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.

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.

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.

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.

scholarly journals Are Ectopic or Abnormal Membrane Hormone Receptors Frequently Present in Adrenal Cushing’s Syndrome?1

2000 ◽  
Vol 85 (10) ◽  
pp. 3531-3536 ◽  
Author(s):  
Hortensia Mircescu ◽  
Jose Jilwan ◽  
Nina N′Diaye ◽  
Isabelle Bourdeau ◽  
Johanne Tremblay ◽  
...  
Keyword(s):  
Medical Therapy ◽  
Cushing’S Syndrome ◽  
Hormone Receptors ◽  
Gastric Inhibitory Polypeptide ◽  
Cushing's Syndrome ◽  
Adrenergic Agonists ◽  
Adrenal Hyperplasia ◽  
Clinical Screening ◽  
Adrenal Hormone

Twenty consecutive patients with adrenal Cushing’s syndrome were studied with an in vivo protocol to determine the prevalence and diversity of the presence of ectopic or abnormal hormone receptors in their adrenal tissues. All six patients with bilateral ACTH-independent macronodular adrenal hyperplasia were found to have one or two abnormal adrenal receptors, including those for gastric inhibitory polypeptide, vasopressin (V1-vasopressin),β -adrenergic agonists, LH/human CG, or serotonin 5-HT4. The presence of abnormal hormone receptors was found to be less frequently present in unilateral adenomas or carcinomas (3 of 14). The identification of abnormal adrenal hormone receptors can allow new pharmacological therapies of hypercortisolism. We suggest that the clinical screening for the presence of abnormal hormone receptors should be conducted in patients with adrenal Cushing’s syndrome and, more particularly, in those with ACTH-independent macronodular adrenal hyperplasia, in the hope of offering medical therapy as an alternative to bilateral adrenalectomy.

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.

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.

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