cyproterone and Adrenal-Hyperplasia--Congenital

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adult; Child; Cyproterone; Cyproterone Acetate; Desoxycorticosterone; Dexamethasone; Drug Therapy, Combination; Female; Fludrocortisone; Genetic Carrier Screening; Haploidy; HLA Antigens; Humans; Hydrocortisone; Infant, Newborn; Male; Prednisone; Pregnancy; Prenatal Diagnosis

The endocrine abnormalities associated with the development of androgen-dependent hirsutism have been presented and discussed in the light of the frequent finding of ultrasonographic abnormalities suggestive of polycystic ovary syndrome. A simple protocol of investigation, which should ideally include pelvic ultrasonography, has been presented. The treatment of hirsutism by combined anti-androgen and oestrogen therapy has been detailed and other approaches discussed.

Topics: Adrenal Glands; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Androgens; Cimetidine; Cyproterone; Female; Hair; Hair Removal; Hirsutism; Humans; Polycystic Ovary Syndrome; Spironolactone; Ultrasonography

Polycystic ovarian disease (PCOD) was first described as a single disease by Stein and Leventhal in 1935, but now has been separated into several distinct entities, comprising a symptom complex. The most frequent presenting symptoms associated with PCOD are obesity, hirsutism, amenorrhea or anovulation, dysfunctional uterine bleeding, irregular menses, and infertility. The common finding of hirsutism in PCOD patients is a reflection of the hyperandrogenism resulting from elevation of all the androgens, including testosterone, androstenediol, dehydroepiandrostrone sulfate (DHEA-S), and androstenedione. Some patients with all the clinical features of PCOD can be shown, through appropriate testing, to have an attenuated form of classic congenital adrenal hyperplasia (CAH). Serum follicle stimulating hormone (FSH) levels are usually low or in the normal range, and serum luteinizing hormone (LH) levels are usually elevated in patients with PCOD, resulting in an altered LH/FSH ratio. Treatment for PCOD must be based on the needs and desires of the individual patient, and on the pathophysiology of the patient's particular abnormalities. When pregnancy is desired, ovulation induction with clomiphene is indicated. Clomiphene is a weak estrogen that induces a transient rise in serum LH and FSH, followed by a gonadotropic pattern similar to normal cycles. A 72% ovulation rate and a 41.8% conception rate have been reported after treatment with clomiphene. In patients who do not respond to clomiphene, or clomiphene with added human chorionic gonadotropin (hCG), human menopausal gonadotropin (hMG) can be used to induce ovulation, but the patient should be closely monitored for multiple ovulation, multiple pregnancy, or hyperstimulation syndrome. For patients not interested in conception, regular menstrual cyclicity can be restored and hyperandrogenism reduced with oral contraceptives (OCs).

Topics: Adrenal Hyperplasia, Congenital; Androgens; Chorionic Gonadotropin; Cimetidine; Clomiphene; Contraceptive Agents, Female; Contraceptive Agents, Male; Cyproterone; Cyproterone Acetate; Female; Follicle Stimulating Hormone; Humans; Luteinizing Hormone; Medroxyprogesterone; Medroxyprogesterone Acetate; Ovary; Ovulation Induction; Polycystic Ovary Syndrome; Spironolactone

Topics: Acne Vulgaris; Adolescent; Adrenal Hyperplasia, Congenital; Adult; Alopecia; Androgens; Cyproterone; Cyproterone Acetate; Female; Hair; Hirsutism; Humans; Male; Polycystic Ovary Syndrome; Racial Groups; Sex Characteristics; Stress, Psychological; Syndrome

The ongoing development and gradual availability of the new anti-androgens hold exciting clinical implications for the future. The biosynthesis and interchangeability of the sex steroids, the roles of the ovaries and adrenals and the value and interpretation of biochemical assays in clinical practice are briefly discussed. Because the anti-androgens are used primarily for seborrhoea/acne/hirsutism/oligomenorrhoea, the pathophysiological basis of this socially debilitating syndrome is discussed. The classification of the anti-androgens, their indications, side-effects, dosage schemes and results of treatment are reviewed. Finally, a summary of a possible clinical management regimen is presented.

