17-ketosteroids and Androgen-Insensitivity-Syndrome

This paper has been designed to review some current concepts about biochemistry, pathophysiology and clinics of androgen-binding proteins, both TeBG and cellular receptors. Some important experimental models to clarify the interactions between androgens and target cells are mentioned. Further, major clinical applications in which binding parameters were or will be of great importance are discussed in details.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Androgen-Binding Protein; Androgen-Insensitivity Syndrome; Breast Neoplasms; Carrier Proteins; Danazol; Disorders of Sex Development; Female; Humans; Hyperthyroidism; Infertility, Male; Liver Cirrhosis; Male; Minoxidil; Receptors, Androgen; Sex Hormone-Binding Globulin; Spironolactone

The testicular feminization syndrome (TFS) in its complete form results in total feminization due to a nuclear inaction of androgens, and the female role should be supported with postpubertal orchiectomy to avoid the risk of malignancy. Incomplete forms of the syndrome (ITFS) include Type I n which some degree of masculinization may be observed, prompting earlier gonadectomy, and Type II or pseudovaginal perineoscrotal hypospadias (PPSH) which is always characterized by pubertal masculinization, necessitating management and support of these patients as males. Other intersex abnormalities which must be differentiated include true hermaphroditism, the Swyer syndrome, males with 17-ketosteroid reductase deficiency, and Reifenstein's syndrome.

Topics: 17-Ketosteroids; Adolescent; Adult; Androgen-Insensitivity Syndrome; Androgens; Animals; Child; Diagnosis, Differential; Disorders of Sex Development; Female; Follicle Stimulating Hormone; Humans; Hydroxysteroid Dehydrogenases; Hypospadias; Infertility, Male; Male; Mice; Rats

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Androgen-Insensitivity Syndrome; Child; Child, Preschool; Chorionic Gonadotropin; Diagnosis, Differential; Disorders of Sex Development; Female; Genetic Counseling; Genitalia, Female; Genitalia, Male; Humans; Hypospadias; Infant; Klinefelter Syndrome; Male; Mosaicism; Pregnanetriol; Progesterone; Psychosexual Development; Sex Chromosome Aberrations; Transsexualism; Turner Syndrome; Virilism

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex; Adrenocortical Hyperfunction; Androgen-Insensitivity Syndrome; Child; Cushing Syndrome; Endocrine Glands; Estrogens; Female; Follicle Stimulating Hormone; Humans; Hydrocortisone; Hypothalamo-Hypophyseal System; Luteinizing Hormone; Male; Menstruation; Ovary; Pituitary Gland, Anterior; Pituitary Hormones; Puberty; Puberty, Precocious; Reproduction; Turner Syndrome

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Androgen-Insensitivity Syndrome; Diagnosis, Differential; Disorders of Sex Development; Female; Follicle Stimulating Hormone; Genitalia, Female; Genitalia, Male; Gynecomastia; Humans; Hypospadias; Infant; Infant, Newborn; Infant, Newborn, Diseases; Infertility, Male; Karyotyping; Male; Sex Chromatin; Sex Determination Analysis; Testosterone; Virilism

Topics: 17-Ketosteroids; Acne Vulgaris; Androgen-Insensitivity Syndrome; Androgens; Female; Glucocorticoids; Hirsutism; Humans; Male; Methods; Polycystic Ovary Syndrome; Protein Binding; Secretory Rate; Skin; Testosterone

Topics: 17-Ketosteroids; Adrenocorticotropic Hormone; Androgen-Insensitivity Syndrome; Castration; Feedback; Humans; Hypogonadism; Klinefelter Syndrome; Leydig Cells; Male; Testis; Testosterone

Topics: 17-Ketosteroids; Androgen-Insensitivity Syndrome; Disorders of Sex Development; Estrogens; Follicle Stimulating Hormone; Humans; Male; Sex Chromosomes; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocorticotropic Hormone; Androgen-Insensitivity Syndrome; Androstanes; Castration; Female; Genitalia; Gonadotropins; Gonads; Humans; Pregnanes; Sterols; Testosterone; Tritium

