17-ketosteroid Levels Research Articles

The Incidence of Associated Anomalies in 105 Patients with Congenital Adrenal Hyperplasia

A survey of 105 patients with defective adrenal steroid 21-hydroxylation, the most common defect in congenital adrenal hyperplasia, has been carried out to determine the presence of additional congenital or developmental anomalies. The following is a report of this survey. The diagnosis of congenital adrenal hyperplasia was suspected in infants who presented with ambiguous genitalia or with defect in electrolyte metabolism manifested by failure to thrive, vomiting and dehydration with hyponatremia and hyperkalemia. Measurement of elevated levels of urinary 17-ketosteroids, 11-keto pregnantriol and pregnantriol confirmed the diagnosis. Seventy-two (68.6%) of the 105 patients surveyed demonstrated defects in electrolyte metabolism and 39 of these were males.

Diurnal Variations in the Levels of Plasma and Urinary Androgens

ABSTRACT In 12 normal male subjects the urinary levels of testosterone, epitestosterone3 and 17-ketosteroids (17-KS) were determined for 6 consecutive 4-hr periods. In 5 of the subjects the levels of plasma luteinizing hormone (LH), testosterone and corticosteroids (CS) and urinary 17-hydroxycorticosteroids (17-OHCS) were also determined to compare with those of urinary testosterone, epitestosterone and 17-KS. Significant variations of plasma CS and testosterone, and urinary 17-KS, 17-OHCS, testosterone and epitestosterone were found. However, the magnitudes of the diurnal variations of plasma testosterone, and urinary testosterone and epitestosterone were much smaller than those observed for plasma CS and urinary 17-KS and 17-OHCS. In two of the 5 subjects there was a large change in the concentration of plasma LH during the day, whereas no change was observed in 3 other subjects. These data do not permit the conclusion that a diurnal variation of plasma testosterone is a result of changing LH levels. A ...

Anorchia. A variety of the "empty scrotum".

A 19-year-old boy, referred for treatment of bilateral undescended testicles, was found to have congenital anorchia. Conditions other than cryptorchidism may mimic this anomaly. These include severe virilization of a female child, idiopathic pseudohermaphorditism, true hermaphroditism, and the male counterpart of Turner's syndrome. A method of evaluating the empty scrotum is proposed to facilitate early diagnosis and treatment of anorchia. The goal is to prevent progressive eunuchoidism and the stigmata of sexual infantilism. Unlike most anorchid patients, the one reported here had normal body measurements and bone age. He also had relatively high levels of urinary total neutral 17-ketosteroids. Increased production of androgen precursors by the adrenal cortex may have prevented eunuchoidism without stimulating secondary sexual development.

Ovarian lipoid cell tumor: urinary steroid response to adrenocorticotropin and chorionic gonadotropin.

ABSTRACT The excretion of 17-hydroxycorticosteroids, total and fractional 17-ketosteroids, pregnanetriol, pregnanediol and testosterone were determined in the urine of a 34-yr-old Negro woman with virilization caused by an ovarian lipoid cell tumor. Urinary levels of 17-ketosteroids (17-KS), androsterone (A) and etiocholanolone (E) were significantly elevated, were not suppressed by dexamethasone, but were augmented by metyrapone, presumably through increased ACTH secretion. Human chorionic gonadotropin (HCG) administration also resulted in a marked increase in 17-KS. Basal levels of pregnanetriol and testosterone glucuronide, significantly above normal, were further elevated by HCG and metyrapone. These findings are interpreted to indicate that the tumor secreted 17-hydroxyprogesterone and an androgen which may have been androstenedione, testosterone or both. 11-Hydroxylase function characteristic of adrenal steroid synthesis was not proved for the tumor, but the increased excretion of pregnanetriol and ...

Urinary steroid excretion before and after dexamethasone administration and steroid content of adrenal tissue and venous blood in virilizing adrenal tumors

Urinary steroid excretion patterns and histopathology of tumors were studied in 5 females with a diganosis of pure virilizing adrenal tumors. The routine 17-ketosteroid and total fractionated 17-ketostnoid values were elevated. The elevation was primarily in the 11-deoxy-17-ketosteroid fraction, with dehydroepiandrosterone being a prominent contributor. In 4 of the 5 cases, the 17-ketosteroids could not be suppressed by dexamethasone administration. There was no discernible relationship between the degree of virilization and urinary levels of 17-ketosteroids. The 17-ketogenic steroids and fractionated tetrahydrocorticoids were normal and suppressed well in 4 cases and partially in the fifth. This apparent separation of corticosteroid and androgen biosynthesis in the adrenal is of considerable interest. Study of peripheral and adrenal vein blood obtained during surgery in 3 cases confirmed adrenal secretion of d ehydroepiandrosterone, dehydroepiandrosterone sulfate, and Δ4-androstenedione. In one case a significant amount of testosterone was secreted by the adrenal.

