Britni Hebert PGY 2 4/9/10 Notes located in presenter note

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Cushings Syndrome

• Britni Hebert
• PGY 2
• 4/9/10
• Notes located in presenter note section below
  each slide.

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Outline

• Presentation
• Diagnosis
• Diagnose Hypercortisolism
• Isolate source
• Therapy

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Normal HPA Physiology
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Presentation

• Weight Gain
• Glucose Intolerance
• HTN
• Hypokalemia
• Proximal Muscle Weakness
• Infections

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Phenotype
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Diagnosing Cushings
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Step 1 Diagnosing Hypercortisolism3 first line
tests

• Low Dose Dexamethasone Suppression Test
• Late Night Salivary Cortisol
• Urinary Cortisol

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Step 2 Isolate Source

• ACTH level
• lt 5 ACTH Independent
• gt20 ACTH Dependent

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in ACTH Dependent Cushings (like our
patient)Isolate Source... of ACTH

• ie High Dose Dexamethasone
• Confirm
• Image

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EAS by Source
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EAS by Source
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Treating Cushings
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Therapy

• Surgery by source when possible
• Adrenalectomy
• Transphenoidal resection
• Tumor Resection

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Therapy

• Adrenal Enzyme Inhibitors
• Ketoconazole first line
• Aminoglutethimide
• Metyrapone
• Etomidate

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Therapy

• Medical Adrenalectomy - Mitotane
• Glucocorticoid Antagonists - Mifepristone
• Somatostatin Analogues - Octreotide

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Summary
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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.

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Resources

• Up To Date
• Clinical Manifestations of Cushings Syndrome
• Establishing the Diagnosis of Cushings Syndrome
• Establishing the Cause of Cushings Syndrome
• Overview of the Treatment of Cushings Syndrome
• Diminishing Adrenal Cortisol Synthesis
• Ectopic ACTH syndrome. Andrea M. Isidori Andrea
  Lenzi, DFM Department of Pathophysiology,
  "Sapienza" University of Rome, Italy