17-ketosteroids and Syndrome

0.25 sodium equilin sulphate and 0.625 mg Premarin were compared as to their effects in 12 postmenopausal syndrome. The initial daily dosage of 0.25 mg equilin had a stronger oestrongenic effect in the alleviation of vasomotor disturbances than subsequently-administered Premarin. No significant difference was found in effect on the vaginal epithelium or the endometrium or in the incidence of irregular bleeding. The number of women in whom withdrawal bleeding was reported was considerably reduced by lowering the dosage to 0.2 mg equilin sulphate. Consequently 0.2 to 0.3 mg equilin sulphate must be considered the optimum dosage.

Topics: 17-Ketosteroids; Autonomic Nervous System; Climacteric; Clinical Trials as Topic; Drug Evaluation; Equilin; Female; Humans; Menopause; Middle Aged; Nervous System Diseases; Ovary; Postoperative Complications; Sleep Wake Disorders; Syndrome; Time Factors

A patient with adrenocortical carcinoma presented with fever, leukocytosis, and increased acute phase reactants. The tumor was infiltrated with neutrophils. Immunohistochemical staining of the tumor showed positive signal for epithelial neutrophil-activating protein-78, an angiogenic and chemotactic CXC chemokine. Conditioned medium from tumor-derived cells (RL-251) showed high concentration of IL-8, epithelial neutrophil-activating protein-78, Gro alpha, and Gro gamma, angiogenic CXC chemokines with a potential role in tumorigenesis. An adrenal cancer/severe combined immunodeficiency mouse chimera was developed. Mice grew tumors rapidly, and circulating levels of IL-8 and epithelial neutrophil-activating protein-78 were detected. In contrast, animals transplanted with NCI-H295 cells, a nonchemokine-secreting cell line, grew tumors more slowly and did not have detectable chemokine levels. Similar to the patient, mice with RL-251 tumors developed marked leukocytosis and neutrophilia, and their tumors were infiltrated with neutrophils. Mice were passively immunized with epithelial neutrophil-activating protein-78 antisera. A marked decrease in tumor growth was observed. Potential for chemokine production by other adrenocortical tumors was investigated by RT-PCR in archival material. Six of seven adrenal carcinomas and one of three adenomas had cDNA for IL-8; six of seven carcinomas and the three adenomas had cDNA for epithelial neutrophil-activating protein-78. We concluded that the clinical presentation of this case resulted from increased tumor production of chemotactic chemokines. Through their angiogenic and chemotactic properties these chemokines may play an important role in adrenal tumorigenesis.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Aged; Chemokine CXCL5; Chemokines, CXC; Circadian Rhythm; Fever; Humans; Hydrocortisone; Immunohistochemistry; Interleukin-8; Leukocytosis; Male; Neutrophil Activation; Neutrophils; Reverse Transcriptase Polymerase Chain Reaction; Syndrome; Tumor Cells, Cultured

A patient with a thymic carcinoid tumour causing ectopic ACTH syndrome is presented. The case illustrates the rapid development of the clinical and laboratory findings often associated with ectopic secretion of ACTH, including severe proximal myopathy, emotional lability, and hypokalaemic alkalosis. Interpretation of conventional tests of pituitary-adrenal function was complicated by intermittent secretion of ACTH by the tumour. The results of selective venous sampling for ACTH ruled out pituitary ACTH hypersecretion and were suggestive of a thymic source; computerized tomography of the chest localized the tumour. In vivo and in vitro investigations confirmed excessive ACTH production by the tumour, and surgical resection plus radiotherapy has resulted in resolution of the syndrome. The diagnostic problems created by intermittent secretion of ACTH by these tumours and the pre-operative and post-operative medical management of these patients are discussed.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocorticotropic Hormone; Adult; Carcinoid Tumor; Female; Hormones, Ectopic; Humans; Hydrocortisone; Syndrome; Thymus Gland; Thymus Neoplasms; Tomography, X-Ray Computed

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex Function Tests; Child; Child, Preschool; Cryptorchidism; Diagnosis, Differential; Gonadal Dysgenesis; Humans; Male; Phenotype; Sex Chromatin; Syndrome; Testis

Topics: 17-Ketosteroids; Adolescent; Adult; Estrogens; Humans; Male; Recurrence; Retinal Hemorrhage; Syndrome

Topics: 17-Ketosteroids; Adolescent; Adrenal Cortex; Androgens; Body Weight; Brain Diseases; Female; Humans; Hypothalamus; Male; Puberty; Syndrome