Topics: 17-Ketosteroids; Adult; Androgen-Insensitivity Syndrome; Androgens; Androstanes; Carbon Isotopes; Castration; Chromatography, Paper; Clinical Trials as Topic; Computers; Feedback; Follicle Stimulating Hormone; Humans; Luteinizing Hormone; Male; Radioimmunoassay; Spectrum Analysis; Testosterone; Time Factors; Tritium; Ultraviolet Rays

Topics: 17-Ketosteroids; Acetates; Adenoma; Androgen-Insensitivity Syndrome; Androgens; Androsterone; Carbon Isotopes; Chemistry, Clinical; Chromatography, Paper; Clinical Trials as Topic; Cushing Syndrome; Etiocholanolone; Female; Hirsutism; Humans; Hyperaldosteronism; Male; Methods; Myoma; Puberty, Precocious; Testosterone; Tritium; Uterine Neoplasms

A 58-yr-old man presented with gynecomastia and elevated serum estrogens. The diagnosis of an estrogen-secreting adrenal tumor was made based upon the finding of a 4-cm left adrenal mass, elevated levels of estradiol in peripheral and left adrenal venous blood, and increased urinary 17-ketosteroids. In addition to marked elevations in estradiol and 17-ketosteroids there was an increased baseline level of 11-deoxycorticosterone and a slightly decreased level of 18-hydroxycorticosterone, suggesting the possibility of impaired P450c11 activity. The effect of ketoconazole administration (600 mg/day) for 4 weeks was studied. Urinary free cortisol and 17-ketosteroid excretion and serum testosterone levels fell acutely (1 week). Serum estradiol levels decreased gradually over the 4-week course. Plasma aldosterone levels were essentially unaltered and 18-hydroxcorticosterone levels fell gradually, but there were marked increases in 11-deoxycorticosterone and corticosterone. Coincident with the increase in 11-deoxycorticosterone there was an increase in blood pressure and a transient fall in serum potassium. We conclude that ketoconazole administration may result in a hypermineralocorticoid state. Therefore, the usefulness of ketoconazole therapy for steroid hormone-producing neoplasms will depend upon the individual tumor's steroidogenic profile.

Topics: 17-Ketosteroids; Adrenal Cortex Neoplasms; Aldosterone; Androgen-Insensitivity Syndrome; Corticosterone; Desoxycorticosterone; Estradiol; Estrogens; Humans; Hydrocortisone; Ketoconazole; Male; Middle Aged; Testosterone

The 15-year old female patient G. G. was referred to the outpatient department for child gynaecology on account of a primary amenorrhoea. The endocrinal parameters found (testosterone, oestradiol, 17 OHCS, 17 KS), chromosome analysis, vaginoscopy and the result of a laparoscopy led to the clinical diagnosis of "testicular feminisation". Satisfactory development of secondary female sex characteristics and sudden increase of complaints caused by an inguinal hernia on the left (gonad situated in the inner inguinal ring) prompted us to perform bilateral extirpation of gonads with simultaneous treatment of the inguinal hernia. Histological examination revealed the existence of a seminoma of the right gonad. This case confirm our stand in respect of extirpation of gonads at the onset of puberty, but it also raised the question as to whether it would be advisable to operate at an even earlier stage in order to avoid the risk of malignant degeneration of intra-abdominally positioned testes.

Topics: 17-Ketosteroids; Adolescent; Amenorrhea; Androgen-Insensitivity Syndrome; Castration; Dysgerminoma; Estradiol; Female; Humans; Hydroxysteroids; Male; Puberty; Testicular Neoplasms; Testosterone; Time Factors

The steroid response of testes to exogenous gonadotropin administration was studied before castration in a patient with the complete form of the testicular feminization syndrome. Urinary steroid determination were made along with measurements of plasma testosterone during adrenal suppression with dexamethasone and after stimulation with human menopausal gonadotropin (HMG) and HMG plus human chorionic gonadotropin (HCG). Our data provide evidence that the gonads in the testicular feminization syndrome synthesize steroids normally. Estrogens and testosterone are secreted by the testes, and the Leydig cells of the testes are capable of responding to exogenous HMG and HCG stimulation. These findins are consistent with the hypothesis that there is no abnormal testicular steroidogenesis in these genotypic males. Additional evidence is provided that these gonads secrete testosterone in amounts comparable to those of normally functioning testes. Failure of virilization in the presence of androgen secretion as indicated by previous authors is probably the result of a deficiency of androgenic action at the end organs. The presence of sparse pubic and axillary hair in the mother and the finding of the same type of karyotype (46,XY) in a sibling suggest that hereditary factors play a role in this disorder.

