17-ketosteroids and Adrenocortical-Hyperfunction

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Amniotic Fluid; Estriol; Female; Fetoscopy; Gestational Age; Humans; Infant, Newborn; Male; Pregnancy; Pregnanetriol; Prenatal Diagnosis; Sex Factors

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 Gland Neoplasms; Adrenal Glands; Adrenocortical Hyperfunction; Anabolic Agents; Androgens; Brain Diseases; Female; Hirsutism; Humans; Hypothalamo-Hypophyseal System; Ovarian Diseases; Ovary; Pituitary-Adrenal Function Tests; Progestins; Skin; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenosine Triphosphate; Adipose Tissue; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Adult; Cholesterol; Cyclic AMP; Endocrine System Diseases; Fatty Acids, Nonesterified; Female; Gonadal Steroid Hormones; Growth Hormone; Humans; Insulin; Lipase; Lipoproteins; Male; Menstruation Disturbances; Middle Aged; Obesity; Triglycerides

Topics: 17-Ketosteroids; Adrenal Glands; Adrenal Insufficiency; Adrenocortical Hyperfunction; Age Factors; Androgens; Female; Humans; Male; Methods; Sex Factors; Testis

Topics: 17-Ketosteroids; Adolescent; Adrenal Gland Diseases; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Child; Child, Preschool; Dehydration; Female; Glucocorticoids; Humans; Hyperplasia; Hypertension; Infant; Infant, Newborn; Male; Mixed Function Oxygenases; Oxidoreductases; Pylorus; Virilism; Vomiting; Water-Electrolyte Balance

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Chemistry, Clinical; Corticosterone; Cushing Syndrome; Diagnosis, Differential; Female; Glucocorticoids; Humans; Hydrocortisone; Hypogonadism; Kidney Failure, Chronic; Male; Methods; Obesity; Pregnancy; Pregnancy Complications

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Androgens; Estrogens; Female; Hirsutism; Humans; Hypertrichosis; Metabolism; Urine

Topics: 17-Ketosteroids; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Anabolic Agents; Hirsutism; Hyperaldosteronism; Hypertrichosis; Hypogonadism; Metabolism; Steroids; Urine

Both pregnenolone and 17-OH-pregnenolone were found to be higher in the plasma of patients with poorly controlled congential adrenal hyperplasia than in normal subjects. The plasma levels of these precursor steroids were significantly correlated with urinary 17-ketosteroid and pregnanetriol excretion and with plasma testosterone. The mechanism where by plasma pregnenolone and 17-OH-pregnenolone levels are elevated in patients with 21-hydroxylase deficiency is unknown, but the phenomenon of product inhibition is suggested as a possible explanation. As 17-OH-pregnenolone in plasma is almost entirely of adrenal origin, its measurement promises to be useful in the management of patients with congenital adrenal hyperplasia. Acute stimulation with ACTH caused negligible changes in the plasma levels of pregnenolone and 17-OH-pregnenolone and failed to distinguish between overly, appropriately, and under-treated patients. However, following repeated stimulation with repository ACTH, the steroid levels rose. These findings indicate limited adrenal responsiveness to ACTH following chronic glucocorticoid treatment of congenital adrenal hyperplasia, even in under-treated patients, and suggest that normal precursor steroid levels in plasma and normal 17-ketosteroid and pregnanetriol excretion can only be achieved by the suppression of total steroidogenesis to less than that occurring in normal subjects.

Topics: 17-alpha-Hydroxypregnenolone; 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Child; Child, Preschool; Clinical Trials as Topic; Female; Humans; Hydrocortisone; Hyperplasia; Infant; Male; Pregnanetriol; Pregnenolone; Progesterone Reductase; Steroid Hydroxylases; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Binding, Competitive; Blood Proteins; Child; Child, Preschool; Circadian Rhythm; Clinical Trials as Topic; Dexamethasone; Female; Fludrocortisone; Glucocorticoids; Humans; Hydrocortisone; Hydroxyprogesterones; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors; Pituitary-Adrenal Function Tests; Pituitary-Adrenal System; Protein Binding; Steroid Hydroxylases

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Aminoglutethimide; Androsterone; Aniline Compounds; Anticonvulsants; Chemistry, Clinical; Clinical Trials as Topic; Cushing Syndrome; Dehydroepiandrosterone; Etiocholanolone; Glucocorticoids; Humans; Hyperplasia; Hypogonadism; Pyridones; Testosterone

Topics: 17-Ketosteroids; Adenocarcinoma; Adolescent; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adult; Clinical Trials as Topic; Creatine; Cushing Syndrome; Dexamethasone; Female; Humans; Hydrocortisone; Male; Middle Aged; Pituitary Function Tests

We present two patients with Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia who showed marked plasma cortisol response to lysine-8-vasopressin (LVP) injection (from 930 and 731 pmol/L to 2177 and 1920 pmol/L, respectively), while plasma ACTH levels remained undetectable. The ACTH independence of cortisol secretion in the two patients was determined from the following endocrinological findings. Plasma cortisol levels were not increased by corticotropin-releasing hormone injections and were not suppressed by high dose (16 mg) dexamethasone administrations. The plasma ACTH levels, measured by two independent sensitive immunoassays, were persistently undetectable even after corticotropin-releasing hormone injection, metyrapone administration, and bilateral adrenalectomy. The particular pathological finding of the two cases, atrophic lesions in nonnodular parts of the adrenal cortexes, also indicated ACTH independence of the macronodular hyperplasia. In vitro examination revealed a direct effect of LVP on cortisol secretion from the adrenal cells of the macronodules. We also examined seven patients with Cushing's syndrome caused by adrenal adenoma and found a statistically significant plasma cortisol response to LVP injection. The direct effect of LVP was also demonstrated in cultured adenoma cells. In conclusion, we discovered a direct adrenal effect of LVP on cortisol secretion in patients with ACTH-independent macronodular hyperplasia and, to a lesser extent, in patients with cortisol-producing adrenal adenoma. The cortisol response to LVP may serve to facilitate their diagnosis and choice of therapy.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Analysis of Variance; Corticotropin-Releasing Hormone; Cushing Syndrome; Dexamethasone; Female; Humans; Hydrocortisone; Immunoradiometric Assay; Lypressin; Male; Middle Aged; Sensitivity and Specificity

Adrenocortical carcinoma is a rare tumor with a poor prognosis. This work was aimed at analyzing the clinical outlook and treatment results of 52 patients with this disease.. This study included patients with adrenocortical carcinoma referred to the Department of Endocrinology at the Center of Postgraduate Medical Education (Warsaw, Poland) during the last 30 years. In 11 patients, the adrenal tumor was found incidentally by ultrasonographic scan. Hormonal examinations made it possible to define the endocrine activity of the tumors, whereas imaging techniques helped to determine their staging. Forty-eight patients underwent surgery, and 36 of them received mitotane. This drug was administered to 26 patients for a range of 10 months to 10 years; 13 patients received mitotane immediately after the operation, and 13 others after a delay. The patients with severe hypercorticism were pretreated before surgery with aminoglutethimide and mitotane.. The study comprised 10 men and 42 women; hormonally active tumors were diagnosed in 39 of them. Cushing's syndrome was the most frequent entity. At diagnosis, 17 cases were classified as localized disease, 15 as regional disease, and 20 as distant disease. Pretreatment with the inhibitors of steroidogenesis improved the survival perspectives in the early postoperative period. As of this writing, there were 12 survivors in the group of 26 patients treated by surgery and long term mitotane therapy and only 2 survivors of 7 patients treated with surgery only.. Surgery with immediate adjuvant long term mitotane administration was the most effective form of therapy for patients with adrenocortical carcinoma.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Adrenocortical Hyperfunction; Adult; Aged; Aminoglutethimide; Chemotherapy, Adjuvant; Cushing Syndrome; Female; Follow-Up Studies; Growth Hormone; Growth Hormone-Releasing Hormone; Humans; Hydrocortisone; Male; Middle Aged; Mitotane; Neoplasm Staging; Survival Rate; Testosterone; Treatment Outcome