A 17 year old female patient with hypertension, amenorrhoea and hirsutism was found to have subnormal levels of plasma and urinary cortisol, significant plasma levels of Reichstein's compound S and 21-deoxycortisol, high urinary levels of THS and pregnanetriolone as well as elevated levels of plasma and urinary testosterone. Treatment with 0.5 mg/day of dexamethasone or 25 mg/day cortisone reduced her hypertension and restored her menstrual cycles, but also resulted in the development of moon face, body striae and a gain in weight. Lower doses of cortisone were without effect. The deficient cortisol production coupled with the presence of unusual intermediates such as Reichstein's compound S and 21-deoxycortisol can be explained by a shift in the substrate specificity of 11beta-hydroxylase from C-21-hydroxylated substrates (i.e. compound S) to C-21-deoxy substrates (i.e. 17-hydroxyprogesterone).

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Amenorrhea; Corticosterone; Female; Hirsutism; Humans; Hydrocortisone; Hydroxyprogesterones; Hypertension; Pregnanetriol; Pregnenediones; Steroid Hydroxylases; Syndrome; Testosterone; Thyrotropin; Transcortin

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Diagnostic Errors; Female; Humans; Infant; Male; Syndrome

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

Sexual development was evaluated in 9 female and 2 male subjects with Prader-Willi syndrome. The process of sexual development and degree of genital development attained were found to be variable but abnormal in all subjects. Hypothalamic-pituitary-gonadal functions were evaluated by measurement of serum Luteinizing Hormone and plasma testosterone responses to stimulation by clomiphene citrate and plasma testosterone responses to stimulation by human chorionic gonadotrophin. The degree of vaginal estrogenization was variable. The testicular biopsies showed abnormalities mainly in the germinal epithelium. In agreement with previous studies, it was concluded that the abnormalities of sexual development in this syndrome are mainly due to a defect in the hypothalamic pituitary axis. Adrenal function was not found to be grossly abnormal. The 17 ketosteroid excretion values were low, probably explaining the rather sparse pubic and axillary hair observed in these patients. The urinary 17-hydroxycorticosteroid creatinine ratios were found to be elevated, probably due to decreased creatinine excretion, reflecting the muscular abnormalities of these subjects.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenocorticotropic Hormone; Adult; Carbohydrate Metabolism, Inborn Errors; Child; Clomiphene; Creatinine; Female; Humans; Hypogonadism; Intellectual Disability; Luteinizing Hormone; Male; Menarche; Metyrapone; Obesity; Syndrome; Testis; Testosterone; Wechsler Scales

In two patients who were severely pigmented (Nelson syndrome) following bilateral adrenalectomy for Cushing syndrome, symptoms of hyper-cortisolism developed while they were receiving only physiologic steroid replacement. Cortical assays proved that endogenous cortisol production had not been obliterated. Even after total adrenalectomy, steroid measurement should be performed to guard against adrenocortical excess.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Function Tests; Adrenalectomy; Adrenocorticotropic Hormone; Adult; Cushing Syndrome; Female; Humans; Hydrocortisone; Pigmentation Disorders; Recurrence; Syndrome

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Abnormalities, Multiple; Adolescent; Female; Hernia, Umbilical; Humans; Maxilla; Micrognathism; Syndrome

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; Abnormalities, Multiple; Adult; Female; Follicle Stimulating Hormone; Humans; Karyotyping; Laparoscopy; Luteinizing Hormone; Physical Examination; Syndrome; Testosterone; Uterus; Vagina

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocorticotropic Hormone; Fasting; Female; Glucagon; Growth Hormone; Humans; Hypopituitarism; Luteinizing Hormone; Pituitary-Adrenal System; Pregnancy; Pregnancy Complications; Puerperal Disorders; Stimulation, Chemical; Syndrome; Thyrotropin; Thyroxine-Binding Proteins

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Anemia, Aplastic; Blood Glucose; Bone Development; Child; Growth Hormone; Humans; Insulin; Male; Syndrome; Testosterone; Thyroxine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Child; Depression, Chemical; Dexamethasone; Diabetes Mellitus; Diabetes Mellitus, Type 1; Dwarfism; Face; Female; Growth Hormone; Hepatomegaly; Humans; Hydrocortisone; Insulin; Metyrapone; Obesity; Syndrome

