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PubMed1967

Testosterone and androstenedione blood production rates in normal women and women with idiopathic hirsutism or polycystic ovaries.

The average plasma testosterone concentration of women with either hirsutism or polycystic ovaries and hirsutism was higher (p < 0.01) than that of normal women although the ranges overlapped. Testosterone blood production rates averaged 830 +/- 120 SE and 1,180 +/- 310 SE mug per day in the two groups of hirsute women and 230 +/- 33 SE mug per day in normal women. The ranges did not overlap. The testosterone metabolic clearance rates of hirsute women (1,090 +/- 140 SE L per day) and of men (1,240 +/- 136 SE L per day) were significantly higher than those of normal women (590 +/- 44 SE L per day). These differences persisted when the metabolic clearance rates were corrected for surface area. We suggest that testosterone metabolic clearance rates vary directly with some function of testosterone production. The mean plasma androstenedione levels (2.8 +/- 0.35 SE and 2.8 +/- 0.30 SE mug per L) and production rates (6,060 +/- 450 SE and 7,360 +/- 345 SE mug per day) of the women with hirsutism or polycystic ovaries, respectively, were significantly higher than those of normal women (1.5 +/- 0.22 SE mug per L; 3,300 +/- 830 SE mug per day). The androstenedione metabolic clearance rates were the same in each group. Plasma androstenedione was the precursor of 49% of plasma testosterone in normal women and of 26% of plasma testosterone in hirsute women. Thus, 74% of the plasma testosterone in these subjects must have been either secreted or derived from a precursor that did not enter the plasma androstenedione pool.

17-Ketosteroids↗
PubMed2000

Idiopathic hirsutism.

Hirsutism, the presence of terminal (coarse) hairs in females in a male-like pattern, affects between 5% and 10% of women. Of the sex steroids, androgens are the most important in determining the type and distribution of hairs over the human body. Under the influence of androgens hair follicles that are producing vellus-type hairs can be stimulated to begin producing terminal hairs (i.e., terminalized). The activity of local 5alpha-reductase (5alpha-RA) determines to a great extent the production of dihydrotestosterone (DHT), and consequently the effect of androgens on hair follicles. While there are two distinct 5alpha-RA isoenzymes, type 1 and type 2, the activity of these in the facial or abdominal skin of hirsute women remains to be determined. Although the definition of idiopathic hirsutism (IH) has been an evolving process, the diagnosis of IH should be applied only to hirsute patients with normal ovulatory function and circulating androgen levels. A history of regular menses is not sufficient to exclude ovulatory dysfunction, since up to 40% of eumenorrheic hirsute women are anovulatory. The diagnosis of IH, when strictly defined, will include less than 20% of all hirsute women. The pathophysiology of IH is presumed to be a primary increase in skin 5alpha-RA activity, probably of both isoenzyme types, and possibly an alteration in androgen receptor function. Therapeutically, these patients respond to antiandrogen or 5alpha-RA inhibitor therapy. Pharmacological suppression of ovarian or adrenal androgen secretion may be of additional, albeit limited, benefit. New therapeutic strategies such as laser epilation or the use of new biological response modifiers may play an important role in offering a more effective means of treatment to remove unwanted hair. Further investigations into the genetic, molecular, and metabolic aspects of this disorder, including only well defined patients, are needed.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗
PubMed1983

Evidence for the importance of peripheral tissue events in the development of hirsutism in polycystic ovary syndrome.

