scholarly journals Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing's Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

2014 ◽  
pp. 739-769 ◽  
Author(s):  
Hershel Raff ◽  
Susmeeta T. Sharma ◽  
Lynnette K. Nieman
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Insufficiency ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Diagnosis And Treatment ◽  
Adrenal Hyperplasia ◽  
Physiological Basis

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.

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

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.

scholarly journals Classification, diagnosis and treatment of ACTH-independent macronodular adrenal hyperplasia

2013 ◽  
Vol 7 (9-10) ◽  
pp. 594 ◽  
Author(s):  
Heng-Chuan Su ◽  
Xin Huang ◽  
Jun Dai ◽  
Wen-long Zhou ◽  
Bao-xing Huang ◽  
...  
Keyword(s):  
High Risk ◽  
Cushing’S Syndrome ◽  
English Literature ◽  
Clinical Manifestations ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Diagnosis And Treatment ◽  
Adrenal Hyperplasia ◽  
Treatment Methods ◽  
Future Therapy

ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a distinctive subtype of Cushing’s syndrome (CS), with different clinical manifestations according to the level of serum cortisol. Based on clinical manifestations and serum cortisol, we divide AIMAH into three types, subclinical AIMAH, clinical AIMAH and high-risk AIMAH, with varied treatment methods being adapted to different subtypes. At the same time, we describe 3 patients who represent these subtypes of this disease, and review some cases of AIMAH which have been previously reported in the English literature. To our knowledge, this is the first article discussing classification, diagnosis and treatment of this disease and should be useful for future therapy of AIMAH.

scholarly journals Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: Long-term follow-up of four cases

2002 ◽  
pp. 237-240 ◽  
Author(s):  
C Lamas ◽  
JJ Alfaro ◽  
T Lucas ◽  
B Lecumberri ◽  
B Barcelo ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Cushing’S Syndrome ◽  
Alternative Treatment ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Unilateral Adrenalectomy ◽  
Free Cortisol

OBJECTIVE: ACTH-independent macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered the treatment of choice, but the patient is obliged to receive lifetime steroid replacement therapy and is susceptible to adrenal insufficiency crisis. New therapeutic alternatives are being proposed as new etiopathological features of the disease are known. Unilateral adrenalectomy of the largest gland can be a safe and effective alternative, but only short-term follow-up is reported in the literature. We present four consecutive patients with ACTH-independent macronodular hyperplasia and long-term remission of Cushing's syndrome after unilateral adrenalectomy. SUBJECTS: Four consecutive patients (two males and two females, mean age 50.3 years) with Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia underwent unilateral adrenalectomy of the largest gland. RESULTS: The weight of the resected glands ranged from 26.8 to 210 g. Two patients suffered transient post-surgical adrenal insufficiency and had steroid replacement therapy for 60 and 14 months respectively. After a mean follow-up of 78.8 months (range 30-137 months) all the patients persist without any evidence of Cushing's syndrome. Urinary free cortisol and serum cortisol, after the adrenal insufficiency stage, have always stayed within their normal ranges, but cortisol circadian rhythm and suppressibility after dexamethasone have never normalized. No further enlargement of the contralateral gland has been documented 62 to 126 months after surgery in three of the four patients. CONCLUSIONS: Unilateral adrenalectomy can be an effective and safe alternative treatment for ACTH-independent macronodular adrenal hyperplasia, and can achieve long-term remission of Cushing's syndrome.

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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