Topics: 17-Ketosteroids; Adolescent; Adult; Carbohydrate Metabolism, Inborn Errors; Chorionic Gonadotropin; Clomiphene; Estradiol; Female; Follicle Stimulating Hormone; Glucose Tolerance Test; Gonadotropin-Releasing Hormone; Growth Hormone; Humans; Hydrocortisone; Hypothalamo-Hypophyseal System; Intellectual Disability; Iodine Radioisotopes; Luteinizing Hormone; Male; Metyrapone; Obesity; Pituitary Gland; Pituitary Gland, Anterior; Prolactin; Radioimmunoassay; Syndrome; Testosterone; Tetrahydrocortisol; Tetrahydrocortisone; Thyrotropin

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Delayed-Action Preparations; Diabetes Mellitus; Diabetes Mellitus, Type 1; Diet, Diabetic; Dwarfism; Face; Female; Follow-Up Studies; Glycosuria; Hepatomegaly; Humans; Insulin; Obesity; Syndrome

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; Adolescent; Electroencephalography; Endocrine System Diseases; Fibrous Dysplasia of Bone; Humans; Male; Pigmentation Disorders; Puberty, Precocious; Radiography; Syndrome

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adult; Alopecia; Deafness; Dermatoglyphics; Follicle Stimulating Hormone; Growth Hormone; Humans; Hydrocortisone; Hypogonadism; Intelligence; Luteinizing Hormone; Male; Metyrapone; Pedigree; Pituitary-Adrenal Function Tests; Syndrome; Testosterone; Thyroid Function Tests

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Aldosterone; Cerebellar Ataxia; Child; Chorionic Gonadotropin; Electromyography; Follicle Stimulating Hormone; Growth Hormone; Hearing Disorders; Humans; Hydrocortisone; Intellectual Disability; Luteinizing Hormone; Male; Nerve Degeneration; Neural Conduction; Pedigree; Syndrome; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adult; Biopsy; Blood Cells; Body Height; Body Weight; Fibroblasts; Gonadotropins; Humans; Hypogonadism; Karyotyping; Male; Mosaicism; Mouth Mucosa; Sex Chromatin; Sex Chromosome Aberrations; Skin; Syndrome; Testis; Testosterone

Topics: 17-Ketosteroids; Adult; Aged; Aldosterone; Chromatography, Gas; Desoxycorticosterone; Dexamethasone; Female; Humans; Hydroxycorticosteroids; Hypertension; Hypokalemia; Male; Middle Aged; Potassium; Radioimmunoassay; Renin; Spironolactone; Syndrome

Four types of acanthosis nigricans (a.n.) must be distinguished: "benign" a.n., pseudo a.n., a.n. as part of a syndrome and malignant a.n. These different types affect different age groups, follow different courses, are associated with different conditions, are or are not reversible, have or do not have a genetic basis and also show great differences in regard to their significance for the patient's life or well-being.

Topics: 17-Ketosteroids; Acanthosis Nigricans; Adenocarcinoma; Adolescent; Adult; Age Factors; Child; Colonic Neoplasms; Diethylstilbestrol; Female; Humans; Male; Nicotinic Acids; Obesity; Syndrome

Topics: 17-Ketosteroids; Female; Humans; Leydig Cell Tumor; Male; Neoplasms; Ovarian Neoplasms; Pathology; Retropneumoperitoneum; Surgical Procedures, Operative; Syndrome; Virilism

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex Hormones; Adrenocorticotropic Hormone; Aldosterone; Birth Order; Consanguinity; Desoxycorticosterone; Genetics, Medical; Humans; Hyperkalemia; Hyponatremia; Infant; Metabolism, Inborn Errors; Pathology; Sex; Syndrome

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

Topics: 17-Ketosteroids; Addison Disease; Adolescent; Adrenal Insufficiency; Adrenocorticotropic Hormone; Chromosome Aberrations; Chromosomes; Cortisone; Desoxycorticosterone; Diffuse Cerebral Sclerosis of Schilder; Humans; Hypoadrenocorticism, Familial; Myocarditis; Nystagmus, Pathologic; Pathology; Sweat; Syndrome; Tuberous Sclerosis; Urine; Water-Electrolyte Balance

Topics: 17-Ketosteroids; Androgens; Female; Humans; Syndrome; Virilism

Topics: 17-Ketosteroids; Adrenal Gland Diseases; Adrenal Glands; Disease; Humans; Steroids; Syndrome; Urinary Tract; Urine