DIAGNOSIS OF CUSHING'S SYNDROME

1969 ◽  
Vol 60 (1) ◽  
pp. 47-59 ◽  
Author(s):  
H. Bethge ◽  
M. Bayer ◽  
W. Winkelmann
Keyword(s):  
Cushing’S Syndrome ◽  
Opposite Effect ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Adrenal System ◽  
Lysine Vasopressin ◽  
Normal Increase ◽  
High Degree

ABSTRACT Adrenal function tests were performed in 9 patients with Cushing's syndrome (6 with adrenal hyperplasia and 3 with adrenal adenoma) in order to study the hypothalamus-pituitary-adrenal system. These tests involved the determination of a) diurnal rhythm of plasma corticosteroids (11-OHCS) and the effect of corticotrophin (ACTH) and dexamethasone administration, b) urinary corticosteroid excretion (17-OHCS), basal and after ACTH, dexamethasone and metapyrapone treatment and c) cortisol production rate. In addition the patients were submitted to insulin-induced hypoglycaemia and intravenous infusion of synthetic lysine-vasopressin and the plasma corticosteroids were determined. All 9 patients regardless of the nature of adrenal pathology responded to insulin-induced hypoglycaemia in the same manner and failed to show the normal increase of 11-OHCS. During lysine-vasopressin infusion an opposite effect was observed: all 6 patients with hyperplasia showed a distinct rise in plasma corticosteroids in contrast to the 3 patients with adenoma who did not respond at all. These findings show that the lysine-vasopressin test may be very useful in differentiating between adrenal hyperplasia and adrenal adenoma. This procedure is recommended as a screening test because of its simplicity and high degree of diagnostic accuracy. Results are discussed with regard to the different pathogenesis of the two forms of Cushing's syndrome.

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 Cushing syndrome secondary to late onset adrenal hyperplasia: presentation and challenges of management

2018 ◽  
Vol 1 (1) ◽  
Author(s):  
Muzzammil Abdullahi ◽  
Mamuda Atiku ◽  
Imam Mohammed Ibrahim
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Late Onset ◽  
Adrenal Adenoma ◽  
Protein Structures ◽  
Cushing's Syndrome ◽  
Response To Treatment ◽  
Adrenal Tumors ◽  
Adrenal Hyperplasia ◽  
Supraclavicular Fossa

Cushing’s syndrome is a clinical disorder caused by overproduction of cortisol. Adrenal adenoma is the cause in 5% of cases of Cushing syndrome. ACTH-independent Cushing’s syndrome in 90% is caused by unilateral adrenal tumors. Of these, adenomas are the cause in 80% of the cases, while the others are adrenocortical carcinoma. Rare causes of Cushing’s syndrome include adrenal hyperplasia. Overproduction of cortisol results in weakened protein structures leading to protuberant abdomen and poor wound healing, glucose is converted to fat and deposited in the abdomen, supraclavicular fossa and cheeks. Other presentations are diabetes, hypertension, osteoporosis, fractures, impaired immune function, glucose intolerance, and psychosis. We report a 21-year-old female who presented with clinical symptoms of newly diagnosed hypertension and diabetes mellitus which after poor response to treatment she was evaluated and diagnosed to have Cushing syndrome. She subsequently had left adrenalectomy and did very well with resolution of patients’ symptoms. The histology came out to be diffuse adrenal hyperplasia.

scholarly journals Food-Dependent Cushing’s Syndrome: Possible Involvement of Leptin in Cortisol Hypersecretion*

1999 ◽  
Vol 84 (10) ◽  
pp. 3817-3822 ◽  
Author(s):  
François P. Pralong ◽  
Fulgencio Gomez ◽  
Louis Guillou ◽  
François Mosimann ◽  
Sebastiano Franscella ◽  
...  
Keyword(s):  
Food Intake ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Cortisol Secretion ◽  
Adrenal Hyperplasia ◽  
Adrenal Cells ◽  
Patient Reported ◽  
Stimulation Of