Nine cases of adrenocortical tumor are presented, six were males. Four were less than three years and five were between 5 and 10 years of age. Clinical virilization was found in 8 children, one had only signs of hypercortisolism and another showed signs of virilization and hypercortisolism simultaneously. Urinary 17-KS and 17-OHCS were high in all patients. Plasma levels of testosterone and of the other adrenal androgens were high in all the cases tested. Plasma level of cortisol was elevated only in few cases. In two out of five cases steroids were only partially suppressed by dexamethasone. Computed tomography and abdominal sonography have been useful tools for the localization of the tumour. The resection of the tumour, independent of histopathological diagnosis, led to a complete normalization of the clinical and hormonal picture in eight cases evaluated at a distance of 2 months 10 years after surgery. In one case a hepatic metastasis was observed and removed three years after surgery.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Adrenocortical Hyperfunction; Child; Child, Preschool; Cortisone; Humans; Infant; Male; Virilism

Serum 17alpha-hydroxyprogesterone (17 OH-P) was measured by a specific radioimmunoassay technique combined with thin-layer chromatography. Normal values for children are less than 1.1 microgram/1 (less than 3.3 nmol/l)--corresponding to values found in the literature. In congenital adrenal hyperplasia (CAH) values up to several hundred microgram/l are found. The values rise after ACTH stimulation and are suppressed by decadrone or cortisone treatment. The rise in 17 ketosteroids and pregnanetriol in untreated CAH is relatively smaller (15--25 fold). This clinical sensitivity of 17 OH-P is thus valuable for the diagnosis of CAH (21 hydroxylase deficiency). Furthermore it is easier to take a blood sample than to collect urine for 24 hours. The usefulness in therapeutic monitoring is being studied.

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Child; Child, Preschool; Chromatography, Thin Layer; Female; Humans; Hydroxyprogesterones; Infant; Male; Pregnanetriol; Radioimmunoassay; Reference Values

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Aldosterone; Angiotensin II; Blood Volume; Child; Child, Preschool; Female; Fludrocortisone; Humans; Hydroxyprogesterones; Male; Renin

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Chromatography, Gas; Female; Humans; Male; Pregnancy

Simultaneous serum concentrations of dehydroepiandrosterone sulfate (DHEA-S) and 17-hydroxyprogesterone (17-OHP) were compared with urinary 17-ketosteroid (17-KS) and pregnanetriol (PT) excretion during therapy in 18 prepubertal patients with the 21-hydroxylase deficiency form of congenital adrenal hyperplasia (CAH). Patients were classified into those in good, poor, or questionable control on the basis of clinical examination, skeletal age, and 17-KS and PT excretion. During therapy, use of serum steroid concentrations was found to be nearly as accurate in judging adequacy of control as use of urine steroid concentrations. Of 34 evaluations, a definite assessment of adequacy of control could be arrived at 25 times using urinary values and 22 times using both serum DHEA-S and 17-OHP concentrations. DHEA-S concentration responded sluggishly when treatment was not adequate, being greater than 100 microgram/dl only in patients significantly undertreated. It was never elevated in well-controlled patients. Mid-afternoon 17-OHP concentrations were less than 200 ng/dl in well-controlled patients but readily escaped suppression and could not be used to differentiate poor from borderline control or from temporary noncompliance. Therefore, an increases DHEA-S concentration indicated poor control and a suppressed 17-OHP concentration indicated good control. The combination of normal DHEA-S level with elevated 17-OHP level, however, did not permit exact evaluation of the degree of control. Of significance is that not all patients with CAH present with an elevated DHEA-S concentration, and only in those in whom an elevated level has been documented can DHEA-S level be used as an index of control during therapy.

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Child; Child, Preschool; Cortisone; Dehydroepiandrosterone; Female; Humans; Hydroxyprogesterones; Infant; Male; Mixed Function Oxygenases; Pregnanetriol

The effects of congenital adrenal hyperplasia on adult height and fertility were studied in 30 afflicted men. The patients' heights ranged from 150.0 to 178.6 cm (mean +/- 1 S.D. of 164.0 +/- 7.6), which is significantly lower than both the mean adult height for American men and that of the patients' parents (P less than 0.005). There was no correlation between adult height and the age at which therapy was begun, possibly because the patients treated before one year of age had the salt-losing form of the syndrome. Therapeutic compliance may also have been involved. Apparently normal fertility, indicated by paternity and normal sperm counts, was found in 18 out of 20 patients evaluated. This group included five untreated patients who were found to be fertile and to have normal plasma testosterone and gonadotropin but elevated androstenedione levels.

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Body Height; Cell Count; Dehydroepiandrosterone; Fertility; Follicle Stimulating Hormone; Glucocorticoids; Humans; Hydroxyprogesterones; Luteinizing Hormone; Male; Pregnanetriol; Spermatozoa

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Androgens; Androstenedione; Child; Child, Preschool; Dehydroepiandrosterone; Female; Humans; Hydrocortisone; Infant; Male; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Aldosterone; Child; Child, Preschool; Female; Humans; Hydrocortisone; Hydroxyprogesterones; Infant; Male; Prednisolone; Pregnanetriol; Radioimmunoassay

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Hydrocortisone; Luteinizing Hormone; Male; Pregnanes; Progesterone; Testosterone

Three otherwise healthy relatives of patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency and salt-wasting presented with clinical and/or biochemical findings, which exceeded those usually seen in heterozygotes: Two females (1 mother and 1 prepubertal sister of a patient with CAH) had marked hypertrichosis and hirsutism and excreted pregnanetriolone in their urine. The mother had increased basal plasma 17alpha-OH-progesterone (296 ng/100 ml), which increased to 7170 ng/100 ml after ACTH as in homozygotes of CAH. One adult male (brother of a patient with CAH) was clinically normal, but also excreted pregnanetriolone and had a high plasma 17alpha-OH-progesterone (1905 ng/100 ml), which increased further to 6352 ng/100 ml after ACTH. It is concluded that these subjects represent unusually marked heterozygotes of CAH rather than mild homozygotes. In females, this condition should be included in the differential diagnosis of idiopathic hirsutism, in males, it will pass unnoticed, unless relatives of patients with CAH are systematically tested.

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Female; Heterozygote; Humans; Hydrocortisone; Hydroxyprogesterones; Male; Mixed Function Oxygenases; Pedigree; Pregnanes; Pregnanetriol; Testosterone

A retrospective study was made of 16 children with 21-hydroxylase-deficient congenital adrenal hyperplasia of the salt-losing variety, who were treated with fludrocortisone and prednisone and were in good health during the period under review. The height velocity of the children was subnormal, height achievement was poor, and their bone ages retarded. Urinary 17-oxosteroid and pregnanetriol excretion were used to monitor the therapy of the children and these data have been related to growth velocities. In spite of urinary steroid figures in excess of those published as desirable for monitoring therapy, the children failed to grow properly, probably as a result of glucocorticoid overdosage. Published urinary steroid criteria are considered too strict and in order to achieve them one would need to give unnecessarily high doses of steroid. Regular measurement of height velocity and skeletal maturation rate are better indicators of therapeutic control and should lead to more satisfactory growth and ultimate height.

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Body Height; Child; Child, Preschool; Female; Fludrocortisone; Humans; Infant; Male; Prednisone; Pregnanetriol; Retrospective Studies

Simultaneous determinations of serum concentrations of 17OH-progesterone, testosterone, androstenedione, and progesterone, and of urinary excretion of 17-ketosteroids and pregnanetriol have been performed at intervals in 31 patients with the C21-hydroxylase form of congenital adrenal hyperplasia. In prepubertal patients there were highly significant correlations between levels of 17OH-progesterone and those of testosterone, androstenedione, and progesterone, respectively. Similar correlations were observed in adolescent girls. In adolescent boys rising 17OH-progesterone levels were reflected by increasing levels of androstenedione and progesterone, but there was no change in serum testosterone concentrations. Levels of serum 17OH-progesterone below 200 ng/dl were uniformly associated with normal serum concentrations of testosterone, androstenedione, and progesterone, and normal urinary 17-ketosteroid and pregnanetriol excretion. In contrast, levels above 1000 ng/dl were accompanied by increased levels of the other steroids except in adolescent males; in this group the finding of unchanging serum testosterone concentrations in spite of rising 17OH-progesterone levels presumably indicates that testosterone of adrenal origin causes suppression of testicular testosterone production, either through a direct effect upon Leydig cells or via suppression of LH release.

