The Molecular and Cellular Etiology of ACTH-Independent Adrenal Disease
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.
United States: Federal Government
|National Institute of Child Health and Human Development (NICHD)||Bethesda, Maryland 20892|