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Cushing’s Syndrome. Britni Hebert PGY 2 4/9/10 Notes located in presenter note section below each slide. Outline. Presentation Diagnosis Diagnose Hypercortisolism Isolate source Therapy. Normal HPA Physiology. Presentation. Weight Gain Glucose Intolerance HTN Hypokalemia
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Cushing’s Syndrome • Britni Hebert • PGY 2 • 4/9/10 • Notes located in presenter note section below each slide.
Outline • Presentation • Diagnosis • Diagnose Hypercortisolism • Isolate source • Therapy
Presentation • Weight Gain • Glucose Intolerance • HTN • Hypokalemia • Proximal Muscle Weakness • Infections
Step 1: Diagnosing Hypercortisolism3 first line tests • Low Dose Dexamethasone Suppression Test • Late Night Salivary Cortisol • Urinary Cortisol
Step 2: Isolate Source • ACTH level • < 5 = ACTH Independent • >20 = ACTH Dependent
in ACTH Dependent Cushings (like our patient)Isolate Source... of ACTH • ie High Dose Dexamethasone • Confirm • Image
Therapy • Surgery by source when possible • Adrenalectomy • Transphenoidal resection • Tumor Resection
Therapy • Adrenal Enzyme Inhibitors • Ketoconazole – first line • Aminoglutethimide • Metyrapone • Etomidate
Therapy • Medical Adrenalectomy - Mitotane • Glucocorticoid Antagonists - Mifepristone • Somatostatin Analogues - Octreotide
Summary • Marked HTN and Hypokalemia are typical of ectopic ACTH secretion. • First Line therapy in unresectable disease = Ketoconazole • CV, HTN, VTE and infection commonly lead to death in the untreated Cushingoid patient. The former does not improve with therapy.
Resources • Up To Date • Clinical Manifestations of Cushing’s Syndrome • Establishing the Diagnosis of Cushing’s Syndrome • Establishing the Cause of Cushing’s Syndrome • Overview of the Treatment of Cushing’s Syndrome • Diminishing Adrenal Cortisol Synthesis • Ectopic ACTH syndrome. Andrea M. Isidori; Andrea Lenzi, DFM – Department of Pathophysiology, "Sapienza" University of Rome, Italy