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Androstenedione; Child; Child, Preschool; Female; Humans; Hydroxyprogesterones; Infant; Infant, Newborn; Male; Pregnanetriol; Progesterone; Testosterone

Extensive hormonal evaluation was performed in a girl with adrenal carcinoma during the primary tumor stage, following adrenalectomy, during the period when metastases were evident and while on treatment with o,p'-DDD. At the age of 14 months a diagnosis of congenital adrenal hyperplasia was made and treatment with dexamethasone (0.125 to 0.25 mg/day) resulted in a fall-off in growth rate, normal advancement in bone age, decrease in virilization and suppression of 17- ketosteroid excretion which continued until 4 3/12 years of age when virilization increased. At five years of age elevated serum and urinary androgen levels unsuppressible with dexamethasone were noted. Following removal of a large right adrenal carcinoma, serum and urinary hormone levels returned to normal. There months following surgery, liver metastases were documented associated with elevated levels of serum androgens. With o,p'-DDD treatment, serum dehydroepiandrosterone sulfate (DS) and urinary 17-ketosteroid (17-KS) excretion fell rapidly while there was a delay in the fall of free androgens. The persistence of free steroid secretion with decreased formation of DS suggests that the o,p'-DDD may have altered sulfatase activity before causing tumor necrosis and total decrease in steroidogenesis.

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Androgens; Child, Preschool; Dehydroepiandrosterone; Depression, Chemical; Dexamethasone; Diagnosis, Differential; Female; Humans; Infant; Mitotane; Neoplasm Metastasis; Virilism

A 22-year-old male with bilateral testicular tumors and the 21-hydroxylase variety of congenital adrenal hyperplasia (CAH) was studied. Preoperatively, on his usual glucocorticoid regimen, his urinary pregnanetriol excretion was increased (8.0-23.5 mg/day), serum LH and FSH were normal to increased (14.3-28.7 mIU/ml and 13.2-19.5 mIU/ml, respectively) and testosterone (T) was normal to decreased (176-600 ng/dl). At surgery, testicular vein concentrations of 17-alpha-hydroxyprogesterone (17-OHP) and adnrostenedione (delta) were increased (30.1 mug/dl and 38.3 mug/dl respectively) while T was decreased (1,503 ng/dl); a positive peripheral vein--testicular vein gradient was not seen for these steroids. Following injection of 10 U of crystalline ACTH into the testicular artery; testicular vein concentrations of 17-OHP, delta and T increased to 729 mug/dl, 2,390 mug/dl and 9,660 ng/dl respectively. Microscopic examination of the testes revealed multinodular tumors composed of polygonal or rounded eosinophilic cells, arranged in cords, nests and clusters. The tumors extended from the hilus and compressed the adjacent testicular tissue. Electron microscopic examination of the tumors showed features, common to steroid-secreting tissues, with abundant smooth endoplasmic reticulum in close proximity to mitochondria which was moderate in number. The adjacent testicular tissue was composed of immature tubules with normally developed Leydig cells in the interstitial tissues. From these data and a survey of previous works, it was postulated that these tumors were dependent upon ACTH for growth and steroid secretion. In view of the high serum LH concentration seen in association with incomplete suppression of adrenal steroid secretion in this study and the association of evidence of gonadotropin secretion with testicular tumors in other CAH patients, LH may also have contributed to the growth of these tumors.

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Androstenedione; Follicle Stimulating Hormone; Humans; Hydroxyprogesterones; Luteinizing Hormone; Male; Microscopy, Electron; Pregnanetriol; Testicular Neoplasms; Testis; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Aldosterone; Androgens; Child; Child, Preschool; Dexamethasone; Diet, Sodium-Restricted; Female; Humans; Hydrocortisone; Male; Potassium; Renin; Sodium

21 years experience with management of seventeen cases of congenital adrenal hyperplasia due to 21-hydroxylase deficiency has been analysed with respect to growth, bone maturation and related events at puberty: age at menarche and the occurrence of menstrual irregularities, this study showed that growth retardation is still a problem; that irregular treatment and prolonged exposure to adrenal androgens or oestrogens, may lead to disturbance in hypothalamo-pituitary-gonadal function and may be the cause fo delayed menarche, or menstrual irregularities in the case of the female. In males the start of puberty and its completion was within the normal range.

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Body Height; Bone Development; Child; Child, Preschool; Clomiphene; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Growth; Humans; Infant; Infant, Newborn; Luteinizing Hormone; Male; Menstruation; Mixed Function Oxygenases; Puberty; Testosterone

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Congenital Hypothyroidism; Female; Humans; Hypothyroidism; Infant

Six patients with congenital virilizing adrenal hyperplasia were evaluated on single- and multiple-dose prednisone schedules. Each of the treatment periods was for one month. Patients were evaluated by 24-hour urinary excretion of 17-ketosteroids and pregnanetriol, as well as 0900 plasma concentrations of 17-hydroxyprogesterone, progesterone, and testosterone. By the criteria of urinary excretion of KS and PNT appropriate for chronologic age, three of the six patients were adequately controlled on prednisone given once a day. Prednisone administered twice daily at 12-hourly intervals either in equally divided doses or with a larger dose in the evening, however, resulted in adequate suppression in all patients. Because of the marked diurnal variation of plasma 17-OHP, the time of day that the sample is drawn is critical. Afternoon samples are often misleadingly low. Plasma 17-OHP concentration may reflect escape from therapeutic control sooner than urinary KS and PNT excretion. There was no correlation between 17-OHP and P values. Plasma concentration of T was not a reliable indicator of good control, since T values were often at prepubertal levels when urinary KS and PNT were elevated.

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Child; Child, Preschool; Drug Administration Schedule; Female; Humans; Hydroxyprogesterones; Male; Prednisone; Pregnanetriol; Progesterone; Testosterone

The temporal relationship between administration of cortisol and serum 17alpha-hydroxyprogesterone was investigated in five patients aged 9-19 years with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. There was marked variability in the 17alpha-hydroxyprogesterone response (determined hourly for 24 h) of individual patients to administration of cortisol. Mean concentration was less than 0.030 micronmol/l in one patient but 0.519 micronmol/l in another. Levels were higher in all patients while off treatment, and were greatest in those with salt-losing adrenal hyperplasia. Growth hormone secretion was not suppressed by treatment with cortisol. Withdrawal of cortisol for 3 days resulted in a significant decrease in the mean serum FSH/LH ratio and a rise in serum testosterone in all subjects. Episodic release of gonadotrophins persisted in the adolescent patients.

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Child; Female; Follicle Stimulating Hormone; Growth Hormone; Humans; Hydrocortisone; Hydroxyprogesterones; Luteinizing Hormone; Male; Testosterone; Time Factors

One hundred and one patients with polycystic ovary syndrome (PCO) were treated during an 11-year interval. Sixty-seven cases were classified as typical PCO with enlarged ovaries of probable ovarian origin (Type I) and 34 as atypical PCO of probable adrenal origin (Type II). Fifty-five patients were treated for anovulatory infertility with clomiphene citrate or other endocrine therapy as indicated. There was a 91% ovulatory response and 51% conception rate from therapy in cases of Type I PCO. In Type II PCO, adrenal suppressive therapy resulted in a 55% ovulatory response. Our data support the concept that anovulation of ovarian origin as seen in PCO Type I responds to clomiphene therapy, while anovulation secondary to adrenal hyperandrogenization should be treated by adrenal suppressive therapy.

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Clomiphene; Female; Humans; Polycystic Ovary Syndrome

Ninety-eight females with congenital adrenal hyperplasia due to a defect in either the 21-hydroxylase or the 11 beta-hydroxylase enzyme were evaluated to determine the effects of glucocorticoid treatment on growth, pubertal development, and fertility. When treatment was begun prior to one year of age, mean final height was 157.4 +/- 7.3 com, well within the normal adult female range, and significantly (p less than 0.001) greater than the mean final height of 150.9 +/- 4.3 cm found in untreated patients. The mean age of menarche in patients treated prior to the age of six years was 13.8 +/- 3.7 years which is significantly (p less than 0.01) delayed compared to that in the normal population of the United States. However, 92% of patients with menstrual delay had inadequate suppression of adrenal androgens and urinary excretion of 17 ketosteroids larger than 7.0 mg/24hours. The increased production of adrenal androgens was the result of poor compliance or an insufficient prescribed dose of glucocorticoids. The fertility rate in patients first treated between six and 20 years of age was 64%. The excretion of urinary 17 KS at the time of pregnancy was 2.5 to 5.3 mg/24 hours. All of the patients who delivered term infants required delivery by cesarean section because of cephalopelvic disproportion. The major problems encountered in the management of adolescent patients were patient noncompliance and physician failure to increase the glucocorticoid dose as the patient's body size increased.

