scholarly journals A Case of Congenital Adrenal Hyperplasia Mimicking Cushing's Syndrome

2012 ◽  
Vol 27 (11) ◽  
pp. 1439 ◽  
Author(s):  
Hye Jeong Kim ◽  
Mira Kang ◽  
Jae Hyeon Kim ◽  
Sun Wook Kim ◽  
Jae Hoon Chung ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia

QUANTITATIVE ESTIMATION OF URINARY PREGNANETRIOL, PREGNANETRIOLONE, TETRAHYDRO S AND Δ5-PREGNENETRIOL IN THE INVESTIGATION OF ADRENOCORTICAL FUNCTION

1969 ◽  
Vol 60 (4) ◽  
pp. 657-668 ◽  
Author(s):  
Frances J. Thomas ◽  
A. W. Steinbeck
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Adrenocortical Carcinoma ◽  
Quantitative Estimation ◽  
Cushing's Syndrome ◽  
Adrenocortical Function ◽  
Adrenal Hyperplasia ◽  
Ectopic Acth ◽  
Modified Method ◽  
Idiopathic Hirsutism

ABSTRACT A modified method for the estimation of urinary pregnanetriol, pregnanetriol, pregnanetriolone, Δ5-pregnenetriol and tetrahydro S was investigated. The steroids, separated by chromatography, were measured quantitatively, tetrahydro S by reaction with blue tetrazolium and the other three as acetaldehydogenic substances. The excretion of these steroids was studied in suspected instances of adrenal and/or ovarian disease. Urinary pregnanetriol and tetrahydro S levels were normal in Cushing's syndrome, secondary adrenocortical carcinoma, the Stein-Leventhal syndrome, idiopathic hirsutism and hypertension. Tetrahydro S was doubtfully elevated in an instance of ectopic ACTH Cushing's syndrome. Pregnanetriol excretion was elevated in untreated cases of congenital adrenal hyperplasia and after treatment in some. Pregnanetriolone was found in all patients with congenital adrenal hyperplasia, including those receiving cortisone. Pregnanetriolone was also detected in Cushing's syndrome, secondary adrenocortical carcinoma, the Stein-Leventhal syndrome, ectopic ACTH Cushing's syndrome and suspected congenital adrenal hyperplasia. Δ5-pregnenetriol was present as a glucuronide in the Stein-Leventhal syndrome and idiopathic hirsutism. The diagnostic implications of these results are discussed.

SEMIQUANTITATIVE ESTIMATION OF URINARY PREGNANETRIOL, PREGNANETRIOLONE AND TETRAHYDRO S IN THE INVESTIGATION OF ADRENOCORTICAL FUNCTION

1969 ◽  
Vol 60 (4) ◽  
pp. 645-656 ◽  
Author(s):  
Frances J. Thomas ◽  
A. W. Steinbeck
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Adrenocortical Function ◽  
Endocrine Disorders ◽  
Adrenal Carcinoma ◽  
Adrenal Hyperplasia ◽  
Validity And Reliability ◽  
Diagnostic Significance

ABSTRACT Following β-glucuronidase hydrolysis, pregnanetriol, pregnanetriolone and tetrahydro S were extracted from urine, chromatographed on florosil and in two paper systems before semiquantitative estimation of pregnanetriol and pregnanetriolone with the phosphoric acid reaction and of tetrahydro S with blue tetrazolium. The chemical validity and reliability of the method were studied and excretions measured in normal subjects and endocrine disorders. Pregnanetriol excretions were compared with published values. Normal pregnanetriol excretions were found in »idiopathic« hirsutism, with Stein-Leventhal ovaries and in Cushing's syndrome due to adrenal adenoma and hyperplasia; increased excretions were found in congenital adrenal hyperplasia and Cushing's syndrome with adrenal carcinoma. Pregnanetriolone was detected only in congenital adrenal hyperplasia, Cushing's syndrome from hyperplasia and one carcinoma. Large amounts of tetrahydro S were found in Cushing's syndrome with adrenal carcinoma. The possible diagnostic significance of the findings is discussed.

Panel Discussion

PEDIATRICS ◽  
1949 ◽  
Vol 3 (4) ◽  
pp. 515-548
Author(s):  
A. WILMOT JACOBSEN ◽  
GEORGE F. KOEPF ◽  
NATHAN B. TALBOT ◽  
LAWSON WILKINS
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Panel Discussion ◽  
Cushing's Syndrome ◽  
Urogenital Sinus ◽  
Adrenal Tumors ◽  
Postnatal Life ◽  
Adrenal Hyperplasia ◽  
Adrenogenital Syndrome ◽  
Acute And Chronic Stress

The role of the adrenal cortex in resistance to stress and strain has been reviewed. Certain experimental data indicating increased secretion of the adrenal cortical hormone in acute and chronic stress has been presented and discussed in terms of Sayers' homeostatic theory of the acute stress mechanism and in terms of Selye's "Adaptation Syndrome." In addition, studies made on a patient with acute rheumatic fever were discussed. Changes in uric acid metabolism compatible with those that might be seen in an alarm reaction were noted in this patient. [SEE THE TABLE I IN SOURCE PDF]. In the adrenogenital syndrome there is increased protein anabolism due to excessive androgen, in Cushing's syndrome inhibition of protein anabolism due to excessive gluconeogenetic hormones. The manifestations of the adrenogenital syndrome differ according to the patient's sex and the age of onset. Congenital adrenal hyperplasia causes pseudohermaphroditism in the female. This can be differentiated from various types of genetic intersexuality by the evidences of excessive androgen from infancy on and by a fairly uniform type of heterosexual development with a persistent urogenital sinus. In the male congenital adrenal hyperplasia causes macrogenitosomia precox which is sometimes accompanied by Addisonian-like symptoms. This can be differentiated from other types of male precocity by the excessive excretion of androgen and lack of maturation of the testes. In postnatal life androgenic adrenal tumors or hyperplasia cause virilization in both sexes unaccompanied by embryonic abnormalities. Occasionally gynecomastia occurs in males. Most of the symptoms of Cushing's syndrome are probably caused by excess of the gluconeogenetic hormones. However, there is also usually an increased secretion of androgen and in some cases evidences of increased electrolyte-controlling hormones.

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