Clinical Trial: Study of Cushing's Syndrome Not Related to ACTH Production
Study Status: Completed
Recruit Status: Completed
Study Type: Observational
Official Title: The Molecular and Cellular Etiology of ACTH-Independent Adrenal Disease
Brief Summary:
This study is designed to provide information about the cause of two unusual types of Cushing's syndrome and to evaluate quality of life before and after cure of the disease. In Cushing's syndrome, the adrenal glands produce too much of the hormone cortisol. This often causes weight gain, skin changes (bruising and stretch marks), and mood changes such as irritability, easy crying and depression. Adrenocorticotrophic hormone (ACTH) normally regulates cortisol production; when cortisol is low, ACTH rises, stimulating the adrenals to produce more cortisol, and when cortisol is high, ACTH levels fall. In two forms of Cushing's syndrome, however, the adrenal glands produce cortisol even when ACTH is low.
Patients 18 years of age and older with Cushing's syndrome not related to ACTH production may participate in this study. Candidates will be have a history and physical examination, electrocardiogram, urine, blood and saliva tests, and a computerized tomography (CT) scan of one or both adrenal glands. They will fill out questionnaires on their disease symptoms, quality of life, and on basic information about themselves, such as marital status, education level, place of residence, etc. Finally, they will have a corticotropin-releasing hormone (CRH) test to confirm that they have the form of Cushing's syndrome under study in this protocol. This test involves collecting blood samples at intervals before and after administration of sheep CRH to measure cortisol and ACTH levels.
Participants will undergo 3 to 7 days of testing to determine if their cortisol level rises after taking certain medicines or eating certain foods. These foods and medicines, chosen to mimic or stimulate substances already in the body, are: glucagon, ACTH, gonadotropin-releasing hormone, vasopressin, thyrotropin-releasing hormone, and a mixed meal consisting
Detailed Summary:
In healthy individuals, ACTH is the major stimulus for cortisol production and cellular growth of the adrenal cortex. Normal or elevated ACTH levels can amplify this stimulus, leading to hypercortisolism and growth of the adrenal gland, a clinical condition recognized as Cushing's syndrome. However, some patients with hypercortisolism of Cushing's syndrome have suppressed rather than normal or high levels of ACTH. This pilot study seeks to better understand the apparently autonomous nature of hypercortisolism in two benign causes of Cushing's syndrome, adrenal adenomas and massive macronodular adrenal disease (MMAD).
Patients with low levels of ACTH and Cushing's syndrome will undergo routine tests to confirm that they have hypercortisolism, low ACTH, and adenoma or MMAD on CT scans. They will fill out a questionnaire about quality of life. They will also undergo 3-7 days of research testing with a variety of agents to investigate whether these agents increase cortisol. Following this, patients will be scheduled for adrenalectomy at the NIH. This is the standard treatment for these conditions. The abnormal tissue obtained at surgery will be used for laboratory studies to evaluate whether the same or other agents may be the cause of excess cortisol production.
After surgery, patients will be discharged to the care of their local health care provider, but will continue to fill out questionnaires on the quality of life for two years.
Sponsor: Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
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Information By: National Institutes of Health Clinical Center (CC)
Dates:
Date Received: September 14, 2000
Date Started: September 2000
Date Completion: September 2002
Last Updated: March 3, 2008
Last Verified: September 2002