Abstract Stimulation of cortisol secretion by food intake has been implicated in the pathogenesis of some cases of ACTH-independent Cushing’s syndrome, via an aberrant response of the adrenal glands to gastric inhibitory polypeptide (GIP). We report here a novel case of food-dependent Cushing’s syndrome in a patient with bilateral macronodular adrenal hyperplasia. In this patient we were able to confirm a paradoxical stimulation of cortisol secretion by GIP in vivo as well as in vitro on dispersed tumor adrenal cells obtained at surgery. In addition to GIP, in vitro stimulation of these cultured tumor adrenal cells with leptin, the secreted product of the adipocyte, induced cortisol secretion. By comparison, no such stimulation was observed in vitro in adrenal cells obtained from another patient with bilateral macronodular adrenal hyperplasia and Cushing’s syndrome that did not depend on food intake, in tumor cells obtained from a solitary cortisol-secreting adrenal adenoma, and in normal human adrenocortical cells. These results demonstrate that as in previously described cases of food-dependent Cushing’s syndrome, GIP stimulated cortisol secretion from the adrenals of the patient reported here. Therefore, they indicate that such a paradoxical response probably represents the hallmark of this rare condition. In addition, they suggest that leptin, which normally inhibits stimulated cortisol secretion in humans, participated in cortisol hypersecretion in this case. Further studies in other cases of food-dependent Cushing’s syndrome, however, will be necessary to better ascertain the pathophysiological significance of this finding.

COMPARISON OF EFFECTS OF 6-DEHYDRO-16-METHYLENE-HYDROCORTISONE (STC 407) AND DEXAMETHASONE ON THE SUPPRESSION OF THE HYPOTHALAMO-PITUITARY-ADRENAL SYSTEM

1970 ◽  
Vol 63 (3) ◽  
pp. 423-430 ◽  
Author(s):  
K. Berthold ◽  
A. Arimura ◽  
A. V. Schally
Keyword(s):  
Cushing’S Syndrome ◽  
Successful Treatment ◽  
Cushing's Syndrome ◽  
Regulatory Function ◽  
Adrenal Hyperplasia ◽  
Adrenal System ◽  
Pituitary Glands ◽  
Adrenal Corticosterone

ABSTRACT The suppressive effects of 6-dehydro-16-methylene-hydrocortisone (STC 407) and dexamethasone on the hypothalamo-pituitary-adrenal system were evaluated in the rat. Four μg dexamethasone or 120 μg STC 407/100 g b. w. was injected ip daily into normal rats for 16 or 31 days. These doses were of approximately equivalent antiinflammatory potencies. Both steroids decreased equally plasma and adrenal corticosterone concentrations as well as adrenal weights. However, CRF activities of the hypothalamus and ACTH contents of the pituitary glands were significantly reduced by treatment with STC 407, but not with dexamethasone. This finding may endorse the successful treatment with STC 407 of Cushing's syndrome due to adrenal hyperplasia in which the fundamental derangement appears to consist of a disturbed regulatory function of the hypothalamus.

scholarly journals Asynchronous Development of Bilateral Nodular Adrenal Hyperplasia in Gastric Inhibitory Polypeptide-Dependent Cushing’s Syndrome1

1999 ◽  
Vol 84 (8) ◽  
pp. 2616-2622 ◽  
Author(s):  
Nina N’Diaye ◽  
Pavel Hamet ◽  
Johanne Tremblay ◽  
Jean-Marie Boutin ◽  
Louis Gaboury ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Adrenal Adenoma ◽  
Gastric Inhibitory Polypeptide ◽  
Cushing's Syndrome ◽  
Early Event ◽  
Oral Glucose ◽  
Adrenal Hyperplasia ◽  
Messenger Ribonucleic Acid ◽  
The Right

Gastric inhibitory polypeptide (GIP)-dependent Cushing’s syndrome has been reported to occur either in unilateral adrenal adenoma or in bilateral macronodular adrenal hyperplasia. A 33-yr-old woman with Cushing’s syndrome was found to have two 2.5- to 3-cm nodules in the right adrenal on computed tomography scan; the left adrenal appeared normal except for the presence of a small 0.8 × 0.6-cm nodule. Uptake of iodocholesterol was limited to the right adrenal. Plasma morning cortisol was 279 nmol/L fasting and 991 nmol/L postprandially, and ACTH remained suppressed. Plasma cortisol increased after oral glucose (202%) or a lipid-rich meal (183%), but not after a protein-rich meal (95%) or iv glucose (93%); the response to oral glucose was blunted by pretreatment with 100 μg octreotide, sc. Plasma cortisol and GIP levels were positively correlated (r = 0.95; P = 0.0001); cortisol was stimulated by the administration of human GIP iv (225%), but not by GLP-1, insulin, TRH, GnRH, glucagon, arginine vasopressin, upright posture, or cisapride orally. A right adrenalectomy was performed; GIP receptor messenger ribonucleic acid was overexpressed in both adrenal nodules and in the adjacent cortex. Histopathology revealed diffuse macronodular adrenal hyperplasia without internodular atrophy. Three months after surgery, fasting plasma ACTH and cortisol were suppressed, but cortisol increased 3.6-fold after oral glucose, whereas ACTH remained suppressed; this was inhibited by octreotide pretreatment, suggesting that cortisol secretion by the left adrenal is also GIP dependent. We conclude that GIP-dependent nodular hyperplasia can progress in an asynchronous manner and that GIPR overexpression is an early event in this syndrome.