Topics: 17-Ketosteroids; Adolescent; Androgen-Insensitivity Syndrome; Castration; Chorionic Gonadotropin; Estrogens; Female; Follicle Stimulating Hormone; Gonadotropins; Humans; Leydig Cells; Luteinizing Hormone; Male; Testis; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adult; Androgen-Insensitivity Syndrome; Castration; Cell Transformation, Neoplastic; Child; Child, Preschool; Estrogens; Female; Humans; Male; Testosterone

The absence of uterus has been regarded by a number of authors as a condition for the diagnosis of testicular feminization, although cases with a rudimentary uterus have been reported. However, their number within the framework of this syndrome cannot be exactly determined. In the present report on a 22 year-old patient with testicular feminization and uterus bicornis solidus a double uterus malformation identical with that described in the Mayer-Rokitansky-küster syndrome was found. Forms of testicular feminization with rudimentary uterus in genetically-male individuals may be explained by the absence of androgens, or the absence of reactivity on the part of the target organ, and a simultaneous disturbance (lessening) of the function of the so-called X-factor (oviduct repressor) during early embryonal development. Our own observations, as well as reports in the literature, suggest a theory according to which testicular feminization may be regarded as a series of morphological variants, from male-oriented forms with vaginal aplasie, hypertrophy of the clitoris and male distribution of pubic hair, to female-orientated ones with a vagina of normal length and a rudimentary uterus. The absence of a uterus as a condition for the definition of the syndrome can be maintained only so far as no cases have, as yet been observed with a normally-developed uterus in a typical position. The karyogram showed a small Y-chromosome. Functional anomalies may only be surmised, since Y-anomalies are frequent (3% in a random collection). The morphology of the testes mirrored the functional embryonal insufficiency (pre-pubertal, undifferentiated testicular tissue with a varying amount of stroma and Leydig cells); the basal excretion of testosterone, 17-ketosteroid fractions and pregnane in the 24-hour urine was within the normal range for males. Oestrogen production over and above the "adrenal values" was also present. The values for plasma testosterone, which are in accordance with those of males of a similar age, are considered as indicating the importance of "androgen resistance in the periphery" as a factor in the aetiology of testicular feminization.

Topics: 17-Ketosteroids; Androgen-Insensitivity Syndrome; Diagnosis, Differential; Estrogens; Female; Genitalia, Female; Humans; Karyotyping; Laparotomy; Male; Syndrome; Testis; Testosterone; Uterus

Topics: 17-Ketosteroids; Androgen-Insensitivity Syndrome; Chorionic Gonadotropin; Humans; Male; Testicular Diseases; Testis; Testosterone

Topics: 11-Hydroxycorticosteroids; 17-Ketosteroids; Adult; Androgen-Insensitivity Syndrome; Androgens; Endocrine Glands; Estrogens; Humans; Karyotyping; Male

Topics: 17-Ketosteroids; Adult; Amenorrhea; Androgen-Insensitivity Syndrome; Estrogens; Female; Gonadotropins; Humans

Topics: 17-Ketosteroids; Adolescent; Adult; Androgen-Insensitivity Syndrome; Androsterone; Dehydroepiandrosterone; Disorders of Sex Development; Estradiol; Estrone; Female; Feminization; Glucocorticoids; Gynecomastia; Humans; Male; Oxidoreductases; Puberty; Radioimmunoassay; Testis; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocorticotropic Hormone; Androgen-Insensitivity Syndrome; Castration; Chorionic Gonadotropin; Chromatography, Gas; Estrogens; Female; Humans; Male; Pregnanediol; Stimulation, Chemical; Testis; Testosterone; Time Factors