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Body Height; Breast; Child; Child, Preschool; Cortisone; Desoxycorticosterone; Female; Fertility; Glucocorticoids; Humans; Hydrocortisone; Infant; Menarche; Prednisone; Pregnancy; Puberty; Sexual Maturation; Steroid Hydroxylases; Time Factors

Plasma renin activity (PRA), aldosterone (Aldo), 17alpha-hydroxyprogesterone (17-OHP) and testosterone (T), together with urine sodium, pregnanetriol, 17-oxosteroids and the 11-oxygenation index (11-OH) were estimated in 23 patients (age 5.7--18 yrs.) with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency during glucocorticoid treatment. Elevated PRA levels (1400--17200 ng Al/l/hr) were found in 13 out of 15 patients with a history of salt loss. Three non-salt losers showed high PRA levels and in the remaining 5 the levels were in the upper normal range (540--900 ng Al/l/hr). Plasma Aldo levels were normal (25--620 pmol/l) in 18 patients and slightly elevated (690--2360 pmol/l) in 5. While these results indicate persistent impairment of sodium homeostasis in CAH patients, no significant correlations between log. PRA, log. Aldo and urinary sodium excretion were found. Mid-day 17-OHP levels ranged from 9 to 117 nmol/l and T from 0.3 to 18.0 nmol/l. Neither the 17-OHP nor the T results correlated well with the clinical assessment of therapeutic control. The results of the urinary steroid determinations showed better agreement with the clinical assessment of treatment and the 17-oxosteroid, pregnanetriol and 11-OH index results appeared to be better discriminants between good and poor control. Twelve of the patients with a history of early salt loss were reinvestigated after one month's treatment with oral 9 alpha-flurohydrocortisone (0.05 mg/day). PRA was reduced in 7 patients and 17-OHP fell in 10 patients. No consistent changes were found in Aldo, T, or urinary sodium and steroid excretion during this low-dose mineralocorticoid treatment.

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Aldosterone; Child; Child, Preschool; Female; Glucocorticoids; Humans; Hydroxyprogesterones; Male; Natriuresis; Pregnanetriol; Renin; Steroid Hydroxylases; Steroids; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Child; Female; Follicle Stimulating Hormone; Humans; Hydrocortisone; Hydroxyprogesterones; Luteinizing Hormone; Testosterone

The manifestations that comprise the disease known as Nelson syndrome are pituitary hyperplasia and cutaneous hyperpigmentation, which sometimes follow bilateral adrenalectomy, in patients with hypercortisolism. We present a comprehensive endocrinologic, structural study of a patient in whom the evidence obtained supports the hypotheses that: (a) the primary disorder in this form of hypercortisolism is probably hypothalamic; (b) the hyperplasia of the adenohypophysis, following adrenalectomy, is closely associated with lowered plasma cortisol levels; and (c) the cillular hyperplasia in the adenohypophysis involves primarily the corticotroph, a cell believed to be associated with the secretion of adrenocorticotrophic hormone and melanocyte-stimulating hormone.

Topics: 17-Ketosteroids; Adrenalectomy; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Female; Humans; Melanocyte-Stimulating Hormones; Pigmentation Disorders; Pituitary Diseases; Pituitary Gland; Pituitary Gland, Anterior; Syndrome

Dehydroepiandrosterone sulfate (DS) concentration was measured in the sera of premature and full-term infants and in children throughout puberty. Panhypopituitary, Addisonian, and virilized children were also studied. DS decreased slowly during the first weeks of life from a high level in neonates to the low levels observed between one to five years. After five years of age, DS concentration started to rise. A steeper increase was observed with the onset of puberty, and adult DS concentrations were reached in late puberty. There was no sex difference in DS concentration at any pubertal stage or bone age. Day-to-day variations were small in childhood and during puberty, but were considerable in premature infants. DS concentrations measured at 0900 h were not significantly different from those at 1700 h. There was a positive correlation of serum DS concentrations with the excretion of urinary 17-ketosteroids in boys and girls (r=0.789). Premature infants had DS concentration in or above the late pubertal range. Five panhypopituitary patients and five Addisonian patients had DS concentrations below normal. DS was markedly elevated in patients with congenital adrenal hyperplasia and in one girl with adrenal carcinoma, and was suppressible with dexamethasone in the former. The ease of measurement and the small amount of blood required make serum DS determination a useful guide for adrenal androgen secretion.

Topics: 17-Ketosteroids; Adolescent; Adrenal Gland Neoplasms; Adrenal Glands; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Age Determination by Skeleton; Androgens; Body Height; Child; Child, Preschool; Dehydroepiandrosterone; Dexamethasone; Female; Humans; Infant; Infant, Newborn; Infant, Premature; Male; Pregnancy; Puberty; Sex Factors

Basal release of gonadotropin and the response to an infusion of 100 mug of synthetic luteinizing hormone releasing hormone (LRH) were studied in a teenage girl with congenital adrenal hyperplasia (CAH). The initial study was done during a period of poor adrenal suppression, and second study was done after adequate adrenal suppression was achieved. To assess adrenal function, circulating levels of adrenal steroid hormones were evaluated continuously over a 24 h period. During the period increased production of adrenal androgens, the pattern of gonadotropin release was that of a prepubertal child. After 3 months of adrenal suppression the pattern of gonadotropin secretion was similar to that of a normal girl in mid-puberty. This demonstrates the rapid change from prepubertal to pubertal gonadotropin dynamics in a teenage patient following adequate suppression of androgens from the adrenal.

Topics: 17-Ketosteroids; Adolescent; Adrenal Glands; Adrenocortical Hyperfunction; Age Determination by Skeleton; Androstenedione; Circadian Rhythm; Cortisone; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Hydroxyprogesterones; Luteinizing Hormone

The circadian periodicity of adrenal function in patients with congenital virilizing adrenal hyperplasia (CAH) was examined by measuring the urinary 17-ketosteroids, 17-hydroxycorticosteroids, sodium, and potassium. Patients with the salt-losing and the non-salt-losing types were studied with and without treatment. Cosine curves were fitted to the data by the least-squares method to determine the mesors, amplitudes, and acrophases of the variable for each patient. The data reveal distinct circadian rhythms for all variables measured whether or not the patient was receiving treatment. The acrophases for 17-ketosteroids and 17-hydroxycorticosteroids were between 1500 and 1800 h. These acrophases are about 6 h later than those for normal subjects. The treatment on a fixed daytime schedule for many years may have shifted the natural rhythm.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Circadian Rhythm; Cortisone; Humans; Potassium; Sodium

Five cases of congenital adrenal hyperplasia due to C21-hydroxylase defect were treated with a combination of aminoglutethimide and prednisolone. In the third year of treatment the urinary levels of 17-oxosteroids increased above normal values while the total 17-hydroxy-corticosteroids were normally low. Specifically, urinary pregnanetriol was normal in 3 cases. To determine the reasons for this disparity the adrenal metabolism of cholesterol, as judged by the urinary steroid metabolites, was studied. Fractionation of urinary steroid metabolites was by thin-layer chromatography (TLC) followed by gas-liquid chromatography (GLC). The results indicate that aminoglutethimide inhibits steroidogenesis less than prednisolone; that a pathway from cholesterol via 17 alpha, 20 alpha-dihydroxycholesterol to dehydroepiandrosterone is likely to operate after long-term aminoglutethimide therapy; that 11 beta-hydroxylase, at least for pregnenes may be inhibited by aminoglutethimide and that the metabolic breakdown of testosterone may be delayed by this drug.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Aminoglutethimide; Child; Child, Preschool; Cholesterol; Chromatography, Gas; Chromatography, Thin Layer; Female; Humans; Prednisolone; Pregnadienetriols; Steroid Hydroxylases; Time Factors

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Blood Glucose; Diagnosis, Differential; Female; Hirsutism; Humans; Hydrocortisone; Menstruation; Ovarian Diseases; Testosterone; Vaginal Smears

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Cortisone; Dexamethasone; Diseases in Twins; Female; Growth; Humans; Hydrocortisone; Hydroxyprogesterones; Infant, Newborn; Male; Pregnanetriol

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Function Tests; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Aldosterone; Dehydroepiandrosterone; Dexamethasone; Follicle Stimulating Hormone; Gynecomastia; Humans; Hydrocortisone; Infant; Luteinizing Hormone; Male; Penis; Prednisone; Steroid Hydroxylases; Testosterone