A comparative study of the biosynthesis of testosterone by human testes and a virilizing interstitial cell tumour.

ABSTRACT A comparative in vitro study of an interstitial cell tumour of the testis from a virilized patient and »normal« testes from another male has been made by incubating homogenates of these tissues with radioactive progesterone, 17α-hydroxyprogesterone, testosterone and androst-4-ene-3,17-dione-4-14C. The patient with the testicular tumour was a 14-year old boy whose clinical studies, reported in detail elsewhere, revealed a history of early puberty and laboratory findings of markedly elevated levels of urinary total 17-ketosteroids and pregnanetriol. These urinary values fell to normal following removal of the right testicular tumour, which was interpreted as being a benign interstitial cell tumour on histologic examination. The radioactive metabolites from the testicular tissue of both subjects were separated, purified and identified by partition column chromatography in various systems with authentic standards, formation of derivatives and isotopic dilution analysis. The interstitial cell tumour converted a significant amount of the substrate progesterone into 16α-hydroyprogesterone, besides testosterone and androst-4-ene-3,17-dione; reduction at C20 was also a major reaction when either progesterone or 17α-hydroxyprogesterone was the substrate. On a comparative basis the activity of C17-20 desmolase was about five times more in the tumour as compared to the testicular tissue.

Bilateral galactocele in a male infant

<h2>Summary</h2> This is the first report of bilateral galactocele in a male infant demonstrated to have negative sex chromatin pattern and normal levels of urinary 17-ketosteroids and total estrogens. The composition of the milklike contents of the cysts was 4.8 per cent fat, 5.7 per cent protein, and 7.5 per cent lactose. Microscopic sections of the cyst walls revealed secretory activity of dilated breast ductules and absence of inflammatory changes or pathologic plugging of ducts. It is concluded that these retention cysts are the result of nonneoplastic obstruction of breast ducts.

Conversion of 17α-hydroxypregnenolone to dehydroepiandrosterone bt adrenal homdgenates of immature and adult guinea pigs

The conversion of 17α-hydroxypregnenolone-7- 3H into dehydroepiandrosterone-7- 3H by incubates of guinea pig adrenal homogenates has been demonstrated and its significance studied. This conversion was greater with homogenates than with minced tissue. The addition of Δ 4-androstenedione to the homogenates significantly increased the specific activity of the formed trapped dehydroepiandrosterone. This conversion required an hydrogen generating system and occurred quite rapidly at pH 6.88. Adrenals from, guinea pigs 1 month and 6 to 8 months old formed dehydroepiandrosterone with radioactivity per gram, of tissue 3 to 7 times that from the adrenals of animals 1 1 2 to 2 years of age. This decrease in the conversion of 17α-hydroxypregnenolone to dehydroepiandrosterone could well account for the decline in the urinary excretion and plasma levels of 17-ketosteroids with increasing age.

Adrenal-Pituitary Function in Bronchial Asthma

THE POSSIBILITY that the adrenal and pituitary glands play an important role in bronchial asthma has been suggested by many investigators. Rackemann in 1945 reported low levels of urinary 17-ketosteroids in patients with bronchial asthma. 1 Since then many investigators have noted similar findings. 2-5 Rose et al, in a study of 58 asthmatic patients, showed that there was a diminished urinary glucocorticoid excretion which persisted as long as the asthmatic attack. 6 Spaner and associates 7 concluded that urinary 11-hydroxysteroids are reduced during an asthmatic attack. Low levels of 17-ketosteroids and normal levels of plasma 11-oxysteroids have been reported by Lemon et al in their study of patients with asthma. 8 Recently Vaccarezza has reported low 17-ketosteroids and normal 17-hydroxysteroids in asthmatic patients. 9 Normal urinary 17-hydroxysteroids and 17-ketosteroids in patients with asthma have been reported by others. 10,11 Siegel et al 12 and Vaccarezza 9 found normal plasma Cortisol levels in asthmatic patients. Evaluation of

Adrenocortical, anterior pituitary and gonadal activity in an extremely obese male