Topics: 17-Ketosteroids; Adolescent; Adult; Androgen-Insensitivity Syndrome; Child; Dermatoglyphics; Disorders of Sex Development; Dwarfism; Female; Gene Frequency; Genitalia, Male; Humans; Hypogonadism; Karyotyping; Leukocytes; Male; Mouth Mucosa; Mullerian Ducts; Pedigree; Spinal Dysraphism; Wolffian Ducts

The metabolic clearance rate (MCR) and blood production rate (BP) of testosterone (T) and dihydrotestosterone (DHT), the conversion of plasma testosterone to plasma dihydrotestosterone, and the renal clearance of androstenedione, testosterone, and dihydrotestosterone have been studied in man. In eight normal men, the MCR(T) (516+/-108 [SD] liters/m(2)/day) was significantly greater than the MCR(DHT) (391+/-71 [SD] liters/m(2)/day). In seven females, the MCR(T) (304+/-53 [SD] liters/m(2)/day) was also greater than the MCR(DHT) (209+/-45 [SD] liters/m(2)/day) and both values were less than their respective values in men (P < 0.001). In men the conversion of testosterone into dihydrotestosterone at 2.8+/-0.3% (SD) was greater than that found in females, 1.56+/-0.5% (SD) (P < 0.001). In five pregnant females the MCR(T) (192+/-36 [SD] liters/m(2)/day), the MCR(DHT) (89+/-30 [SD] liters/m(2)/day) and the conversion of testosterone into dihydrotestosterone (0.72+/-0.15%) (SD) were significantly less than the values found in nonpregnant women. In five females with hyperthyroidism, the MCR for testosterone and dihydrotestosterone were similar to those observed in pregnant females, but the conversion of testosterone into dihydrotestosterone (2.78+/-1.7%) (SD) was greater, and similar to that found in men. In men the production of dihydrotestosterone was 0.39+/-0.1 (SD) mg/day, 50% being derived from the transformation of plasma testosterone. In women the production of DHT was 0.05+/-0.028 (SD) mg/day, only 10% coming from testosterone. During pregnancy, the production of testosterone and dihydrotestosterone are similar to that in normal women. In three patients with testicular feminization syndrome (an adult with hyperthyroidism and two children) these two MCRs were greatly reduced compared to the normal females, but the conversion of testosterone into dihydrotestosterone was in the limits of normal male rangeIn the normal subjects the renal clearance of androstenedione was greater than that of testosterone and dihydrotestosterone. Less than 20% of the dihydrotestosterone and less than 10% of the androstenedione in the urine is derived from the plasma dihydrotestosterone and androstenedione.

Topics: 17-Ketosteroids; Adult; Androgen-Insensitivity Syndrome; Androstanes; Carbon Isotopes; Child, Preschool; Chromatography, Gas; Chromatography, Thin Layer; Dihydrotestosterone; Female; Humans; Hyperthyroidism; Infant; Ketosteroids; Kidney; Male; Metabolic Clearance Rate; Middle Aged; Pregnancy; Protein Binding; Testosterone; Tritium

The metabolism of (14)C-labeled testosterone by cultured human fibroblasts and amniotic fluid cells was investigated. Radiolabeled testosterone was incubated with the cultured cells for 48 hr, and the labeled metabolites present in the medium were subsequently identified. The major metabolic products of testosterone formed by cultured fibroblasts were Delta(4)-androstenedione, dihydrotestosterone, androsterone, and androstanediol. The amount of testosterone metabolized through each of two pathways was calculated and used to form a ratio designated the 17beta-hydroxyl/17-ketonic ratio. Fibroblasts from normal male and female children and adult females had high 17beta-hydroxyl/17-ketonic ratios indicating testosterone metabolism occurred primarily through the 17beta-hydroxyl pathway. There was change in the pattern of testosterone metabolism with age in males, i.e., adult males had much lower 17beta-hydroxyl/17-ketonic ratios than did male children. The testosterone metabolism of fibroblast cultures derived from three children with testicular feminization and their mothers was compared to normal age and sexmatched controls. Fibroblasts of children with testicular feminization metabolized testosterone predominantly through the 17-ketonic pathway and manifested a pattern of testosterone metabolism distinctly different from their sex and age matched controls. The mothers of children with testicular feminization could be distinguished from normal females by their much lower 17beta-hydroxyl/17-ketonic ratios. The much lower amounts of dihydrotestosterone and androstanediol produced by fibroblasts from patients with testicular feminization as compared with normals suggests there is a decrease in testosterone 5alpha-reductase activity in these patients. Cultured amniotic fluid cells metabolized testosterone to the same four major metabolites found in fibroblast cultures, but their activity was much lower than that of fibroblasts. Most of the amniotic fluid cell cultures metabolized testosterone largely through the 17beta-hydroxyl pathway as did fibroblasts from normal children.