Thirty-four cases of corticosurrenaloma with clinical onset before 15 years of age have been studied. Higher frequency in girls (65 percent of the cases) and in young age (80 percent before 5 years of age, 43 percent before 2 years), association with personal or familial other tumors and malformations, are noticeable features. Virilism is the major manifestation in children (83 percent of the cases), either isolated or associated with hypercortisolism and/or feminization. Hormonal assays are of little value, and the dynamic adrenal tests are of some help only in small tumors or isolated hypercortisolism. Radiological diagnosis by urography with cavography, arteriography, is easy in most cases. Evaluation of prognosis is very difficult. Clinical and biological data are not significant, histological data are often of little help. Only a very large mass, and occurrence of metastases (liver, lungs) evidence malignancy. This series does not allow to assess definitely the best therapeutic regimen. Surgery has to be performed as soon as possible. The effects of radiations have not yet been ascertained. High doses of op'DDD have led to a sustained remission of the tumor or metastases in 2 patients, and perhaps to a longer survival in 4 others.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Angiography; Child; Child, Preschool; Cushing Syndrome; Dehydroepiandrosterone; Female; Feminization; Humans; Male; Mitotane; Urography; Virilism

To evaluate the usefulness of blood testosterone (T) in monitoring the effects of therapy in congenital virilizing adrenal hyperplasia due to 21- or 11- hydroxylation defect (CVAH), T levels were measured on 45 occasions in 13 patients with CVAH; 32 urinary 17-ketosteroid levels and 31 preganetriol values were available for comparison. Bone age levels, growth data, and medication are listed to help assess the clinical state of the patient at the time of each T determination. Blood T values were above normal for age and sex in untreated patients with CVAH and declined with glucocorticoid suppression. A blood T value of 20 ng/100 ml appeared to distinguish between well-controlled cases and those with inadequate steroid suppression. Serial measurement of blood T in girls and in prepubertal boys with CVAH provides assistance in evaluating chemical control of the disease, particularly when accurate 24-h urine collections cannot be obtained for 17-ketosteroid and pregnanetriol assessments.

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Age Determination by Skeleton; Body Height; Child; Child, Preschool; Female; Glucocorticoids; Humans; Male; Pregnanetriol; Testosterone; Virilism

The study of metabolism of muscle electrolyte in children with the salt-losing form of congenital adrenal hyperplasia reveals two types of alterations. After admission and during initial therapy with salt and desoxycorticosterone, the changes are typical of those seen in experimental animals with adrenalectomy and excessive replacement therapy. Discontinuation of the sodium supplement after three months of therapy resulted in a return of muscle electrolyte values to normal. During the period of poor growth common to these patients a different pattern was observed. Sodium and water accumulated without alteration in tissue potassium. The mechanism of this alteration is not clear; however, it is consistent with the known effects of excess cortisone on muscle composition. These observations permit the conclusion that at least two fractions of sodium are present in muscle fibers, that which exchanges potassium and that which is independent of potassium metabolism.

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Cortisone; Desoxycorticosterone; Female; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Male; Mixed Function Oxygenases; Muscles; Potassium; Pregnanetriol; Shock; Sodium; Sodium Chloride; Water-Electrolyte Balance

On account of the increasing number of patients with essential obesity the delimitation of the Cushing-syndrome is of actual importance. Two observations of hypercortisolism in hyperplasia of the adrenal glands are reported on and the differential-diagnostically important parameters are demonstrated. According to our opinion anamnesis and clinical findings give the possibility of making a diagnosis already on a large scale. Analyses of the hormones and special X-ray examinations only serve for the preoperative ascertainment and localisation of the endocrine defective function. With the help of literary data causes and possibilities of treatment of Cushing's syndrome are entered.

Topics: 17-Ketosteroids; Adenoma; Adolescent; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adult; Cushing Syndrome; Diagnosis, Differential; Diet, Reducing; Female; Humans; Hydrocortisone; Male; Obesity

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Androstenedione; Androstenols; Androsterone; Cholestanes; Cholesterol; Chromatography, Gas; Dehydroepiandrosterone; Etiocholanolone; Female; Humans; Pregnancy; Pregnanediol; Pregnanetriol; Solvents; Steroids; Testosterone; Time Factors; Trichloroethylene

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Adult; Amenorrhea; Androgens; Androstenedione; Contraceptives, Oral; Dehydroepiandrosterone; Depression, Chemical; Dexamethasone; Drug Combinations; Female; Humans; Hyperplasia; Infertility, Female; Mestranol; Norethynodrel; Ovarian Diseases; Ovarian Neoplasms; Sertoli-Leydig Cell Tumor; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Cushing Syndrome; Drug Therapy, Combination; Humans; Neomycin; Sulfanilamides

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Body Height; Bone Development; Child; Child, Preschool; Cortisone; Disorders of Sex Development; Dose-Response Relationship, Drug; Female; Glucocorticoids; Growth; Humans; Hydrocortisone; Infant; Ketosteroids; Male; Metabolism, Inborn Errors; Mixed Function Oxygenases; Prednisone; Pregnanediol; Pregnanetriol; Puberty, Precocious; Syndrome

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Aminoglutethimide; Cushing Syndrome; Female; Hormones, Ectopic; Humans; Hydrocortisone; Melanoma; Metyrapone; Middle Aged; Mitotane; Neoplasm Metastasis; Palliative Care

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Aged; Circadian Rhythm; Diencephalon; Endocrine System Diseases; Female; Humans; Hypopituitarism; Male; Middle Aged; Pituitary Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Amenorrhea; Androgens; Estrogens; Female; Hirsutism; Humans; Infertility, Female; Mosaicism; Obesity; Pituitary Diseases; Polycystic Ovary Syndrome; Pregnanediol; Pregnanetriol; Sex Chromosome Aberrations; Virilism

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Adult; Amenorrhea; Biopsy; Body Temperature; Endometrium; Female; Humans; Hysterosalpingography; Infertility, Female; Time Factors; Vaginal Smears

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Androstanes; Cholesterol; Female; Humans; Metyrapone

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Adrenal Glands; Adrenocortical Hyperfunction; Adult; Amenorrhea; Cortisone; Dexamethasone; Estriol; Feedback; Female; Fetal Death; Hirsutism; Humans; Hydrocortisone; Hyperplasia; Hypothalamus; Maternal-Fetal Exchange; Pituitary-Adrenal System; Pregnancy; Pregnancy Complications; Pregnanediol; Pregnanetriol; Time Factors

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Androstenedione; Androsterone; Estrogens; Feminization; Gynecomastia; Humans; Hydrocortisone; Male; Pituitary-Adrenal Function Tests; Pregnanetriol; Sex Chromatin; Spermatogenesis; Testis; Testosterone

Topics: 17-Ketosteroids; Adenocarcinoma; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Colon, Sigmoid; Dexamethasone; Hormones, Ectopic; Humans; Male; Middle Aged; Radioimmunoassay; Sigmoid Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Aldosterone; Body Weight; Female; Humans; Hydrocortisone; Male; Middle Aged; Natriuresis; Potassium; Pregnanetriol; Radioimmunoassay; Secretory Rate; Stimulation, Chemical; Tritium

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenalectomy; Adrenocortical Hyperfunction; Adult; Cushing Syndrome; Dexamethasone; Female; Glucocorticoids; Humans; Hyperplasia; Middle Aged; Pituitary Irradiation; Postoperative Care; Postoperative Complications; Radiography

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Child; Cholesterol; Dexamethasone; Female; Humans; Hydrocortisone; Male; Prednisolone; Virilism

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Age Factors; Aged; Diabetes Complications; Female; Glucose Tolerance Test; Humans; Prednisone; Pregnanetriol

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Cushing Syndrome; Diagnosis, Differential; Female; Humans; Hydrocortisone; Middle Aged; Obesity

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Child; Female; Humans; Hyperplasia; Infant, Newborn; Karyotyping; Sex Chromatin; Sex Chromosomes

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Gland Neoplasms; Adrenal Insufficiency; Adrenalectomy; Adrenocortical Hyperfunction; Adult; Alkaline Phosphatase; Cushing Syndrome; Female; Humans; Hyperostosis Frontalis Interna; Hypothalamic Diseases; Hypothyroidism; Leukocytes; Male; Middle Aged; Neutrophils; Obesity; Osteoporosis