Hirsutism can occur in the presence of normal or near normal levels of serum testosterone, unbound testosterone (uT), dehydroepiandrostene sulfate, androstenedione, and dihydrotestosterone. However, we have found that serum androstanediol glucuronide (3 alpha-diol G) is markedly increased in idiopathic hirsutism and it serves as an excellent marker of peripheral androgen metabolism and action. In the present work, we studied 12 hirsute (H) and 12 nonhirsute (NH) patients with polycystic ovary syndrome (PCO) and 13 age and weight matched controls in order to determine if differences in sex steroid levels or peripheral tissue androgen events were associated with hirsutism. Serum unbound estradiol levels and LH-FSH ratios were similar in both groups of women with PCO, and both were significantly higher than levels in controls (P less than 0.05). Whereas serum testosterone, uT, and androstenedione were elevated in both H-PCO and NH-PCO patients compared to controls, the levels in these two groups were similar. Serum dehydroepiandrostene sulfate was higher in PCO patients compared to controls, but H-PCO patients had slightly higher levels than NH-PCO patients. Serum delta 5-androstenediol was also slightly higher in H-PCO compared to NH-PCO patients. Dihydrotestosterone was normal and unconjugated; 3 alpha-diol was higher than normal in both groups of patients with PCO, although H-PCO patients had higher levels than NH-PCO patients. Compared to these relatively minor changes between the PCO patient groups, serum 3 alpha-diol G was markedly elevated in H-PCO patients (approximately 10-fold), yet normal in NH-PCO patients (P less than 0.01). The ratios of serum 3 alpha-diol G-uT were similar in NH-PCO patients and controls, but were elevated in H-PCO patients (P less than 0.01). These data indicate that: 1) women with PCO have increased circulating androgen levels regardless of the presence or absence of hirsutism; and 2) the presence of hirsutism is not only a function of circulating androgen levels, but may also be determined by events in peripheral tissues.

Adolescent↗
PubMed1985

Late-onset adrenal steroid 3 beta-hydroxysteroid dehydrogenase deficiency. I. A cause of hirsutism in pubertal and postpubertal women.

To investigate the adrenal cause of hyperandrogenism in peri- and postpubertal hirsute women, baseline and ACTH-stimulated serum concentrations of delta 5-17-hydroxypregnenolone (delta 5-17P), dehydroepiandrosterone (DHEA) and its sulfate, 17-hydroxyprogesterone (17-OHP), cortisol, delta 4-androstenedione, and testosterone were determined in 116 women with hirsutism or acne of peri- and postpubertal onset with or without menstrual abnormalities. The results were compared with the same steroid concentrations in 30 normal age-matched women. Sixteen of the 116 women with hirsutism whose ACTH-stimulated 17-OHP levels (mean +/- SD, 5404 +/- 3234 ng/dl; normal, 334 +/- 194) were markedly elevated while their ratios of delta 5-17P to 17-OHP (0.4 +/- 0.2; normal, 3.4 +/- 1.5) were low were diagnosed as having nonclassical symptomatic 21-hydroxylase deficiency. Seventeen other hirsute women, including 3 siblings, had very high responses of delta 5-17P (2276 +/- 669 ng/dl; normal, 985 +/- 327) and DHEA (2787 +/- 386 ng/dl; normal, 1050 +/- 384) to ACTH stimulation, with significantly elevated ratios of delta 5-17P to 17-OHP (11 +/- 2.0; normal, 3.4 +/- 1.5) and DHEA to delta 4-androstenedione (7.5 +/- 2.3; normal, 4.6 +/- 1.5). In these hirsute women, the morning serum delta 5-17P and DHEA concentrations were elevated, had a diurnal variation, and were suppressed with dexamethasone administration. We propose that partial adrenal 3 beta-hydroxysteroid dehydrogenase deficiency is the cause of hirsutism in these women. This may represent an allelic variant at the genetic locus for 3 beta-hydroxysteroid dehydrogenase deficiency similar to that reported for symptomatic nonclassical 21-hydroxylase deficiency producing peripubertal excess androgen syndrome.

3-Hydroxysteroid Dehydrogenases↗
PubMed1987

Salivary testosterone in hirsutism: correlations with serum testosterone and the degree of hair growth.

Testosterone (T) concentrations in saliva and serum were measured in 53 women with various degrees of hirsutism and hyperandrogenism. The bioavailability of T was judged by comparing the correlations among the grade of hirsutism, salivary testosterone (SaT), and serum total and free T (fT) and sex hormone-binding globulin (SHBG) levels. The effect of body mass index on the correlations was also studied. The high SaT concentrations [mean, 237.6 +/- 66.7 (+/- SD) pmol/L] compared to the serum fT concentrations (mean, 29.1 +/- 11.8 pmol/L) in hirsute women may reflect the bioavailability of albumin-bound T or an ability of the salivary glands to metabolize steroids. SaT was more closely related to the T/SHBG ratio (mean, 82.5 X 10(-3) +/- 54.8), reflecting the non-SHBG-bound fraction of T, than to serum fT, which might support the former theory. SaT correlated better to the degree of hirsutism (rho = 0.45; P less than 0.01) than did any of the serum T parameters or SHBG. The correlation between SaT and hirsutism was partly dependent on the effect of body mass index. After eliminating this effect, SaT still correlated with hair growth on the total body area (rho = 0.36; P less than 0.05). On the basis of the results, SaT seems to relate to the bioavailable fraction of the hormone and, thus, appears to be an optimal method for studying hirsute women.

