17 Α-Hydroxyprogesterone AND 21-Desoxyhydrocortisone; THEIR METABOLISM AND POSSIBLE ROLE IN CONGENITAL ADRENAL VIRILISM 1

Jailer, Joseph W.; Gold, Jay J.; Wiele, Raymond Vande; Lieberman, Seymour

doi:10.1172/jci103217

Cited by 81 publications

(21 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The quantity of the individual 11-oxygenated 17-ketosteroids could not be exactly determined since 11 beta-hydroxyandrosterone was not chromatographically separated from 11 -ketoetiocholanolone. DISCUSSION The urinary 17-ketosteroid pattern observed in these patients with Cushing's syndrome and bilateral adrenal cortical hyperplasia is a characteristic one, and differs from the ketosteroid pattern found in normal subjects and from that reported in other types of adrenal cortical disease (16,30,32). But the abnormal 17-ketosteroid pattern in Cushing's syndrome with adrenal hyperplasia derives its chief interest from its possible bearing upon the problem of deranged adrenal cortical secretion, and further, from its possible bearing upon the pathogenesis of the disorder.…”

contrasting

confidence: 55%

“…One precursor which might be supposed to account for the altered ratio of etiocholanolone to androsterone is the C21 1 1-deoxy 17-hydroxysteroid, Reichstein's Substance S. Administration of this steroid to human subjects has been found to result in the urinary excretion of greater quantities of etiocholanolone than of androsterone (28,29). Another 1 1-deoxy 17-hydroxy steroid of the C21 series, 17 alpha-hydroxyprogesterone, administration of which has been shown to give rise to a high etiocholanolone: androsterone ratio, does not appear to be a likely precursor because its principal excretory products, pregnanetriol and 17 alpha-hydroxypregnanolone, are not increased in this form of Cushing's syndrome (30). In a few of these patients, pregnanetriol was found in amounts not exceeding normal.…”

mentioning

confidence: 89%

See 1 more Smart Citation

Studies in Cushing's Syndrome. Iii. Urinary 17-Ketosteroids in Patients With Bilateral Adrenal Cortical Hyperplasia*

Jailer¹,

Wiele²,

Christy³

et al. 1959

J. Clin. Invest.

Self Cite

View full text Add to dashboard Cite

contrasting

confidence: 55%

mentioning

confidence: 89%

Studies in Cushing's Syndrome. Iii. Urinary 17-Ketosteroids in Patients With Bilateral Adrenal Cortical Hyperplasia*

Jailer¹,

Wiele²,

Christy³

et al. 1959

J. Clin. Invest.

Self Cite

View full text Add to dashboard Cite

“…They suggest that the salt loss is related to the severity of the enzymatic defect. Since both hydrocortisone and aldosterone require hydroxylation of 21-desoxyhydrocortisone at C21, and since Bongiovanni and Eberlein (19), and Jailer, Gold, Vande Wiele and Lieberman (20) have shown that patients with virilizing adrenal hyperplasia, except those with the hypertensive type, have varying defects of hydroxylation at C21 it might be expected that the "salt losers" would be unable to excrete normal quantities of aldosterone. Our data would suggest that such is the case.…”

Section: Results and Commentsmentioning

confidence: 99%

Aldosterone Excretion in Virilizing Adrenal Hyperplasia *

Blizzard¹,

Liddle²,

Migeon³

et al. 1959

J. Clin. Invest.

View full text Add to dashboard Cite

Sodium depletion with concomitant dehydration occurs as a complicating factor in approximately one-third of patients with virilizing adrenal hyperplasia (1). A number of writers have discussed certain differences between the electrolyte disturbance occurring in this syndrome and that of Addison's disease (2-5) and several hypotheses have been offered to explain the sodium loss and potassium retention which occur (1, 4, 6, 7). Although the role of aldosterone is pertinent in many of these hypotheses, the only reported study of aldosterone excretion in patients with virilizing adrenal hyperplasia is that of Prader, Spahr and Neher (6).In order further to elucidate aldosterone metabolism in this syndrome the present studies were undertaken utilizing subjects both with and without defects of sodium regulation. Accumulated data indicate that patients with virilizing adrenal hyperplasia without associated salt loss excrete normal, or high levels of aldosterone on an ad lib. salt intake, and respond with significant increases in aldosterone excretion when placed on limited sodium diets. In contrast, patients with salt loss probably excrete deficient quantities of aldosterone as compared to the normal individual, and are unable to respond with significant increases in aldosterone excretion when sodium intake is limited. Ancillary studies compare the ability of normal infants and adults to excrete aldosterone, both with ingestion of a normal and a sodium deficient diet. day specimens from infants were pooled. In preparation for assay the urine was acidified to pH 1.0 and allowed to stand at room temperature for 24 hours. The "free" steroids were then extracted with dichloromethane. The extracts were washed with aqueous sodium carbonate, dehydrated with sodium sulfate, and evaporated to dryness under a stream of nitrogen. The residues were redissolved in ethanol. These crude extracts were either: 1) injected into adrenalectomized dogs for assays of aldosterone content by the method of Liddle, Cornfield, Casper and Bartter (8) ; 2) subjected to multiple systems of paper chromatography following which the aldosterone fraction was assayed by the same method; or 3) fractionated and assayed for aldosterone content by the isotope-derivative method of Kliman and Peterson (9). The three methods yielded comparable results when run in parallel on the same urine specimens (Table I). Assays carried out only in adrenalectomized dogs were subject to the limitation of relative lack of sensitivity of this method, i.e., a minimum dose of 1 lAg. of aldosterone (per assay animal) was required to induce a distinct response. As a rule an attempt was made to assay each specimen in at least three or four animals. As a result, unless a specimen contained a total of at least 3 or 4 ,ug. of aldosterone, the responses would be negligible. Under such circumstances it was possible to say only that the amount of aldosterone was "less than" a certain value. In such instances (indicated in the tables and graphs by the symbol < or J) the specimen might h...

show abstract

“…Hydroxy-MT, for example, is three times as potent as MT as a myotrophic agent. This is very interesting when considered in connection with the observation of Jailer et at (1955) that the somatic development of congenital adrenal virilism patients is frequently advanced beyond the development of body hirsutism ordinarily associated with testosterone treatment, and that the urine of such patients is rich in 11-oxygenated 17-ketosteroids.…”

Section: Methodsmentioning

confidence: 99%

ANDROGENIC AND MYOTROPHIC PROPERTIES OF ORALLY ADMINISTERED 9-FLUORO-ll-OXY-METHYLTESTOSTERONES

1956

View full text Add to dashboard Cite

17 Α-Hydroxyprogesterone AND 21-Desoxyhydrocortisone; THEIR METABOLISM AND POSSIBLE ROLE IN CONGENITAL ADRENAL VIRILISM 1

Cited by 81 publications

References 23 publications

Studies in Cushing's Syndrome. Iii. Urinary 17-Ketosteroids in Patients With Bilateral Adrenal Cortical Hyperplasia*

Studies in Cushing's Syndrome. Iii. Urinary 17-Ketosteroids in Patients With Bilateral Adrenal Cortical Hyperplasia*

Aldosterone Excretion in Virilizing Adrenal Hyperplasia *

ANDROGENIC AND MYOTROPHIC PROPERTIES OF ORALLY ADMINISTERED 9-FLUORO-ll-OXY-METHYLTESTOSTERONES

Contact Info

Product

Resources

About