Topics: 17-Ketosteroids; Acne Vulgaris; Adolescent; Adrenocortical Hyperfunction; Adult; Androgens; Child; Dexamethasone; Endocrine System Diseases; Estrogens; Female; Hirsutism; Humans; Menstruation Disturbances; Obesity; Ovarian Diseases; Progesterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Amenorrhea; Carcinoma; Creatinine; Estrogens; Female; Hirsutism; Humans; Hydrocortisone; Hyperplasia; Neoplasm Metastasis; Pituitary-Adrenal System; Pregnanetriol; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Glands; Adrenalectomy; Adrenocortical Hyperfunction; Aldosterone; Blood Pressure; Female; Humans; Hyperaldosteronism; Hyperplasia; Middle Aged; Potassium; Remission, Spontaneous; Renin; Secretory Rate

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-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Carcinoma, Bronchogenic; Dexamethasone; Female; Hormones, Ectopic; Humans; Lung Neoplasms; Middle Aged

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Child; Dehydroepiandrosterone; Female; Humans; Hydrocortisone; Hydroxysteroid Dehydrogenases; Metabolism, Inborn Errors; Pituitary-Adrenal Function Tests; Pregnanes; Pregnanetriol; Steroids

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Aminoglutethimide; Anemia, Hemolytic; Aniline Compounds; Anticonvulsants; Autopsy; Bronchial Neoplasms; Estrogens; Humans; Male; Middle Aged; Pyridones; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Abnormalities, Multiple; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Corticosterone; Desoxycorticosterone; Female; Growth Hormone; Humans; Hydrocortisone; Hydroxyprogesterones; Hyperplasia; Hypoglycemia; Infant; Islets of Langerhans; Kidney Diseases; Metabolism, Inborn Errors; Mixed Function Oxygenases; Pregnanediol; Pregnanetriol; Pregnenolone; Vanilmandelic Acid

Topics: 17-Ketosteroids; Acetates; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Androgens; Androstanes; Butyrates; Child; Chromatography, Gas; Female; Formates; Humans; Male; Middle Aged; Propionates; Sterols; Valerates

Topics: 17-Ketosteroids; Addison Disease; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenal Glands; Adrenal Hyperplasia, Congenital; Adrenal Insufficiency; Adrenocortical Hyperfunction; Cholesterol; Cushing Syndrome; Deficiency Diseases; Dehydroepiandrosterone; Female; Menstruation Disturbances; Mixed Function Oxygenases; Virilism

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Chemistry, Clinical; Chromatography, Gas; Cushing Syndrome; Dehydroepiandrosterone; Diagnosis, Differential; Female; Glucocorticoids; Hirsutism; Humans; Pregnanediol; Pregnanetriol

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Androgens; Carcinoma, Bronchogenic; Chromatography, Gas; Chromatography, Thin Layer; Cushing Syndrome; Female; Humans; Lung Neoplasms; Male; Middle Aged; Neoplasm Metastasis

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma, Basophil; Adrenalectomy; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Chromatography, Paper; Cushing Syndrome; Dexamethasone; Female; Heart Arrest; Humans; Metyrapone; Pituitary Neoplasms; Pituitary-Adrenal Function Tests; Postoperative Complications

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Cushing Syndrome; Female; Humans; Hydrocortisone; Middle Aged

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Aminoglutethimide; Bronchial Neoplasms; Cushing Syndrome; Glucocorticoids; Humans; Hyperpituitarism; Lung Neoplasms; Metyrapone

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Aged; Bone Neoplasms; Esophageal Neoplasms; Female; Humans; Lung Neoplasms; Male; Middle Aged; Neoplasm Metastasis; Neoplasms; Skin Neoplasms; Stomach Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Body Weight; Diabetes Mellitus; Female; Humans; Obesity

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Androgens; Androstanes; Child, Preschool; Female; Humans; Hyperplasia; Mixed Function Oxygenases; Pregnanetriol; Testosterone

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Androsterone; Blood Protein Disorders; Chemistry, Clinical; Chromatography, Gel; Dehydroepiandrosterone; Diabetes Complications; Diabetes Mellitus; Etiocholanolone; Family; Female; Genital Diseases, Male; Hemophilia A; Humans; Intellectual Disability; Male; Pregnanetriol; Protein Binding; Transcortin

Topics: 17-Ketosteroids; Adenoma; Adolescent; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Androsterone; Chemistry, Clinical; Chromatography, Paper; Dehydroepiandrosterone; Diagnosis, Differential; Etiocholanolone; Hirsutism; Humans; Hydrocortisone; Hydroxysteroid Dehydrogenases; Hyperplasia; Metabolism, Inborn Errors; Middle Aged

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Carbon Isotopes; Chemistry, Clinical; Chromatography, Paper; Crystallization; Female; Humans; Hyperplasia; Ovarian Cysts; Polycystic Ovary Syndrome; Progesterone

Topics: 17-Ketosteroids; Adolescent; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Cushing Syndrome; Dexamethasone; Female; Humans; Hyperplasia; Male; Metyrapone; Pituitary-Adrenal Function Tests; Pregnanes

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Addison Disease; Adrenal Gland Neoplasms; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Cushing Syndrome; Dexamethasone; Humans; Hydrocortisone; Hypopituitarism; Hypothalamo-Hypophyseal System; Methods; Metyrapone; Pituitary-Adrenal Function Tests; Pregnanediol; Pregnanetriol; Stimulation, Chemical

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adult; Blood Cell Count; Eosinophils; Humans; Middle Aged; Pituitary-Adrenal System; Tuberculosis, Pulmonary

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Abdomen; Adrenocortical Hyperfunction; Alopecia; Animals; Asthenia; Dexamethasone; Dog Diseases; Dogs; Feeding and Eating Disorders; Female; Glucocorticoids; Glucose Tolerance Test; Humans; Hyperplasia; Hypertrophy; Liver Diseases; Male; Obesity; Pituitary-Adrenal Function Tests; Radiography; Thirst; Thyrotropin

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Aminoglutethimide; Aniline Compounds; Humans; Infant; Lung Neoplasms; Male; Neoplasm Metastasis; Pulmonary Embolism; Pyridones; Virilism

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenocortical Hyperfunction; Adult; Child; Child, Preschool; Cortisone; Dexamethasone; Estradiol; Estriol; Estrone; Female; Follicle Stimulating Hormone; Gonadotropins, Pituitary; Humans; Hyperplasia; Luteinizing Hormone; Male; Pituitary-Adrenal System; Prednisolone; Pregnanediol; Pregnanetriol

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Aldosterone; Carbon Dioxide; Child; Child, Preschool; Chlorine; Desoxycorticosterone; Female; Humans; Hypertension; Infant, Newborn; Male; Natriuresis; Potassium; Prednisolone; Renin; Sodium; Sodium Chloride; Virilism

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Aldosterone; Child; Child, Preschool; Dexamethasone; Diet, Sodium-Restricted; Female; Humans; Infant; Male; Natriuresis; Renin; Secretory Rate; Sodium

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Child; Chromatography, Paper; Cushing Syndrome; Female; Glucuronidase; Humans; Hydroxysteroid Dehydrogenases; Male; Methods; Middle Aged; Pregnanetriol; Pseudomonas

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenocarcinoma; Adrenocortical Hyperfunction; Aged; Carcinoma; Carcinoma, Bronchogenic; Carcinoma, Squamous Cell; Estrogens; Female; Hormones; Humans; Hydrocortisone; Lung Neoplasms; Male; Middle Aged; Pituitary-Adrenal Function Tests; Sex Factors; Steroids

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Dexamethasone; Female; Hirsutism; Humans; Ovary

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Cushing Syndrome; Diagnosis, Differential; Glucocorticoids; Humans; Hyperaldosteronism; Middle Aged

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Child, Preschool; Electrocardiography; Female; Humans; Infant, Newborn; Male

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Chorionic Gonadotropin; Dexamethasone; Diagnosis, Differential; Female; Hirsutism; Humans; Obesity; Pituitary-Adrenal Function Tests; Polycystic Ovary Syndrome

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Dexamethasone; Diagnosis, Differential; Humans

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Glands; Adrenocortical Hyperfunction; Adult; Aminoglutethimide; Anticonvulsants; Cushing Syndrome; Female; Humans; Hydrocortisone; Middle Aged; Natriuresis; Secretory Rate

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adult; Chlorpromazine; Humans; Hypopituitarism; Infertility, Male; Male; Nutrition Disorders; Puberty, Precocious; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenocortical Hyperfunction; Adult; Cushing Syndrome; Female; Humans; Male; Phenethylamines; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adult; Androstanes; Androsterone; Carcinoma; Chemistry, Clinical; Chromatography, Thin Layer; Cushing Syndrome; Dehydroepiandrosterone; Etiocholanolone; Female; Humans; Kidney Neoplasms; Lung Neoplasms; Male; Methods; Ovarian Diseases; Testicular Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Aminoglutethimide; Animals; Anticoagulants; Carcinoma; Cushing Syndrome; Female; Humans; Hydrocortisone; Metyrapone; Middle Aged; Pituitary-Adrenal Function Tests; Secretory Rate