Adolescent↗
PubMed1995

A prospective randomized trial comparing finasteride to spironolactone in the treatment of hirsute women.

Enhanced 5 alpha-reductase activity has been found in the skin of the majority of women with hirsutism. Finasteride is a specific competitive inhibitor of 5 alpha-reductase, preferentially inhibiting the type 2 isoenzyme. Therefore, we randomly assigned 14 hirsute women in a 2:1 ratio to 1 of 2 treatment arms: 1) finasteride (F) treatment (n = 9; 5 mg, orally, daily), or 2) spironolactone (S) treatment (n = 5; 100 mg, orally, daily). Each group was treated for 6 months. Patients were evaluated at baseline and after 3 and 6 months of treatment. The 2 groups were similar in age, weight, hip/waist ratio, baseline Ferriman-Gallwey score (F, 19 +/- 2; S, 19 +/- 2), and baseline androgen levels. Finasteride treatment resulted in a significant increase in testosterone (T; P < 0.01) and the T/dihydrotestosterone ratio (P < 0.01). Finasteride caused a significant decrease in 5 alpha-androstane-3 alpha,17 beta-diol glucuronide (3 alpha-diolG; P < 0.05), the 3 alpha-diolG/T ratio (P < 0.01), and the 3 alpha-diolG/androstenedione ratio (P < 0.05). All changes were consistent with 5 alpha-reductase inhibition. In contrast, spironolactone treatment did not result in significant changes in serum hormone levels. Both treatments produced a significant decrease in anagen hair diameters [F, -14.0 +/- 6.7% (P < 0.05); S, -13.4 +/- 3.8% (P < 0.05)] and Ferriman-Gallwey scores [F, -2.1 +/- 0.4 (P < 0.05); S, -2.5 +/- 0.7 (P < 0.05)]. In conclusion, despite significantly different effects on androgen levels, finasteride and spironolactone treatment resulted in a similar clinical effect on hirsutism. Both caused significant, but limited, improvement in hirsutism. Although promising, further studies with finasteride are needed to verify its effectiveness as a treatment for hirsutism. Such studies will also provide a better understanding of the relative contribution of 5 alpha-reductase isoenzymes to hirsutism.

Adolescent↗
PubMed1999

Androgen receptor-mediated hypersensitivity to androgens in women with nonhyperandrogenic hirsutism: skewing of X-chromosome inactivation.

Idiopathic hirsutism may result from an increase in the androgen receptor (AR)-mediated sensitivity of the hair follicle. The AR gene is located on the X-chromosome and contains a highly polymorphic trinucleotide repeat (CAGn) in its first exon, whose length and methylation pattern affect both AR expression and function. We analyzed these CAG repeats in the genomic DNA from 16 nonhyperandrogenic hirsute patients (Ferriman score: 16 +/- 4.7, mean +/- SD) and 10 normal controls (Ferriman score: 3 +/- 1.4), who were similar in their hormonal profiles. We found no difference in the number of CAG repeats between hirsute patients and controls, and no correlation between number of repeats and the Ferriman score or hormonal values. However, after DNA digestion with methylation-sensitive HpaII and measurement of the optical density, we found a marked decrease in the hirsute group (P < 0.0001), which was greater than in the control group (P = 0.0003). In addition, in the hirsute patients, the shorter of the two alleles was preferentially less methylated (P = 0.007), suggesting skewing of X-chromosome inactivation in the patients but not in the controls. When the mean optical density of both alleles was correlated with the Ferriman score, we observed a significant negative correlation (P = 0.02, r = -0.45), which became stronger when the shorter alleles were analyzed separately (P = 0.01; r = 0.48). We conclude that nonhyperandrogenic hirsutism is associated with skewing of X-chromosome inactivation in peripheral blood lymphocytes. This leads to the longer of the two AR alleles being preferentially methylated, allowing for the shorter (and presumably, more functional) allele to be expressed on the active X-chromosome. Further studies need to be performed to investigate whether this phenomenon is present in androgen-sensitive tissues in these patients.

