Harvey W. Cushing's 1932 description1 of the syndrome that resultsfrom long-term exposure to glucocorticoids has not been improvedupon, but our understanding of its pathophysiologic featuresand our ability to diagnose and treat the disorder have increaseddramatically.
Normal HypothalamicPituitaryAdrenal Physiology
Corticotropin-releasing hormone (CRH)2 is synthesized in thehypothalamus and carried to the anterior pituitary in portalblood (Figure 1). CRH, whose secretion is regulated by a varietyof neurotransmitters,3 is the most potent regulator of corticotropinsecretion. Arginine vasopressin and other hypothalamic agentsalso stimulate corticotropin secretion.4,5
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Figure 1. Physiologic and Pathophysiologic Features of the HypothalamicPituitaryAdrenal Axis in Normal Subjects and . . . [Full Text of this Article]
Pathophysiologic Features of Cushing's Syndrome
Corticotropin-Dependent Cushing's Syndrome
Cushing's Disease
Ectopic Corticotropin Syndrome
Ectopic CRH Syndrome
PseudoCushing's Syndrome
Corticotropin-Independent Cushing's Syndrome
Adrenocortical Tumors
Bilateral Micronodular Hyperplasia
Bilateral Macronodular Hyperplasia
Diagnosis
Diagnosis of Cushing's Syndrome
Clinical Diagnosis
Laboratory Confirmation
Distinguishing between Corticotropin-Dependent and Corticotropin-Independent Cushing's Syndrome
Determining the Source of Excess Corticotropin Secretion
High-Dose Dexamethasone Suppression Tests
Metyrapone Stimulation Test
Petrosal Venous Sinus Catheterization
Determining the Source of Corticotropin in Patients Whose Hypersecretion Cannot Be Suppressed with Dexamethasone
Imaging Procedures
Role of the CRH Stimulation Test
Treatment
Cushing's Disease
Ectopic Corticotropin and CRH Syndromes
Primary Adrenal Disease
Summary
Source Information
From the Division of Endocrinology, Department of Medicine, Vanderbilt Medical Center North, Nashville.
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