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Animals; Dog Diseases; Dogs; Electroencephalography; Female; Metyrapone; Pituitary-Adrenal Function Tests

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Child; Child, Preschool; Chromatography, Gas; Ethisterone; Female; Humans; Infant; Male; Metyrapone; Pregnanetriol

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Ataxia; Child; Child, Preschool; Dwarfism; Gonads; Humans; Intellectual Disability; Myxedema; Obesity; Puberty, Precocious

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adult; Cushing Syndrome; Female; Humans; Hydrocortisone; Male; Middle Aged; Obesity; Peptic Ulcer; Secretory Rate

Topics: 17-Ketosteroids; Adenoma; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenalectomy; Adrenocortical Hyperfunction; Adult; Carcinoma; Child; Child, Preschool; Cushing Syndrome; Female; Humans; Hyperaldosteronism; Hypertension; Intestinal Polyps; Male; Pheochromocytoma

Topics: 17-Ketosteroids; Adrenal Glands; Adrenocortical Hyperfunction; Cerebrovascular Disorders; Glucocorticoids; Humans; Hypertension; Methods; Pituitary-Adrenal Function Tests; Potassium; Sodium

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenocortical Hyperfunction; Adult; Aged; Female; Glucocorticoids; Humans; Male; Middle Aged; Myocardial Infarction

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Antineoplastic Agents; Cushing Syndrome; Dichlorodiphenyldichloroethane; Female; Humans; Male

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Aged; Cushing Syndrome; Dexamethasone; Female; Humans; Male; Metyrapone; Middle Aged; Pituitary Function Tests; Pituitary-Adrenal Function Tests; Pregnanediol; Pregnanetriol; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Female; Humans; In Vitro Techniques; Male; Middle Aged; Pituitary-Adrenal Function Tests; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Child; Child, Preschool; Female; Glucocorticoids; Humans; Male

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adult; Aged; Diagnosis, Differential; Female; Humans; In Vitro Techniques; Male; Middle Aged; Obesity; Pituitary-Adrenal Function Tests

The isotopic estimation of the cortisol secretion rate in man is now an accepted and reliable method of assaying adrenal cortical function.Seven years' experience with its clinical use is reviewed and some practical aspects of technique are considered. The mean normal resting cortisol secretion rate is 16.2 +/- 5.7 mg. daily. In all of 14 cases of hypopituitarism studied secretion has been less than 2.1 mg. daily. In all of 26 cases of established Cushing's syndrome, the cortisol secretion was above 36 mg. The method can be used to follow day-by-day changes in adrenal cortisol activity and examples of such use are given. The urinary 17-ketogenic steroid excretion frequently gives results which conflict with secretion rate estimates, and can lead to erroneous clinical conclusions.

Topics: 17-Ketosteroids; Addison Disease; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Carbon Isotopes; Cushing Syndrome; Humans; Hydrocortisone; Hypopituitarism; Physiology; Pituitary-Adrenal Function Tests; Tritium; Urine

Topics: 17-Ketosteroids; Adolescent; Adrenalectomy; Adrenocortical Hyperfunction; Carbon Isotopes; Child; Cushing Syndrome; Humans; Hydrocortisone; Metabolism; Pituitary ACTH Hypersecretion; Pituitary Irradiation; Pituitary-Adrenal Function Tests

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Chromatography, Gel; Colorimetry; Cryptorchidism; Cushing Syndrome; Fluorometry; Humans; Hydrocortisone; Male; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Alkalosis; Carcinoma; Carcinoma, Squamous Cell; Esophageal Neoplasms; Humans; Hypokalemia; Liver Neoplasms; Neoplasm Metastasis; Pathology; Pituitary-Adrenal Function Tests

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Acid Phosphatase; Adenosine Triphosphatases; Adrenal Gland Neoplasms; Adrenal Glands; Adrenocortical Hyperfunction; Alkaline Phosphatase; Electron Transport Complex II; Esterases; Glucosephosphate Dehydrogenase; Histocytochemistry; Humans; Oxidoreductases; Succinate Dehydrogenase; Urine

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Chemical Fractionation; Chromatography; Dose Fractionation, Radiation; Humans; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Acne Vulgaris; Adolescent; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adult; Amenorrhea; Brain; Diagnosis; Female; Hirsutism; Humans; Hypertension; Metabolic Diseases; Mixed Function Oxygenases; Obesity; Urine; Virilism

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenocarcinoma; Adrenalectomy; Adrenocortical Hyperfunction; Aged; Dexamethasone; Diethylstilbestrol; Humans; Male; Neoplasm Metastasis; Neoplasms; Pathology; Prostatic Neoplasms; Surgical Procedures, Operative; Toxicology; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Blood; Cushing Syndrome; Humans; Hydrocortisone; Pathology; Urine

The production of ACTH-like material by tumours arising in non-endocrine tissue may initiate severe adrenocortical hyperfunction. The pathogenesis and clinical and laboratory features of Cushing's syndrome associated with such tumours are characteristic. The autonomous production by the tumour of ACTH-like material cannot be suppressed by exogenous corticoids. The onset of clinical symptoms is rapid; muscle wasting, general weakness, thirst and peripheral edema predominate, and the classical signs of Cushing's syndrome may be absent. High levels of plasma 17-hydroxycorticosteroids and urinary 17-hydroxycorticosteroids and 17-ketosteroids, usually with normal levels of urinary aldosterone, commonly occur. Hypokalemic alkalosis unresponsive to replacement therapy may cause death. In the case reported herein, the intriguing possibility exists that two hormone-like substances were produced by the primary growth and its metastases: one, ACTH-like, to account for the adrenal hyperplasia and Cushing's syndrome; and another, gastrin-like, giving rise to the ulcerogenic diathesis.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma, Islet Cell; Adrenalectomy; Adrenocortical Hyperfunction; Autopsy; Carcinoma, Islet Cell; Chloramphenicol; Cushing Syndrome; Drug Therapy; Gastrins; Humans; Hypokalemia; Neoplasm Metastasis; Neoplasms; Pancreatic Neoplasms; Pathology; Spironolactone; Zollinger-Ellison Syndrome

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Aldosterone; Female; Heparin; Humans; Male; Middle Aged; Urine

Topics: 17-Ketosteroids; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Aldosterone; Humans; Pemphigus

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenalectomy; Adrenocortical Hyperfunction; Adult; Cushing Syndrome; Dietary Proteins; Fasting; Female; Humans; Hydrocortisone; In Vitro Techniques; Male; Obesity; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Adult; Female; Hirsutism; Humans; Metronidazole; Skin

Five patients with bronchogenic carcinoma associated with adrenocortical hyperfunction are described. The clinical features, laboratory studies and autopsy findings are discussed and compared with previously reported cases. Four patients presented most of the typical features of this disorder as previously described, whereas the fifth was atypical in some respects. Typical features included: acute onset of adrenocortical hyperfunction in a middle-aged male, rapid downhill course, slight or absent physical signs of Cushing's syndrome, frequently impaired glucose tolerance, markedly elevated plasma and urinary 17-hydroxycorticosteroids not suppressed by exogenous steroids, absent diurnal variation of plasma corticoids, hypokalemic alkalosis with normal aldosterone excretion, and tumour histology of the oat cell variety. The adrenal glands of two patients were of normal or slightly increased weight, and mean 17-ketosteroid excretion values were normal in three; this contrasts with the marked increase in adrenal weight and 17-ketosteroid excretion in most reported cases.