It has been suggested that excretion levels of 17-ketosteroids and 17-hydroxyeorticosteroids are related to body weight. Adrenal overactivity as measured by 17-hydroxycorticosteroids has been ascribed to obesity. In this case the urinary 17-hydroxycorticosteriods, 17-ketosteroids, and gonadotropins were estimated in a 525 pounds, young, apparently well male. The mean values of the urinary 17-hydroxycorticosteroids (15.4 mg./24 hr.) indicated some degree of adrenal overactivity, and the mean values for the 17-ketosteroids and gona-dotropins (14.3 mg./24 hr. and 28MU/ 24 hr., respectively) indicated that there was no evidence of hypogonadotropic or hypogonadal function. It is concluded that while there was evidence of some adrenal overactivity as measured by 17-hydroxycorticosteroids, the magnitude of this steroid production did not exceed that of far less obese subjects as reported by others 7 . Thus there appears to be no correspondence between steroid excretion levels and absolute body weight.

Further studies of adrenocortical function in patients with carcinoma of the lung

Investigation of adrenocortical function in patients with progressive bronchogenic carcinoma without Cushing's syndrome has been extended with special reference to (1) disease progression (patient's survival time); (2) cell type of carcinoma; (3) brain metastases; and (4) extra-adrenal cortisol metabolism. The concentrations of 17-hydroxycorticosteroids (17-OHCS) in plasma were usually normal in the patients who survived the longest, greater than thirty weeks; were increased abnormally in patients who survived five to thirty weeks; and were further increased, often markedly, in patients within five weeks of death. The mean urinary excretion of 17-OHCS per gram creatinine was significantly higher than in the control group at all survival periods and increased further as death approached. Within five weeks of death, despite a considerable scatter of values, 42 per cent of the patients showed increased urinary excretion of 17-OHCS per gram creatinine, whereas in patients who survived for more than thirty weeks the incidence was 20 per cent. The abnormally high steroid excretion in the group five weeks before death was found predominantly in patients with oat cell and undifferentiated cell carcinoma; the mean urinary levels of both 17-OHCS and 17-ketosteroids (17-KS) were significantly higher in these patients than in patients with squamous cell and adenocarcinoma. The mean response of 17-OHCS in plasma to ACTH stimulation was essentially normal and did not change as the interval to death shortened, but the response of 17-OHCS in urine diminished, suggesting a decline in adrenocortical reserve. Nine of forty-two patients demonstrated hyperresponsiveness to ACTH administration, as reflected in 17-OHCS in plasma and/or urine. An increased incidence of elevated steroid levels was found in patients believed to have brain metastases but, in general, it was probably related to the advanced stage of the disease rather than to specific brain involvement. In selective cases, however, brain metastases may have influenced adrenocortical hyperfunction. Although the increased urinary excretion of 17-OHCS suggests augmented adrenal cortisol secretion, there was in addition considerable evidence for altered extra-adrenal cortisol metabolism in patients as the carcinoma progressed, as follows: High levels of 17-OHCS in plasma were found often in conjunction with normal urinary excretion of 17-OHCS. Urinary output of unconjugated 17-OHCS increased proportionately more than total 17-OHCS in patients with the most advanced disease resulting in a higher ratio of unconjugated to total 17-OHCS. There was a proportionately slightly higher increase in urinary steroid levels of 17-KS and 17-ketogenic versus 17-OHCS following ACTH administration. It is concluded that a spectrum of adrenocortical hyperfunction and altered cortisol metabolism exists in patients with progressively invasive bronchogenic carcinoma as an integral part of the disease. The adrenocortical hyperfunction is most pronounced in patients with advanced oat cell and undifferentiated carcinoma, indicating a connection between the high incidence of Cushing's syndrome reported in these types of patients.

The adrenosympathetic and adrenocortical function in cardiac insufficiency ∗

Levels of epinephrine, norepinephrine, 17-ketosteroids and total 17-hydroxycorticosteroids were determined in the urine, and both free and conjugated 17-hydroxycorticosteroids in the plasma, in 120 patients with cardiac insufficiency of different degrees, according to which they were divided into five groups. On the first hospital day, some patients gave assay values indicative of a distinct but transient adrenal medullary and cortical overactivity. In about one-fourth of our patients the excretion of epinephrine was augmented, especially in patients with insufficiency of medium severity (possibly as a result of hypoxic stress), whereas excretion was lowest in patients with the most severe insufficiency. Excretion of norepinephrine was augmented in only a few isolated instances. On an-average it was within normal limits. Except for a few elevated values, the free 17-hydroxycorticosteroids in the plasma were normal in the morning but approximately twice normal in the evening, indicative of a narrowed diurnal rhythm, especially in the patients with more severe insufficiency. This distribution remained fairly constant during the period of hospital observation. Cojugated 17-hydroxycorticosteroids in the plasma were generally normal with a narrow diurnal rhythm. In patients with most severe insufficiency some elevated morning and evening values were observed. These tended to become normal with treatment. The daily excretion of total 17-hydroxycorticosteroids in the urine was within normal range. 17-Ketosteroid excretion was low in accordance with the advanced age of most of the patients. There was no clear quantitative relationship between the substances assayed and the etiology, symptomatology, urinary volume, effectiveness of treatment and prognosis of the respective cases. Our results permit the assumption of a limited and temporary adrenal medullary over-activity in a minority of instances but they did not reveal any significant augmentation of adrenocortical activity in cardiac failure.

