ORAL AND INTRAVENOUS SUPPRESSION TESTS IN THE DIAGNOSIS OF CUSHING'S SYNDROME

1965 ◽  
Vol 33 (3) ◽  
pp. 515-524 ◽  
Author(s):  
V. H. T. JAMES ◽  
J. LANDON ◽  
V. WYNN
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Early Period ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Adrenal Tumour ◽  
Bilateral Adrenal Hyperplasia ◽  
Steroid Excretion ◽  
Synthetic Glucocorticoids ◽  
Urinary Steroid

SUMMARY Adrenocortical suppression tests, based on the fall in urinary 17-hydroxy-corticosteroid excretion during the oral administration of dexamethasone, were found to be of value in the diagnosis of Cushing's syndrome, but less useful in differentiating bilateral adrenal hyperplasia from adrenal tumour. Such tests have the disadvantage of requiring accurate urine collections and of taking several days to perform. A test is described, based on the decrease in plasma cortisol concentration during i.v. infusion of dexamethasone at a rate of 1 mg./hr. The results obtained in 12 patients with Cushing's syndrome and bilateral adrenal hyperplasia differed from those found in control subjects in that there was a delay between the start of the infusion and the fall of plasma cortisol, and the rate of fall was less rapid. The values found after 180 min., expressed either as μg./100 ml. or as a percentage of the resting level, differed significantly (P < 0·001) in the two groups. The test proved valuable as an aid to the diagnosis of Cushing's syndrome, was easy to perform, and could be completed in 3 hr. In some patients with Cushing's syndrome, the administration of synthetic glucocorticoids appeared to result in an increased urinary steroid excretion. A transient increase in plasma cortisol levels was also observed in some of these patients during the early period of dexamethasone infusion. It is thought that this finding reflects an alteration in steroid metabolism induced by dexamethasone and fluorocortisol.

Cushing's syndrome due to unilateral nodular adrenal hyperplasia: a new pathophysiological entity?

1980 ◽  
Vol 93 (4) ◽  
pp. 495-504 ◽  
Author(s):  
Robert G. Josse ◽  
Robert Bear ◽  
Kalman Kovacs ◽  
H. Patrick Higgins
Keyword(s):  
Adrenal Gland ◽  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Electron Microscopic Examination ◽  
Cushing's Syndrome ◽  
High Dose ◽  
Adrenal Hyperplasia ◽  
Electron Microscopic ◽  
High Dose Dexamethasone ◽  
Steroid Excretion

Abstract. A case is presented of a 43-year-old Caucasian female with Cushing's syndrome, malignant hypertension and renal insufficiency. Plasma cortisol values were elevated (19.5 μg/100 ml at 0800 h and 18.5 μg/100 ml at 2200 h) with loss of diurnal variation and failure of suppression with low and high dose dexamethasone. 17-ketogenic steroid excretion remained unchanged after metyrapone administration. Adrenal vein plasma cortisol values showed prominent left-sided gradient. Gross, histologic and electron-microscopic examination of the left adrenal gland revealed changes of nodular adrenal hyperplasia. The presence of a suppressed right adrenal gland was confirmed post-operatively by a five day ACTH infusion. This case may represent a previously unreported variant of Cushing's syndrome due to unilateral nodular adrenal hyperplasia.

STEROID EXCRETION AND PLASMA CORTISOL IN 41 CASES OF CUSHING'S SYNDROME

1966 ◽  
Vol 51 (4) ◽  
pp. 511-525 ◽  
Author(s):  
Ingrid Ernest
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Cushing's Syndrome ◽  
Similar Data ◽  
Control Material ◽  
Present Material ◽  
Control Range ◽  
The Mean ◽  
Obesity Hypertension ◽  
Steroid Excretion