Adult↗
PubMed1999

A prospective randomized trial comparing low dose flutamide, finasteride, ketoconazole, and cyproterone acetate-estrogen regimens in the treatment of hirsutism.

Sixty-six hirsute women were randomized and treated with 1) flutamide (n = 15), 250 mg/day; 2) finasteride (n = 15), 5 mg/day; 3) ketoconazole (n = 16), 300 mg/day; and 4) ethinyl estradiol (EE)-cyproterone acetate (CPA; n = 20), 0.01 mg EE/day for the first week, 0.02 mg EE/day for the second week, and 0.01 mg EE/day for the third week, followed by a pause of 7 days, then 12.5 mg CPA/day added during the first 10 days of every month for 12 months. Hirsutism was evaluated by the Ferriman-Gallwey score, and hair diameter and hair growth rate were determined by a special image analysis processor in basal conditions and after 90, 180, 270, and 360 days of treatment. All treatments produced a significant decrease in the hirsutism score, hair diameter, and daily hair growth rate: flutamide, -55 +/- 13%, -21 +/- 14%, and -37 +/- 18%; finasteride, -44 +/- 13%, -16 +/- 12%, and -27 +/- 14%; ketoconazole, -53 +/- 18%, -14 +/- 12%, and -30 +/- 21%; and EE-CPA, -60 +/- 18%, -20 +/- 11%, and -28 +/- 21%. Some differences existed among treatments with regard to effectiveness; EE-CPA and flutamide seem to be the most efficacious in improving hirsutism. For the hirsutism score, a greater decrease was seen with EE-CPA (-60 +/- 18%) than with finasteride (-44 +/- 13%; P < 0.01) and a greater decrease was seen with flutamide (-58 +/- 18%) than with finasteride (-44 +/- 13%; P < 0.05). Flutamide is the fastest in decreasing hair diameter; EE-CPA is the fastest in slowing down hair growth, even though at the end of the treatment there was a significant difference between flutamide and finasteride only (-41 +/- 18% vs. -27 +/- 14%; P < 0.05). Flutamide, ketoconazole, and EE-CPA induced a significant decrease in total and free testosterone, 5alpha-dihydrotestosterone, dehydroepiandrosterone, dehydroepiandrosterone sulfate, and androstenedione plasma levels. During the EE-CPA treatment, gonadotropins were suppressed, and the sex hormone-binding globulin level increased. Finasteride induced a decrease in dehydroepiandrosterone sulfate and 5alpha-dihydrotestosterone and an increase in testosterone levels. Very few side-effects were observed during treatment with low doses of flutamide, EE-CPA, and particularly finasteride. Flutamide induced a decrease whereas EE-CPA induced an increase in triglycerides and cholesterol, showing higher values within the normal range. Ketoconazole induced several side-effects and complications, and several people dropped out of the study. Despite different modalities of action and significantly different effects on androgen levels, low doses of flutamide, finasteride, and EE-CPA constitute very satisfactory alternative therapeutic regimens in the treatment of hirsutism.

Adult↗
PubMed2005

Spironolactone for hirsutism in polycystic ovary syndrome.

OBJECTIVE: To evaluate the level of evidence for treatment of hirsutism associated with polycystic ovary syndrome (PCOS) with spironolactone. DATA SOURCES: Studies and reports were located in MEDLINE (1966-January 2005), EMBASE, and International Pharmaceutical Abstracts through the second week of January 2005. DATA SYNTHESIS: Hirsutism is a common clinical problem and is often associated with PCOS. Research has been performed to assess the efficacy of spironolactone therapy in patients with hirsute characteristics. Five studies that evaluated the effectiveness of spironolactone for hirsutism in PCOS were identified and reviewed. CONCLUSIONS: The effects of multiple treatment options on the subjective and objective measures of hirsutism have displayed varying results. The outcomes reported to date have shown a positive trend toward using spironolactone in women with PCOS and hirsutism.