The coexistence of pheochromocytoma and adrenocortical hyperplasia.

ABSTRACT A case of hirsutism, acne, polydipsia, polyuria, weight loss and one known episode of hypertension is reported. The patient was a 22-year-old woman. Laboratoiy investigations revealed glycosuria, hyperglycemia, hypermctabolism, and elevated levels of urinary catechol amines, urinary 17-ketosteroids and plasma 17-hydroxycorticoids. Upon x-ray examination, a left suprarenal mass could be seen. At operation a large benign pheochromocytoma was found in the left adrenal and also marked adrenocortical hyperplasia on the left. Nine months after surgery, the hirsutism and acne were improved. Glucose tolerance, the basal metabolic rate, and the results of the histamine test and the adrenocortical function studies were all entirely normal. Since the patient did not require cortisone therapy for a significant period of time postoperatively, and since unilateral adrenalectomy had been curative, it was inferred that the contralateral adrenal cortex at the time of operation was neither atrophic nor affected by...

Cushing's syndrome with multinodular adrenal glands.

A case of Cushing's syndrome in a 15-year-old girl with symptoms of one year's duration, is described. The levels of plasma and urinary 17-hydroxycorticostcroids and urinary 17-ketogenic steroids were elevated but the levels of urinary 17-ketosteroids and pregnanetriol were normal. Stimulation with ACTH produced a low, sluggish response in the concentration of plasma 17-hydroxycorticosteroids and only minimal changes in urinary steroids. Administration of 9α-fluorohydrocortisone produced no change in urinary steroid excretion. Following bilateral adrenalectomy the manifestations of Cushing's syndrome disappeared. The adrenals showed diffuse multinodularity of the inner cortex. The relation of this lesion to other adrenal lesions producing Cushing's syndrome is discussed. MELLINGER and Smith in 1956 reported an unusual case of Cushing's syndrome of about thirty years' duration in a patient who had multiple small nodules throughout the cortex of one adrenal. Biopsy of the same gland earlier in the course of the disease had not shown this lesion (1). The contralateral gland was not atrophic and did not show the nodular change. The nodular adrenal pattern was believed to be peculiar to very long-standing Cushing's syndrome.

Studies on adrenal cortical activity in patients with rheumatoid arthritis: the diurnal pattern and twenty-four hour levels of urinary total 17-hydroxycorticosteroids and 17-ketosteroids.

The total and diurnal patterns of excretion of 17-hydroxycorticosteroids and 17-ketosteroids were studied in both ambulatory and hospitalized rheumatoid patients and in comparable controls. While daily excretion of 17-ketosteroids was within normal limits, the excretion of 17-hydroxycorticosteroids was diminished, due to a lower output during the early morning hours. Le totales e le curvas diurne del excretion de 17-hydroxycorticosteroids e 17-cetosteroides esseva studiate in patientes rheumatoide-ambulatori e hospitalisate-e in comparabile subjectos de controlo. Durante que le excretion diurne de 17-cetosteroides se teneva intra le limites normal, le excretion de 17-hydroxycorticosteroides esseva reducite in consequentia de un plus basse rendimento durante le prime horas matinal.

Levels of 17-hydroxycorticosteroids and 17-ketosteroids in maternal and cord plasma in term anencephaly.

In plasma samples obtained from blood drawn simultaneously from the mother and the umbilical cord in 5 cases of term anencephalic infants the concentrations of 17-OH-CS were similar to those observed in normal infants, the cord levels being approximately half to a fourth the levels in the corresponding mother. Since the anencephalic infant has an atrophic adrenal devoid of fetal zone, it is concluded that 17-OH-CS in the normal infant are not produced by the fetal zone of the adrenal cortex. In 1 case of anencephaly, no 17-KS were detected in the plasma of either the mother or the umbilical cord. In another case the levels were lower than normal. A case of anencephaly is presented with a “normal” pharyngeal pituitary and with the usual atrophic adrenals, further suggesting that the fetal zone is not directly under the influence of the pituitary gland or that the pituitary is not secreting hormone.

