Food-dependent Cushing syndrome: a new entity of organic hypercorticism

Matejka G, et al. Rev Med Interne. 1996.

Abstract

Diagnosis of Cushing’s syndrome is quite difficult in endocrinology. Spontaneous Cushing’s syndrome is usually divided into two subgroups, one which is dependent on corticotropin (ACTH) and another one which is not.

In the first class are Cushing’s disease, the ectopic corticotropin syndrome and the rare ectopic corticotropin-releasing hormone (CRH) syndrome; these ACTH-dependent Cushing’s syndrome have usually diffusely enlarged adrenal glands.

In the second class are cortisol producing unilateral adrenocortical adenomas or carcinomas, and the recent Cushing’s syndrome with food dependent periodic hormonogenesis.

This food dependent Cushing’s syndrome is an ACTH-independent Cushing’s syndrome with multinodular enlargement of both adrenal glands. Pathogenesis is an aberrant adrenal sensitivity to physiologic secretion of gastric inhibitory peptide (GIP). Ectopic expression of GIP receptors on adrenal cells involve pathologic food induced cortisol secretion.

Food dependent Cushing’s syndrome is a new cause of Cushing’s syndrome. Food induced cortisol secretion may have to be explored in the ACTH-independent Cushing’s syndrome.

PMID

8758532 [PubMed – indexed for MEDLINE]

Full text

Full text from provider (Elsevier Science) Article in French.

From http://www.ncbi.nlm.nih.gov/m/pubmed/8758532/

Biography of a Food-Dependent Cushing’s patient

The low-dose dexamethasone suppression test: a reevaluation in patients with Cushing’s syndrome

J Clin Endocrinol Metab. 2004 Mar;89(3):1222-6.

Findling JW1, Raff H, Aron DC.

Abstract

Low-dose dexamethasone suppression testing has been recommended for biochemical screening when Cushing’s syndrome is suspected. The criterion for normal suppression of cortisol after dexamethasone is controversial.

To assess diagnostic utility (sensitivity), we report the results of low-dose dexamethasone suppression testing in 103 patients with spontaneous Cushing’s syndrome. There were 80 patients with Cushing’s disease (78%), 13 with the ectopic ACTH syndrome (13%), and 10 with cortisol-producing adrenocortical adenomas (10%). Fourteen (18%) of 80 patients with Cushing’s disease suppressed serum cortisol to less than 5 micro g/dl (<135 nmol/liter) after the overnight 1-mg test, whereas six patients (8%) actually showed suppression of serum cortisol to less than 2 micro g/dl (<54 nmol/liter). In addition, the 2-d, low-dose dexamethasone suppression test yielded false-negative results in 38% of patients when urine cortisol was used and 28% when urinary 17-hydroxycorticosteroids were used. Serum cortisol after the 1-mg test correlated with baseline urinary free cortisol (r = 0.705, P < 0.001), plasma ACTH level (r = 0.322, P = 0.001), and urinary free cortisol after the 2-d test (r = 0.709, P = 0.001).

This study provides evidence that low-dose dexamethasone may suppress either plasma cortisol or urinary steroids to levels previously thought to exclude Cushing’s syndrome and that these tests should not be used as the sole criterion to exclude the diagnosis of endogenous hypercortisolism.

PMID:
15001614
[PubMed – indexed for MEDLINE]

From http://www.ncbi.nlm.nih.gov/pubmed/15001614

Crooke’s changes common in patients with Cushing’s syndrome, high cortisol production

 

Evidence of Crooke hyaline changes in the pituitary gland points to a higher likelihood of Cushing’s syndrome in adults, with the changes in basophil cells occurring in 75% to 80% of patients with the hormonal disorder, according to research in The Journal of Clinical Endocrinology & Metabolism.

In a retrospective review of hospital patient records from adults with Cushing’s syndrome who underwent pituitary surgery, researchers also found that a higher degree of cortisol production, as well as exposure to excess glucocorticoids, are often associated with Crooke’s changes in adults.

“The presence of Crooke’s changes is a clear indication of the presence of Cushing’s syndrome, although the absence of Crooke’s changes does not exclude it,” the researchers wrote.

Edward H. Oldfield, MD, FACS, of the department of neurological surgery at University of Virginia Health System, and colleagues analyzed electronic hospital data from 213 consecutive patients with Cushing’s syndrome who received pituitary surgery between 2008 and March 2014. Researchers reviewed analysis of the normal pituitary tissue included with the specimens obtained at surgery, as well as cortisol production measured by 24-hour urine.

Within the cohort, Crooke’s changes occurred in 74% of patients; Crooke’s changes occurred in 81% of patients with an adrenocorticotropic hormone tumor.

Researchers also found that 91% of patients with an adrenocorticotropic hormone-producing tumor and a urinary free cortisol test at least fourfold the upper limit of normal had evidence of Crooke’s changes vs. 74% of patients with a urine cortisol amount that was less than fourfold the upper limit of normal (P = .008).

“Our results clearly demonstrate a correlation between the degree of cortisol production and the presence of Crooke’s changes,” the researchers wrote. “Patients with cortisol production exceeding fourfold upper limit almost all had Crooke’s changes.”

Researchers said study results indicate that the presence of Crooke’s changes may be used to indicate that a patient has Cushing’s syndrome following a pituitary surgery in which no tumor is found.

“However, the absence of Crooke’s changes does not reliably indicate the absence of Cushing’s syndrome, as 19% of patients with a proven [adrenocorticotropic hormone-producing tumor] did not have Crooke’s changes,” the researchers wrote. by Regina Schaffer

Disclosure: The researchers report no relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7B838a3557-f284-4fda-b93d-73dbb4823667%7D/crookes-changes-common-in-patients-with-cushings-syndrome-high-cortisol-production

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