FSH, and the LH/FSH ratio), and measures of glycemic parameters (fasting levels of glucose, insulin, hemoglobin A(1c), and the glucose and insulin areas under the curve during an oral glucose challenge) among study groups. Of the 410 patients recruited, 305 (74.4%) met evaluability criteria and were included in the analyses. The patients' baseline characteristics were similar across all treatment arms. Ovulatory rates were significantly greater for patients receiving TGZ-300 and TGZ-600 than for those receiving PBO (0.42 and 0.58 vs. 0.32; P < 0.05 and 0.0001, respectively). Of PCOS patients treated with TGZ-600, 57% ovulated over 50% of the time compared with 12% of placebo-treated patients. There was a significant decrease in the Ferriman-Gallwey score with TGZ-600 compared with PBO (0.22 +/- 0.53 vs. -2.21 +/- 0.49; P < 0.05, respectively). Free testosterone decreased and sex hormone-binding globulin increased in a dose-related fashion with troglitazone treatment, and all three troglitazone treatment groups were significantly different from placebo. Nearly all glycemic parameters showed dose-related decreases with troglitazone treatment. The total number and severity of adverse events (including elevations in liver enzymes) and the proportion of patients withdrawn from the study due to the development of adverse effects were similar between treatment groups. Troglitazone improves the ovulatory dysfunction, hirsutism, hyperandrogenemia, and insulin resistance of PCOS in a dose-related fashion, with a minimum of adverse effects.

Adult↗
PubMed2001

Adrenocortical secretion of dehydroepiandrosterone in healthy women: highly variable response to adrenocorticotropin.

Excess adrenal androgen (AA) levels are observed in 25--50% of women with the polycystic ovary syndrome (PCOS), and AA excess in PCOS may represent selection bias. Thus, it is possible that AA secretion among the general population is highly variable, and that those women who are predisposed to secreting greater amounts of AA have a greater probability of having PCOS. We now hypothesize that the levels of AAs are highly variable among normal nonhyperandrogenic women, and that this heterogeneity is the result of a variable response of AAs to ACTH stimulation. To test this hypothesis we prospectively studied the response of dehydroepiandrosterone (DHA) and cortisol (F) to a 60-min acute stimulation with ACTH-(1--24) in 56 healthy eumenorrheic nonhirsute healthy women with a mean age of 28.9 yr (range, 20--37 yr.) and a mean body mass index (BMI) of 29.2 kg/m(2) (18.2--46.2 kg/m(2)). Baseline samples and poststimulation samples were assayed for DHA and F. The basal and ACTH-stimulated levels of DHA, but not those of F, were negatively correlated with age, although neither the basal nor ACTH-stimulated responses of DHA and F varied with BMI. After controlling for age, the basal F level was negatively correlated to its net increment (i.e. Delta F; r = -0.54; P < 0.001), whereas there was no significant relationship between basal DHA and Delta DHA. We also compared the intersubject variability (coefficient of variation) for basal and stimulated levels of DHA and F. For basal (DHA(0)), 60 min (DHA(60)), and net increment in (Delta DHA) DHA levels, the coefficients of variation were 67.9%, 61.4%, and 76.0%, respectively; for F(0), F(60), and Delta F, they were 40.4%, 16.9%, and 31.3%, respectively. The variance in Delta DHA was significantly higher, and the variance in F(60) was significantly lower than that in all other variables; DHA(0), DHA(60), F(0), and Delta F had similar variances. In conclusion, in our population of healthy reproductive-aged women we observed that both basal and ACTH-stimulated levels of DHA after ACTH-(1--24) stimulation had significantly greater intersubject variance (approximately 60--70%) compared with the basal and poststimulation levels of F (approximately 15--40%). These data support the hypothesis that among normal women, AA (i.e. DHA) levels are highly variable compared to those of F. In addition, the intersubject variability in DHA levels is at least in part due to a variable response of AAs to ACTH stimulation. Whether the AA excess frequently observed in PCOS is due to the greater risk of those women with higher AA levels, basally and after ACTH stimulation, remains to be confirmed.