Topics: 17-Ketosteroids; Acne Vulgaris; Adrenal Hyperplasia, Congenital; Androgen Antagonists; Androgens; Androsterone; Contraceptives, Oral, Hormonal; Cyclopentanes; Cyproterone; Dermatitis, Seborrheic; Dihydrotestosterone; Estradiol Congeners; Female; Follicle Stimulating Hormone; Glucocorticoids; Hirsutism; Humans; Hydroxysteroids; Luteinizing Hormone; Male; Medroxyprogesterone; Nandrolone; Prostatic Neoplasms; Receptors, Androgen; Sex Hormone-Binding Globulin; Steroids; Testosterone

Topics: Adolescent; Adrenal Hyperplasia, Congenital; Age Determination by Skeleton; Androgens; Child; Child Development; Child, Preschool; Chlormadinone Acetate; Choriocarcinoma; Chorionic Gonadotropin; Corpus Luteum Hormones; Cyproterone; Estradiol; Estrogens; Female; Follicle Stimulating Hormone; Gonadotropins, Pituitary; Growth Disorders; Hormones, Ectopic; Humans; Hypothyroidism; Luteinizing Hormone; Pregnancy; Pseudohypoparathyroidism; Puberty, Precocious; Sertoli Cell Tumor; Sex Factors; Testosterone

Thirty late-onset adrenal hyperplasia patients consulting for isolated hirsutism were randomly divided into two groups; group 1 (n = 16) was treated with hydrocortisone in order to suppress androgen adrenal secretion, and group 2 (n = 14) received cyproterone acetate (CPA) antiandrogen therapy to inhibit peripheral androgen activity. The clinical and hormonal effects of each type of treatment were evaluated. Before treatment, the clinical and hormonal profiles of the two patient groups did not differ significantly. Excellent clinical evolution in terms of the regression of hirsutism was observed in the CPA-treated patients (54% decrease in the clinical score in 1 yr), in contrast with the slight decrease in hirsutism (26%) after hydrocortisone treatment. In hydrocortisone-treated patients, plasma androgen decreased to normal levels: testosterone from 3.05 +/- 1.45 to 1.46 +/- 0.42 nmol/L and delta 4-androstenedione from 13.6 +/- 4.1 to 6.33 +/- 1.47 nmol/L. Conversely, in CPA-treated patients, only a slight decrease in testosterone from 2.98 +/- 1.98 to 2.29 +/- 0.64 nmol/L and in delta 4-androstenedione from 12.9 +/- 5.9 to 9.86 +/- 2.23 nmol/L was observed. This slight decrease in plasma androgens contrasts with the rapid clinical improvement after CPA. These results emphasize the importance of peripheral receptivity to androgens in the clinical expression of hyperandrogenism. Moreover, they indicate that peripheral antiandrogen therapy may be more appropriate in late-onset adrenal hyperplasia patients than conventional adrenal inhibition using cortisone therapy.

Topics: Adolescent; Adrenal Hyperplasia, Congenital; Adult; Androstenedione; Cyproterone; Cyproterone Acetate; Estradiol; Female; Hirsutism; Humans; Hydrocortisone; Testosterone

In patients with adrenal hirsutism or enzyme deficiencies in steroidogenesis, elevated adrenal androgens could be normalized by dexamethasone. We were interested to see if dexamethasone would be as effective as cyproterone acetate in treating hirsutism in selected patients with adrenal pathogenesis. Therefore 28 patients with hirsutism of adrenal origin or enzyme deficiency were treated cyclically either with cyproterone acetate and ethinylestradiol (2 mg cyproterone acetate + 0.035 mg ethinyl-estradiol days 1-21, +10 mg cyproterone acetate days 1-15) (n = 15) or with 0.25-0.5 mg dexamethasone daily at 10 pm (n = 13). In the dexamethasone group there was a significant drop in dehydroepiandrosterone and dehydroepiandrosterone sulfate levels within 9 months, but there was a diminution in hirsutism in only four women (31%); in four out of seven menstrual irregularities decreased. In the cyproterone acetate group hirsutism diminished significantly in 66% (n = 10) without suppression of adrenal androgens. Weight gain occurred in a few cases in both groups; other side effects developed in 33% in the cyproterone acetate group. Preselection of patients with hirsutism is useful with respect to diagnosis; adrenal pathogenesis should not generally indicate dexamethasone treatment of hirsutism unless there is a desire for pregnancy, because cyproterone acetate is a more powerful agent in reducing hair growth.