Topics: 17-Ketosteroids; Amniotic Fluid; Androgen-Insensitivity Syndrome; Androstanes; Androsterone; Carbon Isotopes; Cells, Cultured; Child; Child, Preschool; Chromatography, Thin Layer; Dihydrotestosterone; Female; Fibroblasts; Humans; In Vitro Techniques; Male; Mixed Function Oxygenases; Pregnancy; Skin; Sterols; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adult; Androgen-Insensitivity Syndrome; Androstanes; Carbon Isotopes; Dihydrotestosterone; Disorders of Sex Development; Female; Humans; Male; Metabolic Clearance Rate; Testosterone; Tritium

Topics: 17-Ketosteroids; Androgen-Insensitivity Syndrome; Cytodiagnosis; Cytogenetics; Diagnosis, Differential; Disorders of Sex Development; Female; Genitalia, Female; Humans; Infant, Newborn; Karyotyping; Male; Mosaicism; Mouth Mucosa; Sex Chromosome Aberrations

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adult; Androgen-Insensitivity Syndrome; Androstanes; Chromatography, Paper; Dehydroepiandrosterone; Estradiol; Estriol; Humans; In Vitro Techniques; Leydig Cell Tumor; Male; Pregnanediol; Pregnanetriol; Pregnenolone; Testosterone; Tritium

In 22 children with male pseudohermaphroditism, plasma concentrations of testosterone and dehydroandrosterone sulfate were measured before and after HCG stimulation. In ten of them, nitrogen retention was measured both before and during treatment with testosterone-propionate. The results of this investigation allow the patients to be classified in the following groups 1) nine in which both the testicular function and the end-organ sensitivity to androgens were normal; 2) nine patients had an abnormal testicular function; 3) four patients had the testicular feminization syndrome. In addition, in one adolescent patient with familial male pseudohermaphroditism and gynecomastia, the pattern of plasma androgens under basal conditions, after HCG and following gonadectomy, suggest that the patient had an incomplete 17-ketosteroid reductase defect.

Topics: 17-Ketosteroids; Adolescent; Alcohol Oxidoreductases; Androgen-Insensitivity Syndrome; Androstenedione; Androsterone; Child; Child, Preschool; Chorionic Gonadotropin; Dehydroepiandrosterone; Disorders of Sex Development; Estrone; Female; Humans; Infant; Infant, Newborn; Male; Nitrogen; Pedigree; Testis; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenocorticotropic Hormone; Adult; Androgen-Insensitivity Syndrome; Chorionic Gonadotropin; Estradiol; Estriol; Estrogens; Estrone; Follicle Stimulating Hormone; Gynecomastia; Humans; Leydig Cells; Luteinizing Hormone; Male; Medroxyprogesterone; Metabolism, Inborn Errors; Radioimmunoassay; Testicular Diseases; Testicular Neoplasms; Testis; Testosterone; Veins; Vena Cava, Inferior