ABSTRACT Urinary excretion of 17-hydroxycorticosteroids (17-OHCS) and of Porter-Silber chromogens as well as plasma cortisol at different times of the day were measured repeatedly in 41 cases of Cushing's syndrome and the results were compared with similar data from 55 non Cushing patients suffering from obesity, hypertension and other symptoms often associated with Cushing's syndrome. The Cushing patients showed a marked variation in steroid output and in many cases the excretion was within the control range during one or several days. In only four of all patients, however, did the mean excretion of 17-OHCS overlap with that found in the control material. There was a marked overlapping between the Cushing and the non Cushing patients with respect to plasma cortisol. At midnight, however, most Cushing patients (34/36) at repeated determinations showed one or several figures higher than those of the control material. In the present material there was a marked overlapping between the Cushing (15 cases) and the non Cushing patients (14 cases) with respect to the increase in steroid excretion after intravenously administered corticotrophin (ACTH). In 14 cases of Cushing's syndrome due to hyperplasia the administration of metapyron caused an increase in steroid excretion. One such case failed to react to metapyron as did 3 cases of cortisol producing tumours.

scholarly journals MON-LB44 Cushing’s Syndrome and Illegal Receptors

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Caroline Poku ◽  
Randa Sharag Eldin ◽  
Abubakr Hassab Elrasoul Babiker Mohamed ◽  
Sadiq Sobia
Keyword(s):  
Cushing’S Syndrome ◽  
Hormone Receptors ◽  
Age Distribution ◽  
Cushing's Syndrome ◽  
Endocrine Surgery ◽  
Adrenal Hyperplasia ◽  
Unilateral Adrenalectomy ◽  
Production Studies ◽  
Bilateral Adrenal Hyperplasia ◽  
Adrenal Masses

Abstract Case presentation: A 48-year-old female with HTN presented to the endocrinology clinic for the evaluation of incidental bilateral adrenal masses noted on chest CT for dyspnea workup. At the time of the presentation, she reported generalized fatigue, significant weight gain in the past year and shortness of breath. Her physical exam was remarkable for central obesity. Lab work showed elevated cortisol after 1 mg dexamethasone suppression test x 2 and elevated 24- hour urine cortisol. Plasma free metanephrine levels and aldosterone/ renin ratio were normal. MRI abdomen was done and showed bilateral adrenal masses (left: 5.6 cm, right: 3.2 cm). Patient was diagnosed with Cushing’s syndrome secondary to primary bilateral adrenal hyperplasia and was referred to endocrine surgery who recommended unilateral adrenalectomy. The decision was made to remove the larger left side adrenal mass. On post-operative day one her am cortisol decreased to 2.1 and she was started on hydrocortisone 20 mg in the morning and 10 mg in the evening. Discussion: Primary bilateral adrenal hyperplasia is a rare cause (&lt; 2 %) of endogenous Cushing’s syndrome, usually occurs in a bimodal age distribution, in childhood and in the fifth- sixth decades. Presentation is variable with most patients having no symptoms or subclinical Cushing’s. The theory is the larger nodule size corelates with the higher cortisol production. Studies have shown between 60-70% of cases has aberrant ectopic hormone receptors which leads to increased cortisol production not only from ACTH but also from other ligands such as serotonin and vasopressin. Aberrant receptor testing examines whether cortisol or other steroid production increases in response to either physiologic or pharmacologic stimulus. Multiple genetic mutations have been associated, the most frequent is mutations in the Armadillo repeat- containing 5 gene identified in 2013. Treatment can either be medical or surgical. Medical therapy can be initiated if testing for an aberrant receptor is positive. In recent years there has been a trend towards doing unilateral adrenalectomy instead of bilateral, with initial remission of symptoms reported in about 84% of cases after unilateral adrenalectomy although there is a small risk of recurrence. Post operatively after unilateral adrenalectomy patients should be monitored for adrenal insufficiency. Our patient declined aberrant receptor testing and opted for surgery and is doing well post operatively. Conclusion: Primary bilateral adrenal hyperplasia is a rare cause of endogenous Cushing’s syndrome which can be treated either medically or surgically.

scholarly journals Aberrant Membrane Hormone Receptors in Incidentally Discovered Bilateral Macronodular Adrenal Hyperplasia with Subclinical Cushing’s Syndrome

2001 ◽  
Vol 86 (11) ◽  
pp. 5534-5540 ◽  
Author(s):  
Isabelle Bourdeau ◽  
Pierre D’Amour ◽  
Pavel Hamet ◽  
Jean-Marie Boutin ◽  
André Lacroix
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Hormone Receptors ◽  
Gastric Inhibitory Polypeptide ◽  
Urinary Free Cortisol ◽  
Cushing's Syndrome ◽  
Cortisol Secretion ◽  
Adrenal Hyperplasia ◽  
Free Cortisol ◽  
Cortisol Levels