Topics: 3-Hydroxysteroid Dehydrogenases; Adrenal Hyperplasia, Congenital; Adult; Androgen Antagonists; Cyproterone; Cyproterone Acetate; Dexamethasone; Female; Gonadal Steroid Hormones; Hirsutism; Humans; Steroid Hydroxylases

Topics: Adrenal Hyperplasia, Congenital; Age Determination by Skeleton; Androgen Antagonists; Child; Cyproterone; Drug Therapy, Combination; Female; Fludrocortisone; Flutamide; Growth; Humans; Hydrocortisone; Mineralocorticoid Receptor Antagonists; Spironolactone; Testolactone

Topics: Adrenal Hyperplasia, Congenital; Buserelin; Child; Cyproterone; Cyproterone Acetate; Dexamethasone; Female; Humans; Male; Medroxyprogesterone; Medroxyprogesterone Acetate; Puberty, Precocious; Virilism

We have compared three treatments of congenital adrenal hyperplasia (CAH) for their effect on physical development. Thirteen girls and two boys with CAH due to 21-hydroxylase deficiency were treated with three different treatments, hydrocortisone (HC), dexamethasone (DXM) and cyproterone acetate (CA). The results showed that height growth was better with HC and CA than DXM, and bone excessive maturation was more suppressed with DXM and CA than HC. A dose-dependent relationship was revealed between body weight and dose of HC. Iatrogenic obesity was found in 42.9% and 38.1% of the patients treated with DXM and HC, but none of the patients treated with CA did became obese. Physical growth was better with CA treatment than HC or DXM treatment, but CA may have a suppressive effect on the pituitary-adrenal axis observed carefully, especially on prepubertal and pubertal cases.

Topics: Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Body Height; Bone Development; Child, Preschool; Cyproterone; Cyproterone Acetate; Dexamethasone; Female; Follicle Stimulating Hormone; Humans; Hydrocortisone; Infant; Infant, Newborn; Luteinizing Hormone; Male; Obesity; Pregnanetriol

We describe three patients in whom at the age of 4-7 yrs the diagnosis adrenogenital syndrome (21-hydroxylase deficiency) was made. There was no salt-loss. Two boys presented with precocious pubic hair development and increased growth velocity and bone maturation. The third patient was a severely virilized girl raised as a boy until she was 4 yrs old. Following an extensive psychiatric evaluation, it was decided to raise her as a girl and feminoplasty was performed. Clinical and biochemical evidence of central precocious puberty was present in one boy at the time of diagnosis at age 7 and developed under hydrocortisone substitution therapy in the two other patients. The precocious puberty was treated in two patients with the anti-androgen cyproterone acetate. In one boy suppression of pituitary gonadotropin secretion was obtained by LH-RH analogue (Buserelin) treatment.

Topics: Adrenal Hyperplasia, Congenital; Buserelin; Child; Child, Preschool; Cyproterone; Cyproterone Acetate; Disorders of Sex Development; Female; Genitalia; Humans; Hydrocortisone; Male; Puberty, Precocious

Topics: Adrenal Hyperplasia, Congenital; Androgen Antagonists; Androgens; Cyproterone; Cyproterone Acetate; Dehydroepiandrosterone; Dehydroepiandrosterone Sulfate; Drug Combinations; Ethinyl Estradiol; Female; Humans; Infertility, Female; Testosterone

Twenty six females suffering from congenital adrenal hyperplasia due to 21-hydroxylase-deficiency with and without salt wasting were evaluated to determine their growth patterns with special regard to their pubertal growth spurt. 17 patients have been substituted with hydrocortisone 20-25 mg/m2 and if necessary with 9 alpha-fluoro-cortisol 0.025-0.15 mg (group 1), 9 patients have got an additional cyproterone-acetate-therapy during their pubertal development (group 2). Group 1: A pubertal growth spurt was found in 14 females (82.4%) with a peak height velocity of 6.2 +/- 1.6 (SD) cm/yr. At the beginning of puberty the early treated girls showed a progressing retardation of bone age. Therefore the peak height velocity based on advancing bone age was elevated up to 8.7 +/- 1.4 (SD) cm/yr bone age (p = 0.01). Salt wasting (n = 10) did not seem to influence the pubertal growth velocity and the pubertal growth spurt (p = 0.1, p greater than 0.1), but the growth until cessation which was found to be markedly decreased (p less than 0.1). Group 2: Cyproterone-acetate suppressed symptoms of puberty but did not extinguish growth spurt. During 6.3 +/- 1.3 (SD) yrs of treatment bone age advanced only 4.2 +/- 0.7 (SD) yrs. However, in this period height increased by 20.8 +/- 6.4 (SD) cm which corresponds to a mean height velocity of 3.3 +/- 0.8 (SD) cm/yr, and 4.9 +/- 0.8 (SD) cm/yr bone age. Furthermore at puberty peak height velocity results in 4.5 +/- 0.7 (SD) cm/yr and in 10.4 +/- 4.6 (SD) cm/yr bone age.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adolescent; Adrenal Hyperplasia, Congenital; Age Determination by Skeleton; Body Height; Child; Cyproterone; Cyproterone Acetate; Drug Therapy, Combination; Female; Humans; Puberty; Steroid Hydroxylases