Serum profiles of steroid hormones in patients with Cushing's syndrome determined by a new HPLC/RIA method

1991 ◽  
Vol 37 (8) ◽  
pp. 1329-1333 ◽  
Author(s):  
H Ueshiba ◽  
M Segawa ◽  
T Hayashi ◽  
Y Miyachi ◽  
M Irie
Keyword(s):  
Steroid Hormones ◽  
Cushing’S Syndrome ◽  
High Performance ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Serum Concentrations ◽  
Adrenal Hyperplasia ◽  
Steroid Profile ◽  
17 Hydroxyprogesterone ◽  
Normal Adults

Abstract We developed a method for simultaneously measuring steroid hormones in very small volumes of serum, using a combination of high-performance liquid chromatography (HPLC) and radioimmunoassay (RIA). By this method, aldosterone, cortisol, 11-deoxycortisol, estrone, estradiol, androstenedione, dehydroepiandrosterone, deoxycorticosterone, 17-hydroxyprogesterone, testosterone, pregnenolone, and progesterone could be determined in a single 100-microL aliquot of serum from normal adults and patients with Cushing's syndrome. The steroid profile associated with Cushing's syndrome caused by adrenal adenoma was quite distinct from that associated with the syndrome caused by adrenal hyperplasia. Serum concentrations of androstenedione, dehydroepiandrosterone, estrone, estradiol, 17-hydroxyprogesterone, pregnenolone, and testosterone were significantly higher in patients with adrenal hyperplasia than in those with an adenoma. We compared the results of this HPLC/RIA method with those of 125I RIAs. The use of a HPLC/RIA system to obtain an accurate and sensitive profile of a range of serum steroids, as described here, obviates the need for large volumes of blood.

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.

GASCHROMATOGRAPHIC DETERMINATION OF STEROIDS IN THE URINE OF PATIENTS WITH CUSHING'S SYNDROME

1971 ◽  
Vol 67 (2) ◽  
pp. 303-315 ◽  
Author(s):  
A. J. Moolenaar ◽  
A. P. van Seters
Keyword(s):  
Liquid Chromatography ◽  
Cushing’S Syndrome ◽  
Normal Subjects ◽  
Diagnostic Value ◽  
Cushing's Syndrome ◽  
Gas Liquid Chromatography ◽  
Obese Subjects

ABSTRACT The 17-oxosteroids were estimated in the urine of 27 patients with Cushing's syndrome by gas-liquid chromatography (G. L. C.). The values of the various steroid fractions are compared with those of normal subjects, patients with thyrotoxicosis and obese subjects. The effect of the age of the patients on the diagnostic value of the invidual 17-oxosteroids and their ratios is discussed.

SIMPLIFIED DEXAMETHASONE SUPPRESSION TEST

1969 ◽  
Vol 61 (2) ◽  
pp. 219-231 ◽  
Author(s):  
V. H. Asfeldt
Keyword(s):  
Cushing’S Syndrome ◽  
Normal Subjects ◽  
Cushing's Syndrome ◽  
Dexamethasone Suppression Test ◽  
Clinical Value ◽  
Suppression Test ◽  
The One ◽  
Steroid Excretion ◽  
Dexamethasone Suppression

ABSTRACT This is an investigation of the practical clinical value of the one mg dexamethasone suppression test of Nugent et al. (1963). The results, evaluated from the decrease in fluorimetrically determined plasma corticosteroids in normal subjects, as well as in cases of exogenous obesity, hirsutism and in Cushing's syndrome, confirm the findings reported in previous studies. Plasma corticosteroid reduction after one mg of dexamethasone in cases of stable diabetes was not significantly different from that observed in control subjects, but in one third of the insulin-treated diabetics only a partial response was observed, indicating a slight hypercorticism in these patients. An insufficient decrease in plasma corticosteroids was observed in certain other conditions (anorexia nervosa, pituitary adenoma, patients receiving contraceptive or anticonvulsive treatment) with no hypercorticism. The physiological significance of these findings is discussed. It is concluded that the test, together with a determination of the basal urinary 17-ketogenic steroid excretion, is suitable as the first diagnostic test in patients in whom Cushing's syndrome is suspected. In cases of insufficient suppression of plasma corticosteroids, further studies, including the suppression test of Liddle (1960), must be carried out.

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