Cortisol secretion in adrenal Cushing’s syndrome can be regulated by the aberrant adrenal expression of receptors for gastric inhibitory polypeptide, vasopressin, catecholamines, LH/human CG (LH/hCG), or serotonin. Four patients with incidentally discovered bilateral macronodular adrenal hyperplasia without clinical Cushing’s syndrome were evaluated for the possible presence of aberrant adrenocortical hormone receptors. Urinary free cortisol levels were within normal limits, but plasma cortisol levels were slightly elevated at nighttime and suppressed incompletely after dexamethasone administration. Plasma ACTH was partially suppressed basally but increased after administration of ovine CRH. A 51-yr-old woman had ACTH-independent increases of plasma cortisol after 10 IU AVP im (292%), 100 μg GnRH iv (184%), or 10 mg cisapride orally (310%); cortisol also increased after administration of NaCl (3%), hCG, human LH, and metoclopramide. In a 61-yr-old man, cortisol was increased by AVP (349%), GnRH (155%), hCG (252%), and metoclopramide (191%). Another 53-yr-old male increased plasma cortisol after AVP (171%) and cisapride (142%). Cortisol secretion was also stimulated by vasopressin in a 54-yr-old female. This study demonstrates that subclinical secretion of cortisol can be regulated via the aberrant function of at least V1-vasopressin, LH/hCG, or 5-HT4 receptors in incidentally identified bilateral macronodular adrenal hyperplasia.

A CASE OF CUSHING'S SYNDROME STUDIED BY URINARY CORTICOTROPHIN ASSAY

1963 ◽  
Vol 42 (2) ◽  
pp. 163-167 ◽  
Author(s):  
Masami Nakamura ◽  
Masaji Hirano ◽  
Hiroshi Ibayashi ◽  
Takuo Fujita ◽  
Nakaaki Ohsawa ◽  
...  
Keyword(s):  
Diurnal Variation ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Abnormal Response ◽  
Before And After ◽  
Total Adrenalectomy ◽  
Bilateral Adrenal Hyperplasia ◽  
Distinct Increase ◽  
Daily Total

ABSTRACT A 20 year old female developed typical features of Cushing's syndrome, with increased urinary and plasma 17-OHCS, which showed a distinct diurnal variation, exaggerated response to exogenous ACTH, and suppression of response to 3 mg dexamethasone daily. Total adrenalectomy was performed for bilateral adrenal hyperplasia. ACTH in urine showed a distinct increase before and after operation. More than 30 mg of cortisol was required to suppress the elevated urinary ACTH, suggesting an abnormal response of the pituitary gland to exogenous steroid.

Bilateral adrenal hyperplasia: a rare cause of Cushing's syndrome in chidren

2014 ◽  
Author(s):  
Akshatha Taranath Kamath ◽  
Shiny Goyal Mandliya ◽  
Sanjay Rao ◽  
Sampath Satish Kumar
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Bilateral Adrenal Hyperplasia

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.

CUSHING'S SYNDROME ASSOCIATED WITH A »CORTICOTROPHIN«-PRODUCING BRONCHIAL NEOPLASM

1967 ◽  
Vol 56 (2) ◽  
pp. 321-332 ◽  
Author(s):  
J. Landon ◽  
V. H. T. James ◽  
W. S. Peart
Keyword(s):  
Side Effects ◽  
Cushing’S Syndrome ◽  
Bronchial Carcinoma ◽  
Cushing's Syndrome ◽  
Adrenal Function ◽  
Clinical Usefulness ◽  
Adrenal Hyperplasia ◽  
Melanocyte Stimulating Hormone ◽  
Bilateral Adrenal Hyperplasia ◽  
Chemical Evidence

ABSTRACT A patient is reported who developed bilateral adrenal hyperplasia and Cushing's syndrome in association with an undifferentiated bronchial carcinoma. »Corticotrophin« was demonstrated by radioimmunoassay in the tumour and its metastases and »melanocyte-stimulating hormone« by bioassay in a metastasis. Treatment with Metyrapone resulted in bio-chemical evidence of improvement but side-effects limited its clinical usefulness. The use of pituitary-adrenal function tests in distinguishing patients with this syndrome from those with Cushing's disease is discussed.

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