Topics: 17-Ketosteroids; Adult; Aged; Androgen-Insensitivity Syndrome; Androstanes; Androsterone; Autoradiography; Carbon Isotopes; Chromatography, Gas; Chromatography, Thin Layer; Female; Humans; Hydrocortisone; In Vitro Techniques; Male; Middle Aged; Skin; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adult; Androgen-Insensitivity Syndrome; Androstanes; Androstenols; Carbon Isotopes; Gonads; Humans; Male; Pregnenolone; Progesterone; Steroids; Testis; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Glands; Androgen-Insensitivity Syndrome; Breast; Castration; Diethylstilbestrol; Estradiol; Estrogens; Female; Humans; Methyltestosterone; Pituitary Gland; Pituitary-Adrenal Function Tests; Puberty

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Androgen-Insensitivity Syndrome; Chorionic Gonadotropin; Estrogens; Testosterone; Vaginal Smears

Topics: 17-Ketosteroids; Adult; Androgen-Insensitivity Syndrome; Body Weight; Castration; Cell Nucleus; Color Vision Defects; Creatinine; Dihydrotestosterone; Estrogens; Eunuchism; Humans; Hypogonadism; Intellectual Disability; Male; Nitrogen; Olfaction Disorders; Phosphates; Receptors, Drug; Sex Chromosome Aberrations; Stimulation, Chemical; Testosterone

Topics: 17-Ketosteroids; Adolescent; Amenorrhea; Androgen-Insensitivity Syndrome; Estrogens; Female; Humans; Karyotyping; Sex Chromatin; Urography

Topics: 17-Ketosteroids; Administration, Oral; Adolescent; Androgen-Insensitivity Syndrome; Androstanes; Carbon Isotopes; Diet; Dihydrotestosterone; Female; Humans; In Vitro Techniques; Injections, Intramuscular; Ketosteroids; Male; Methyltestosterone; Nitrogen; Oxidoreductases; Phosphorus; Physical Exertion; Skin; Testosterone; Time Factors; Tritium

Topics: 17-Ketosteroids; Androgen-Insensitivity Syndrome; Androstanes; Animals; Chromatography, Thin Layer; Cloaca; Crystallization; Dihydrotestosterone; Embryo, Mammalian; Female; Fetus; Genitalia, Male; In Vitro Techniques; Male; Mullerian Ducts; Pregnancy; Rabbits; Rats; Sterols; Testosterone; Tritium; Wolffian Ducts

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adult; Amenorrhea; Androgen-Insensitivity Syndrome; Carbon Isotopes; Chromatography; Dehydroepiandrosterone; Estrogens; Female; Humans; In Vitro Techniques; Karyotyping; Male; Pregnenolone; Progesterone; Sex Chromosomes; Testis; Testosterone; Tissue Extracts; Tritium; Turner Syndrome

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Adult; Androgen-Insensitivity Syndrome; Androsterone; Castration; Dehydroepiandrosterone; Estrogens; Etiocholanolone; Female; Glucocorticoids; Gonadotropins; Gonads; Humans; Vaginal Smears

Topics: 17-Ketosteroids; Adolescent; Androgen-Insensitivity Syndrome; Androsterone; Castration; Child; Dehydroepiandrosterone; Disorders of Sex Development; Etiocholanolone; Humans; Leydig Cells; Male; Middle Aged; Mosaicism; Sex Chromosome Aberrations; Testis

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Androgen-Insensitivity Syndrome; Cytodiagnosis; Estrogens; Female; Gonadotropins; Humans; Karyotyping; Male; Sex Chromosomes; Sex Determination Analysis; Testis

Topics: 17-Ketosteroids; Acetates; Adult; Androgen-Insensitivity Syndrome; Androstanes; Carbon Isotopes; Cholesterol; Chromatography, Paper; Chromatography, Thin Layer; Dehydroepiandrosterone; Disorders of Sex Development; Estradiol; Humans; Male; Microscopy, Electron; Pregnenolone; Sterols; Sulfatases; Sulfates; Testis; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adult; Androgen-Insensitivity Syndrome; Androstanes; Androsterone; Carbon Isotopes; Disorders of Sex Development; Humans; Male; Oxidoreductases; Skin; Testosterone; Tritium