Effect of corticotrophin on the plasma 17-hydroxycorticosteroids in normal and thiouracil treated guinea pigs.

The presence of adrenal-thyroid interaction was suggested by Hoskins, who in 1910 reported hypertrophy of the adrenal glands of guinea pigs treated with thyroid extract. Since this date numerous reports concerned with the effect of thyroid hormones on the adrenal glands have appeared, but the mechanism of interaction has not been explained. Adrenal atrophy following thyroidectomy (Evans et al., 1939) or thiouracil treatment (Baumann & Marine, 1954; Deane & Greep, 1947; Freedman & Gordon, 1950; Zarrow et al., 1957) has been demonstrated in various animals. The depression of adrenal function was also noted in human subjects after thyroidectomy, and in the presence of primary myxoedema (Statland & Lehrman, 1950). The clinical and experimental data concerned with the secretion of adrenal steroids in hypothyroidism is, however, limited. Some authors observed low levels of urinary 17-ketosteroids in a small number of myxoedematous patients (Friedgood, 1942; Talbot et al., 1943). Diminished urinary 17-ketosteroids

Urinary Steroid Excretion after Total Adrenalectomy: II. Levels of 17-Ketosteroids and Formaldehy-Dogenic Substances in Patients on Varying Dosages of Cortisone Acetate and Glycyrrhizin

No AccessJournal of Urology1 Dec 1957Urinary Steroid Excretion after Total Adrenalectomy: II. Levels of 17-Ketosteroids and Formaldehy-Dogenic Substances in Patients on Varying Dosages of Cortisone Acetate and Glycyrrhizin Perry B. Hudson, Bertha P. Boutis, andA.B. Michael E. LombardoPh.D. Perry B. HudsonPerry B. Hudson More articles by this author , Bertha P. BoutisBertha P. Boutis More articles by this author , and Michael E. LombardoMichael E. Lombardo More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(17)66506-6AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail © 1957 by The American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 78Issue 6December 1957Page: 811-816 Advertisement Copyright & Permissions© 1957 by The American Urological Association Education and Research, Inc.MetricsAuthor Information Perry B. Hudson More articles by this author Bertha P. Boutis More articles by this author Michael E. Lombardo More articles by this author Expand All Advertisement PDF downloadLoading ...

17-Ketosteroid and pituitary follicle-stimulating hormone excretion in myxedema, before and during treatment with thyroxine.

IN PATIENTS with myxedema, a decreased urinary excretion of 17-ketosteroids has been a uniform finding (1–4). Values under 3 mg. per day have been observed in women and under 7 mg. in men. The administration of desiccated thyroid to myxedematous patients has been reported to be ineffectual in restoring the excretion of steroids to normal, even when the clinical response to thyroid, including normalization of the basal metabolic rate and serum cholesterol level, was complete (1, 3, 5). Patients with hypothyroidism but without myxedema apparently have normal levels of urinary 17-ketosteroids (1). Several observers have found that in hyperthyroid persons the decrease in 17-ketosteroid excretion is not striking, the values being usually in the subnormal or low-normal range (1–4, 6). Satisfactory treatment of the hyperthyroidism is accompanied by a rise to normal of the 17-ketosteroids (1). No significant correlation has been noted between the clinical data, such as severity and duration of the thyrotoxic stat...

Urinary steroid excretion after total adrenalectomy. I. Levels of 17-ketosteroids in cancer patients maintained on varying amounts of cortisone acetate and glycyrrhizin.

PROMPTED by the reports of Groen (1, 2) concerning successful use of glycyrrhizinic acid for maintenance of a patient suffering from Addison's disease, we have recently investigated the feasibility of using the same compound as replacement medication in cases of total bilateral adrenalectomy. Glycyrrhizin is, from the standpoint of structural formulae, but distantly related to the adrenocortical steroids. Nevertheless, it has been shown (3) to influence many of those metabolic phenomena previously considered to be under the control of the adrenal cortex. Therefore, it has seemed reasonable to investigate the possible in vivo conversion of glycyrrhizin to biologically active steroids. For this purpose, patients maintained on decreasing daily dosages of cortisone acetate have been studied. Two females with advanced disseminated carcinoma of the breast and 1 male with metastatic prostatic carcinoma were utilized for this study. Both of the females were postmenopausal, and the male had previously been castrat...