Topics: Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Androgen Antagonists; Androgens; Bromocriptine; Cimetidine; Cyproterone; Cyproterone Acetate; Drug Combinations; Estradiol Congeners; Female; Hirsutism; Humans; Polycystic Ovary Syndrome; Progesterone Congeners; Spironolactone; Thyronines; Time Factors

Topics: Adrenal Hyperplasia, Congenital; Androgens; Contraceptives, Oral; Cyproterone; Female; Hair Removal; Hirsutism; Humans; Ovarian Cysts; Testosterone

Topics: Adolescent; Adrenal Hyperplasia, Congenital; Body Height; Child; Child, Preschool; Cyproterone; Cyproterone Acetate; Female; Humans; Infant; Japan; Male; Puberty, Precocious; Sexual Maturation; Surveys and Questionnaires

Topics: Acne Vulgaris; Adolescent; Adrenal Hyperplasia, Congenital; Adult; Androgen Antagonists; Cyproterone; Female; Hirsutism; Humans; Polycystic Ovary Syndrome; Testosterone

Topics: Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Androgen Antagonists; Contraceptives, Oral; Cushing Syndrome; Cyproterone; Cyproterone Acetate; Dexamethasone; Female; Hair Removal; Hirsutism; Humans; Sex Hormone-Binding Globulin; Stimulation, Chemical

Five females with idiopathic hirsutism, ten with adrenogenital syndrome, four with acne vulgaris, and three with Stein-Leventhal syndrome were treated for nine months with large or small doses of cyproterone acetate (100 or 2 mg daily, respectively) in combination with oestrogen. The drug had a good effect on the acne vulgaris and moderated the hirsutism. In the various patient groups the serum testosterone level was decreased to between 58.6% (+/- 28.7% S.E.) and 10.8% (+/- 8.2% S.E.) of the basal value. No essential difference in effectiveness was observed between the large and small doses. Based on study of the side-effects, it is recommended that laboratory liver tests are used for control.

Topics: 17-Ketosteroids; Acne Vulgaris; Adolescent; Adrenal Hyperplasia, Congenital; Adult; Alanine Transaminase; Aspartate Aminotransferases; Cyproterone; Cyproterone Acetate; Female; Hirsutism; Humans; Hydrocortisone; Polycystic Ovary Syndrome; Testosterone

Topics: Acne Vulgaris; Adolescent; Adrenal Cortex Function Tests; Adrenal Hyperplasia, Congenital; Child; Cyproterone; Cyproterone Acetate; Dose-Response Relationship, Drug; Female; Hirsutism; Humans; Male; Paraphilic Disorders; Puberty, Precocious

Topics: Adrenal Hyperplasia, Congenital; Adult; Cyproterone; Estrogens; Female; Hair Removal; Hirsutism; Humans; Middle Aged; Progesterone; Racial Groups

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Androgen Antagonists; Body Height; Child; Child, Preschool; Cyproterone; Follicle Stimulating Hormone; Gonadotropins, Pituitary; Growth Hormone; Humans; Luteinizing Hormone; Pregnadienes; Puberty, Precocious; Testosterone

Topics: Acromegaly; Adrenal Hyperplasia, Congenital; Anabolic Agents; Androgen Antagonists; Animals; Cushing Syndrome; Cyproterone; Estrogens; Female; Gerbillinae; Humans; Hypopituitarism; Laryngeal Diseases; Male; Polycystic Ovary Syndrome; Pregnadienes; Sebaceous Glands; Skin; Testosterone