Topics: 17-Ketosteroids; Acetone; Adolescent; Androgen-Insensitivity Syndrome; Androstanes; Biotransformation; Carbon Isotopes; Chromatography, Thin Layer; Dehydroepiandrosterone; Disorders of Sex Development; Ethanol; Humans; In Vitro Techniques; Male; Methanol; Pregnenolone; Progesterone; Testis; Testosterone; Tritium; Water

Topics: 17-Ketosteroids; Adrenal Insufficiency; Adrenalectomy; Adult; Aged; Androgen-Insensitivity Syndrome; Castration; Chemistry, Clinical; Climacteric; Cryptorchidism; Disorders of Sex Development; Erectile Dysfunction; Estrogens; Eunuchism; Humans; Hypogonadism; Hypophysectomy; Hypothalamo-Hypophyseal System; Male; Middle Aged; Paraplegia; Puberty, Precocious; Spinal Cord Diseases; Testosterone

Topics: 17-Ketosteroids; Adult; Androgen-Insensitivity Syndrome; Androstanes; Androsterone; Carbon Isotopes; Chorionic Gonadotropin; Dihydrotestosterone; Disorders of Sex Development; Glucuronates; Humans; Hypogonadism; Hypopituitarism; Male; Middle Aged; Sulfates; Testosterone; Tritium; Turner Syndrome

Topics: 17-Ketosteroids; Adolescent; Androgen-Insensitivity Syndrome; Androstanes; Carbon Isotopes; Chromatography, Thin Layer; Disorders of Sex Development; Estradiol; Estriol; Estrogens; Estrone; Humans; In Vitro Techniques; Male; Testis; Testosterone

Topics: 17-Ketosteroids; Adult; Age Factors; Amenorrhea; Androgen-Insensitivity Syndrome; Counseling; Disorders of Sex Development; Female; Humans; Karyotyping; Phenotype

Topics: 17-Ketosteroids; Adult; Androgen-Insensitivity Syndrome; Disorders of Sex Development; Female; Genetics, Medical; Gonadotropins; Humans; Male; Pedigree; Sex Determination Analysis; Testis; Uterus

Topics: 17-Ketosteroids; Adolescent; Adrenal Gland Diseases; Adrenal Hyperplasia, Congenital; Amenorrhea; Androgen-Insensitivity Syndrome; Disorders of Sex Development; Estrogens; Female; Gonadotropins, Pituitary; Humans; Hypogonadism; Ovarian Diseases; Turner Syndrome

Topics: 17-Ketosteroids; Adolescent; Androgen-Insensitivity Syndrome; Androsterone; Blood Group Antigens; Chromosome Aberrations; Chromosome Disorders; Disorders of Sex Development; Genetics, Medical; Humans; Male; Testis

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocorticotropic Hormone; Androgen-Insensitivity Syndrome; Androgens; Blood; Carbon Isotopes; Castration; Chorionic Gonadotropin; Disorders of Sex Development; Gonadotropins; Humans; Leydig Cells; Male; Sertoli Cells; Testis; Testosterone; Urine

Topics: 17-Ketosteroids; Adolescent; Androgen-Insensitivity Syndrome; Chromosomes; Cytogenetic Analysis; Diagnosis; Disorders of Sex Development; Female; Genetics, Medical; Histology; Humans; Male; Pathology; Sex Chromatin; Surgical Procedures, Operative; Testis; Urine; Vulva

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Androgen-Insensitivity Syndrome; Disorders of Sex Development; Estrogens; Female; Humans; Infant; Male; Progesterone; Sex Chromatin; Testis; Thyroid Function Tests; Vaginal Smears

Topics: 17-Ketosteroids; Adolescent; Androgen-Insensitivity Syndrome; Child; Disorders of Sex Development; Estradiol Congeners; Estrogens; Gonadotropins; Humans; Intelligence Tests; Male; Sex Chromatin; Urine

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenocorticotropic Hormone; Androgen-Insensitivity Syndrome; Androgens; Estrogens; Gonadotropins; Humans; Hypogonadism; Klinefelter Syndrome; Male; Turner Syndrome; Urine

Topics: 17-Ketosteroids; Androgen-Insensitivity Syndrome; Disorders of Sex Development; Humans; Male