Female↗
PubMed2004

A comparison between spironolactone and spironolactone plus finasteride in the treatment of hirsutism.

OBJECTIVE: To compare the clinical efficacy and safety of the combination of spironolactone (100 mg/day) plus finasteride (5 mg/day) and spironolactone (100 mg/day) alone in the treatment of hirsutism. PATIENTS AND MEASUREMENTS: Sixty-five hirsute women were randomly assigned to one of these two treatment groups. Hirsutism score was measured according to the modified Ferriman-Gallwey scoring system. Baseline and post-treatment assessments were carried out in each subject by an investigator blinded to the therapy. Both groups had similar demographic properties at baseline. The serum levels of total and free testosterone, dehydroepiandrosterone sulphate and sex hormone-binding globulin were measured at the beginning and after 1 year of therapy. Blood chemistry and side-effects were evaluated during the study. Hirsutism scores were decreased significantly in both groups at the end of the year. RESULTS: The mean percentage change in hirsutism scores from baseline in the spironolactone plus finasteride-treated group (51.3%) was significantly (P<0.005) higher than in the group treated with spironolactone alone (36.6%). Patients from both treatment groups experienced similar side-effects. CONCLUSIONS: We have concluded that a combination of spironolactone plus finasteride is a safe and effective therapy in the treatment of hirsutism.

Adult↗
PubMed2000

Treatment of hirsutism with lowest-dose flutamide (62.5 mg/day).

Flutamide has been used successfully in the treatment of hirsutism. However, flutamide may cause important side-effects. The aim of this study was to evaluate the clinical and hormonal effects of lowest-dose flutamide therapy. Sixty-one women with hirsutism aged 18-37 years (mean +/- SD 23.4 +/- 5.9 years) were included in the study. Patients received 62.5 mg flutamide once per day for a period of 12 months. A hirsutism score was determined according to a modified Ferriman-Gallwey scoring system. Before therapy, multiscreen blood chemistry, hormonal analysis and complete blood counts were performed. These parameters and hirsutism scores were repeated at 3, 6, 9 and 12 months during therapy. The modified Ferriman-Gallwey scores significantly decreased from 19.1 +/- 4.9 to 5.8 +/- 3.3 during the study (p < 0.0001). The percentage reductions in hirsutism scores (mean +/- SD) were 60.3 +/- 14.4% at 6 months, and 70.3 +/- 13.2% at 12 months. No significant side-effects or modifications in the menstrual cycles were observed. There were no significant differences in any of the hormone levels during therapy. In conclusion, the lowest dose of flutamide, 62.5 mg/day, is a well-tolerated therapeutic agent and can be used in the treatment of hirsutism.

Adolescent↗
PubMed1992

Low ratio of androstenedione to testosterone in plasma and saliva of hirsute women.

To gain insight into the passage of androstenedione (A4) through the salivary gland, we measured concentrations of plasma total (TA4), free (FA4), and salivary (SA4) androstenedione during administration of dexamethasone and synthetic corticotropin to control subjects and hirsute women and compared these data with plasma total (TT), free (FT), and salivary testosterone (ST) concentrations. TA4 and FA4 were significantly lower in control subjects throughout (0, 15, 45, and 75 min) (P less than 0.05). SA4 in control subjects was significantly lower only in the follicular phase at 0 and 15 min. There was no significant difference between the increments in SA4 in response to corticotropin. The concentration of SA4 in the control women at 0 min was not significantly different from the concentration of FA4. At 45 and 75 min after corticotropin administration, however, SA4 was slightly higher than FA4 (P less than 0.01). In hirsute women, however, the concentration of SA4 was significantly lower than FA4 at all times (P less than 0.05). TT, FT, and ST concentrations were about twofold higher in the hirsute women than in control subjects throughout. In both groups, ST concentrations were three times as high as FT concentrations (P less than 0.001). The SA4:ST ratio was significantly lower than the FA4:FT ratio in both groups (P less than 0.001) because of higher ST than FT concentrations and similar or even lower SA4 concentrations in both groups. Both FA4:FT and SA4:ST ratios were lower in hirsute women, except for the FA4:FT ratio in control subjects in the luteal phase. Our data are compatible with 17-hydroxysteroid oxidoreductase activity in the salivary gland. If the difference in the ratios of A4 to T between hirsute women and control subjects is attributed to this hypothetical enzymatic activity, it would suggest a more rapid conversion of A4 to T in the hirsute group.