Adrenal Cortex↗
PubMed2001

[Infertility treatment before assisted reproductive techniques].

The prevalence of infertility has increased in recent years, but the medical services to treat these problems are not available for most of the affected couples. The prognosis for fertility is important in order to determine the therapeutic capacity of each service, and to select the couples that could be treated at a primary level, or to send them to more advanced levels of reproductive technology. In practice, the infertility is treated in primary medical levels and the assisted reproductive technology is available only to a limited sector of the poblation. In general, the managed-care plans do not compensate directly for infertility treatments, but they are indirectly paying some therapeutic procedures for fertility.

Adolescent↗
PubMed2000

A multicenter study of women with nonclassical congenital adrenal hyperplasia: relationship between genotype and phenotype.

Characteristic presentation of nonclassical adrenal hyperplasia (NCAH) due to 21-hydroxylase deficiency was compared between women carrying a severe and a mild CYP21 mutation (Group 1, N = 26) versus homozygotes for mild mutations (Group 2, N = 8). The diagnosis was based on elevated ACTH-stimulated 17OH-progesterone (17OHP). Genotyping for 10 mutations was performed by PCR-based techniques. Jewish patients predominated among Group 2 (25% vs 11.5% in Group 1); however, 85% of all patients were non-Jewish Caucasians. Average age of presentation was 23-25 years, and did not differ between groups. Hirsutism, and to a lesser extent oligomenorrhea and acne, were more prevalent among Group 1 women. There was a trend to higher basal 17OHP among Group 1 patients (mean +/- SEM; 1354+/-323 vs 714+/-129 ng/dl, P< or =0.25). The lack of significant difference was perhaps due to the relatively few homozygotes for 2 mild mutations (24%). V281L was carried on approximately 48% of all alleles, and about 16% carried either P30L or P453S. Approximately 38% of alleles and 77% of patients carried a classic mutation. These data have important implications for genetic counseling. In summary, we describe differences in clinical, hormonal, and genetic characteristics among a multiethnic group of females with NCAH.

17-alpha-Hydroxyprogesterone↗
PubMed2000

Prevalence of 21-hydroxylase-deficient nonclassic adrenal hyperplasia and insulin resistance among hirsute women from Puerto Rico.

OBJECTIVE: To determine the prevalence of 21-hydroxylase (21-OH)-deficient nonclassic adrenal hyperplasia (NCAH) and insulin resistance in hirsute women from Puerto Rico. DESIGN: Cross-sectional prospective study. SETTING: Clinical research center. PATIENT(S): 100 consecutive untreated hirsute women. MAIN OUTCOME MEASURE(S): Fasting total T, free T, DHEAS, insulin, and glucose were measured, and a 60-minute acute ACTH-(1-24) stimulation for 17-hydroxyprogesterone (17-HP) was performed. A diagnosis of 21-OH-deficient NCAH was considered when the stimulated 17-HP level was >30.3 nmol/L. The glucose/insulin ratio was calculated as a measure of insulin resistance (normal value, > or =4.5). RESULT(S): Patients had a mean (+/-SD) age of 26.8+/-6.6 years; 82 were oligomenorrheic. Overall, 12%, 8%, and 60% of patients had elevated levels of DHEAS, total T, or free T, respectively. One patient was identified as having 21-OH-deficient NCAH. Eight women, none of whom had NCAH, were found to be hyperglycemic; four of these women had type 2 diabetes mellitus. Excluding hyperglycemic patients, a glucose/insulin ratio of <4.5, consistent with IR, was found in 51.7%. CONCLUSION(S): The prevalence of 21-OH-deficient NCAH among patients from Puerto Rico does not differ significantly from that reported for other non-Jewish, non-Hispanic white populations.

Adrenal Hyperplasia, Congenital↗