Topics: 17-Ketosteroids; Adrenal Glands; Adrenocortical Hyperfunction; Adult; Alkalosis; Autopsy; Blood; Carcinoma, Bronchogenic; Clinical Laboratory Techniques; Glucose Tolerance Test; Humans; Hypokalemia; Lung Neoplasms; Male; Middle Aged; Urine

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenalectomy; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Aldosterone; Androgens; Cushing Syndrome; Humans; Hyperplasia; Hypertrophy; Pathology; Pituitary-Adrenal Function Tests; Sympatholytics

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenalectomy; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Cushing Syndrome; Humans; Hyperaldosteronism; Pheochromocytoma

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Blood Chemical Analysis; Cortisone; Female; Hair; Hirsutism; Humans; Hypertrichosis; Pregnancy; Pregnancy Complications; Progesterone; Testosterone; Urine; Virilism

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenalectomy; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Blood Chemical Analysis; Cortisone; Cushing Syndrome; Dexamethasone; Humans; Hyperpigmentation; Pigmentation Disorders; Pituitary ACTH Hypersecretion

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenalectomy; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Corticosterone; Cushing Syndrome; Dexamethasone; Disorders of Sex Development; Glucose Tolerance Test; Humans; Surgical Procedures, Operative; Urine

Topics: 17-Ketosteroids; Adrenalectomy; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Corticosterone; Cushing Syndrome; Humans; Hyperplasia; Injections, Intramuscular; Suppositories; Urine

Korsakoff's syndrome of obscure etiology was observed in a 34-year-old single woman with an 11-year history of hirsutism and mood swings, and previous hospitalizations for mania three years ago and depression 11 years ago.Recently the virilism had intensified with increased muscularity and coarsening of facial features. The 24-hour urinary 17-ketosteroids ranged between 14.4 mg. and 21.5 mg. and were suppressed by dexamethasone. The 17-hydroxycorticosteroid excretion was normal. These and other findings suggested a diagnosis of adrenal virilism due to adrenocortical hyperplasia. In the absence of other discernible causes it appeared that the adrenal pathology was responsible for the Korsakoff's syndrome. Both conditions responded well to glucocorticoid therapy although low doses were necessary to avoid mania.It is speculated that the encephalopathy was due to an associated adrenal insufficiency. Although hypoadrenalism is accepted as a complication of only the infant form of adrenal virilism, it is noteworthy that this patient had pathological pigmentation of her skin.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Amobarbital; Antipsychotic Agents; Barbiturates; Female; Glutethimide; Hirsutism; Humans; Hypertrichosis; Korsakoff Syndrome; Phenothiazines; Prednisone; Thioridazine; Trifluoperazine; Virilism; Wernicke Encephalopathy

Topics: 17-Ketosteroids; Acid-Base Equilibrium; Adrenocortical Hyperfunction; Alkalosis; Carcinoma; Electrocardiography; Humans; Hypokalemia; Lung Neoplasms; Lymphatic Metastasis; Potassium; Sodium; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Dexamethasone; Fatigue; Fever; Humans; Hyperplasia; Male; Pituitary-Adrenal Function Tests; Prednisone; Urine

Topics: 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Isoniazid; Radiography, Thoracic; Toxicology; Tuberculosis; Tuberculosis, Pulmonary

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocortical Hyperfunction; Drug Therapy; Humans; Isoniazid; Obesity; Toxicology; Tuberculosis; Tuberculosis, Pulmonary

Topics: 17-Ketosteroids; Acromegaly; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Androgens; Chromatography; Ketones; Metabolism; Mineralocorticoid Receptor Antagonists; Pituitary-Adrenal Function Tests; Urine

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Age Determination by Skeleton; Blood Chemical Analysis; Classification; Cortisone; Desoxycorticosterone; Drug Therapy; Humans; Hydrocortisone; Hyperplasia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Urine

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Endocrine System Diseases; Estrogens; Female; Gonadotropins; Humans; Hyperplasia; Hysterectomy; Obesity; Ovary; Physiology; Pituitary Gland; Postoperative Complications; Pregnanediol; Rats; Research; Thyroid Diseases; Urine; Uterus

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Abdomen; Abdomen, Acute; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Betamethasone; Diagnosis; Humans; Neoplasms; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Betamethasone; Dexamethasone; Diethylstilbestrol; Drug Therapy; Female; Follicle Stimulating Hormone; Hirsutism; Humans; Hypertrichosis; Hysterectomy; Ovarian Neoplasms; Polycystic Ovary Syndrome; Steroids; Urine

Topics: 17-Ketosteroids; Acne Vulgaris; Adrenocortical Hyperfunction; Alopecia; Chromatography; Female; Hirsutism; Humans; Hypertrichosis; Staphylococcal Infections; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocortical Hyperfunction; Geriatrics; Humans; Lung Neoplasms; Neoplasms; Pathology; Syndrome; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Diseases; Adrenal Cortex Hormones; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Aldosterone; Androsterone; Cortisone; Dehydroepiandrosterone; Humans; Hydrochlorothiazide; Pharmacology; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Addison Disease; Adrenal Cortex; Adrenal Gland Diseases; Adrenal Insufficiency; Adrenocortical Hyperfunction; Androsterone; Cushing Syndrome; Dehydroepiandrosterone; Humans; Hydrochlorothiazide; Hypoadrenocorticism, Familial; Hypopituitarism; Pharmacology; Pituitary-Adrenal Function Tests

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Chromatography; Humans

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenocortical Hyperfunction; Chemical Fractionation; Chromatography; Dose Fractionation, Radiation; Humans

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Gland Diseases; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Humans; Hyperplasia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Metabolic Diseases; Sodium Chloride

Topics: 17-Ketosteroids; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenalectomy; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Blood Chemical Analysis; Chromatography; Cortisone; Cushing Syndrome; Dexamethasone; Humans; Hydrocortisone; Neoplasms; Pharmacology; Pituitary-Adrenal Function Tests; Urine

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenalectomy; Adrenocortical Hyperfunction; Cushing Syndrome; Humans; Hypophysectomy; Osteoporosis; Pituitary ACTH Hypersecretion; Radiography

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Diabetes Mellitus; Diabetic Retinopathy; Pregnancy; Urine

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Black People; Diabetes Mellitus; Diabetic Retinopathy; Humans; Research; Sex; Urine; White People

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Dehydroepiandrosterone; Dexamethasone; Female; Hirsutism; Humans; Hypertrichosis; Menstruation Disturbances; Polycystic Ovary Syndrome; Urine

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Androgens; Androsterone; Cysts; Female; Humans; Ovary; Polycystic Ovary Syndrome; Testosterone; Thyroidectomy

Topics: 17-Ketosteroids; Adiposity; Adolescent; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Child; Chromatography; Gigantism; Ketones; Metyrapone; Mineralocorticoid Receptor Antagonists; Pharmacology; Pituitary Gland; Pituitary-Adrenal Function Tests; Urine

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Aldosterone; Cholesterol; Cushing Syndrome; Follicle Stimulating Hormone; Humans; Metabolism; Pituitary-Adrenal Function Tests; Pregnanediol

Topics: 17-Ketosteroids; Addison Disease; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenal Glands; Adrenocortical Hyperfunction; Cushing Syndrome; Humans; Hypopituitarism; Ketones; Metyrapone; Mineralocorticoid Receptor Antagonists; Physiology; Pituitary Gland; Pituitary-Adrenal Function Tests

Topics: 17-Ketosteroids; Acromegaly; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Clinical Laboratory Techniques; Cushing Syndrome; Cysts; Female; Humans; Hypogonadism; Hypopituitarism; Metyrapone; Mineralocorticoid Receptor Antagonists; Neoplasms; Ovary; Pharmacology; Pituitary Gland; Pituitary Neoplasms; Radioisotope Teletherapy; Scleroderma, Systemic; Sexual Infantilism; Toxicology; Urine

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Chromatography

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Aldosterone; Glucocorticoids; Humans

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Cushing Syndrome; Humans; Steroids

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenocortical Hyperfunction; Chemical Fractionation; Chromatography; Humans

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenocortical Hyperfunction; Chromatography

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Cushing Syndrome; Dose Fractionation, Radiation; Hormones; Humans; Pituitary ACTH Hypersecretion; Urinary Tract

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenocortical Hyperfunction; Blood Glucose; Cholesterol; Choline; Humans; Steroids

Topics: 17-Ketosteroids; Acromegaly; Addison Disease; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Androgens; Cushing Syndrome; Dehydroepiandrosterone; Endocrine System Diseases; Humans; Pituitary ACTH Hypersecretion; Puberty, Precocious; Steroids; Urine

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenocortical Hyperfunction; Hyperplasia; Hypertrophy; Steroids; Urine

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenocortical Hyperfunction; Body Fluids; Cortisone; Hyperplasia; Steroids

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Hormones; Adrenocortical Hyperfunction; Body Fluids; Cushing Syndrome; Humans; Radiotherapy; Steroids; Urine; X-Ray Therapy

Topics: 17-Ketosteroids; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Cushing Syndrome; Humans; Hyperplasia