Adrenocorticotropic Hormone↗
PubMed1991

[Hyperprolactinemia and hypophyseal hypothyroidism as cofactors in hirsutism and androgen-induced alopecia in women].

A more comprehensive hormonal diagnosis than has previously been performed shows that androgen-dependent diseases of hair growth are due to more varied hormonal disturbances than elevated androgen serum levels alone. In 46 female patients with androgenic hair loss and 27 patients with hirsutism, the levels of the androgens testosterone, free testosterone, androstenedione, dehydroepiandrosterone sulphate and 17-hydroxyprogesterone and of sex hormone-binding globulin, cortisol, oestradiol and the hypophyseal hormones follicle-stimulating hormone and luteinizing hormone were determined and compared with the hormone levels of 27 female patients without endocrine disorders. Of the androgens, only androstenedione showed a slightly significant elevation in hirsutism. Cortisol was elevated significantly in androgenic hair loss, and with a low degree of significance in hirsutism. In view of the complex hormonal interactions of thyroxin, prolactin and androgens and thyroid hormones the thyrotropin-releasing hormone (TRH)-stimulation test was performed in 38 female patients with androgenic hair loss and 27 with hirsutism, and the results were compared with those recorded in 45 female control persons. The test is based on feedback mechanisms between hypothalamic TRH and hypophyseal TSH and prolactin and peripheral thyroid hormones. Baseline concentrations of TSH prior to stimulation were significantly elevated in hirsutism, while in androgenic hair loss both baseline and stimulated TSH levels were significantly elevated; thus, hypothyroidism is a significant finding in both clinical pictures. In the case of prolactin, both baseline and stimulated levels were highly significantly elevated in hirsutism, while in androgenic hair loss the stimulated levels were significantly elevated.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1982

Testosterone : testosterone estradiol binding globulin ratio in evaluating hirsute women.

Testosterone : testosterone estradiol binding globulin (TEBG) ratio is evaluated in 35 hirsute women (age 12 to 38 years), and 14 healthy nonhirsute normal controls (age 21 to 40 years). This is an attempt to emphasize the significance of TEBG in evaluating hirsute women. This is a significant negative linear correlation between the weight of the patients and TEBG concentrations (r = 0.414, P less than 0.05). There is negative linear correlation between TEBG and serum free testosterone concentration that reached significance only in the hirsute nonobese. T:TEBG ratio is significantly high in hirsute women compared to the controls. T:TEBG ratio shows a significant positive linear correlation with free testosterone concentration. In addition, T:TEBG ratio is a direct simple method of evaluating hirsute women. Demulen and Dexamethazone treatment produced a significant fall in T:TEBG ratio in hirsute women.

Adolescent↗
PubMed1982

Treatment of hirsutism with spironolactone.

Clinical and endocrine evaluations of 39 patients with hirsutism were performed to determine the effectiveness and site(s) of action of an antiandrogenic compound, spironolactone. Treatment with spironolactone at a dose of 200 mg/day resulted in a clear beneficial effect on the quantity and quality of facial hair growth in 19 of 20 patients with moderate to severe hirsutism. Regression of hirsutism in terms of diameter, density, and the rate of facial hair growth was noticeable within two months. The maximal effect was observed at six months and was maintained at 12 months of treatment. Spironolactone was equally effective in reducing hirsutism in women with polycystic ovary syndrome and idiopathic hirsutism. These clinical observations were associated with a prompt and sustained reduction in levels of androgen of ovarian origin without affecting levels of adrenal androgen and cortisol. Apart from diuresis, which was limited to the first few days of treatment, there were no discernible side effects the one year of this study. Our results indicate that spironolactone is a highly effective and safe agent for the treatment of hirsutism through its inhibitory action on both ovarian androgen secretion and peripheral androgen action.

Amenorrhea↗
PubMed1993

[3 beta-hydroxysteroid dehydrogenase defect: frequency of presentation in a sample of Chilean hirsute women].

The defect of 3 beta hydroxysteroid dehydrogenase (3 beta HSD) is frequent among hirsute women and clearly dependent on the ethnic composition of the studied population. Our aim was to study the frequency of 3 beta HSD deficit in a group of Chilean hirsute women. Basal and post ACTH concentrations of cortisol, 17 hydroxyprogesterone and 17 hydroxypregnenolone were measured by RIA in 40 hirsute post puberal women and in 15 normal age matched female volunteers. Criteria for considering a 3 beta HDS deficit were 17 hydroxypregnenolone values and 17 hydroxypregnenolone/17 hydroxyprogesterone and 17 hydroxypregnenolone/cortisol ratios after ACTH stimulation over the 95% confidence intervals of normal women. Basal dehydroepiandrosterone sulphate and testosterone levels were also measured in hirsute women. All samples were obtained during the follicular phase of the menstrual cycle. ACTH stimulated hormone values and ratios were diagnostic for 3 beta HDS deficit in 7.5% of hirsute women. Basal testosterone was over 80 ng/dl in 47.5% and dehydroepiandrosterone sulphate over 3.9 micrograms/ml in 52.5% of these women. There was no correlation between dehydroepiandrosterone or testosterone values and ACTH stimulated hormone values. It is concluded that 3 beta HSD is frequent in hirsute women and that its diagnosis requires the determination of ACTH stimulated 17 hydroxypregnenolone values and 17 hydroxypregnenolone/17 hydroxyprogesterone ratio.

17-alpha-Hydroxypregnenolone↗
PubMed1993

[Hirsutism due to a late-onset of hydroxylase-21 deficit: apropos of 4 cases].

OBJECTIVE: To present four cases of Hirsutism due to a late-starting deficit of Hydroxylase-21. DESIGN: Retrospective, descriptive study. SETTING: Primary Care team. PATIENTS AND OTHERS PARTICIPANTS: Four women (21, 22, 32 and 36 years old) were studied because they presented hirsutism. MEASUREMENTS AND MAIN RESULTS: All the patients had begun to be hirsute at puberty. Three had regular cycles and one, irregular. Two women (32 and 36) had had various children. In all the cases the hirsutism was moderate. One woman suffered severe acne. Basal testosterone was only high in one case; DHEA-S in two. The values of basal 17-OH-Progesterone were high in three women. The ACTH stimulation test showed positive for three patients: it was not performed on the fourth due to her very high levels of 17-OH-Progesterone. CONCLUSIONS: Late-starting Hydroxylase-21 deficit is a frequent cause of hirsutism in Primary Care. It must be considered and investigated as a cause when faced with a clinical picture compatible with benign hirsutism, in order to advise the woman on the best treatment and avoid iatrogenic attitudes.

Adrenal Hyperplasia, Congenital↗
PubMed1997

Late onset adrenal hyperplasia due to 3 beta-hydroxy-delta 5-steroid dehydrogenase deficiency in north Indian hirsute women.

The presence of late onset 3 beta-hydroxy steroid dehydrogenase (3 beta-HSD) type of congenital adrenal hyperplasia was studied in 58 north Indian hirsute women. The age range of these patients was 15 to 42 yr. Fifty two per cent of these patients had body mass index > 25. Basal serum testosterone, luteinizing hormone, follicle stimulating hormone, dehydroepiandrosterone sulphate (DHEAS), and 17 hydroxy progesterone (17 OHP) were estimated. All the patients underwent adrenocorticotropin (ACTH) stimulation test after an overnight dexamethasone suppression for the estimation of DHEAS, 17 OHP, and 17 hydroxy pregnenolone (delta 5-17p). Five (8.6%) hirsute women showed an exaggerated 17 OHP response to ACTH indicating 21-hydroxylase deficiency. Eight (13.8%) hirsute women had elevated basal DHEAS and ACTH-stimulated DHEAS as well as delta 5-17P responses indicative of 3 beta-HSD deficiency. In one patient hirsutism was the presenting manifestation of tumoural hyperandrogenism. Our findings indicate the presence of both 21-hydroxylase and 3 beta-HSD deficiency in north Indian hirsute women, with, 3 beta-HSD deficiency being the major cause of hirsutism in this population.

Adolescent↗