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Endocrine Disorders

Endocrine Disorders. Learning Objectives. Pituitary Adenoma Acromegaly /gigantism Cushing’s disease Discuss epidemiology, causes, clinical manifestations, lab results, complications, treatment, and lifestyle modifications for both disease states. Major Endocrine Glands. 1 – Pineal gland

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Endocrine Disorders

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  1. Endocrine Disorders

  2. Learning Objectives • Pituitary Adenoma • Acromegaly/gigantism • Cushing’s disease • Discuss epidemiology, causes, clinical manifestations, lab results, complications, treatment, and lifestyle modifications for both disease states

  3. Major Endocrine Glands 1 – Pineal gland 2 – Pituitary gland 3 – Thyroid gland 4 – Thymus 5 – Adrenal gland 6 – Pancreas 7 – Ovary 8 – Testes

  4. Pituitary adenoma • A pituitary adenoma is an abnormal growth in the pituitary gland. • Problems occur when: • Overproduction of a certain hormone – Acromegaly and Cushing’s disease • Other pituitary cells growing larger and pressing on cells causing pituitary cells to not make as much hormone. • They can press on nearby structures in the brain such as the optic nerve

  5. Types of Pituitary Tumors • Endocrine Inactive – Does not secrete any hormones • Endocrine Active – Does secrete hormones

  6. Related Tumors

  7. Pituitary adenoma • Mass Effects: • Onset may be gradual due to tumor growth or sudden due to tumor bleeding (apoplexy) • Loss of peripheral vision, blurry vision • Fronto-orbital headache • Hypopituitarism (pituitary gland failure) • Fatigue, weight gain, depression, decreased libido, impotence, infertility, loss of menses

  8. Endonasal Pituitary Adenoma Removal http://www.youtube.com/watch?v=fY7p1bhg1P0&list=PLwWS6c78u0byN9EdQEIX772eV9ApAEigl&feature=player_detailpage#t=336

  9. Acromegaly/gigantism • Overview: • Caused by excessive and extended growth hormone secretion → ↑ IGF → causes growth changes • Due to a benign tumor in the anterior pituitary • Timing is important! • Gigantism before fusion of epiphysealplates • Acromegaly after fusion

  10. “It’s not my fault being the biggest and the strongest. I don’t even exercise” – Fezzik, The Princess Bride

  11. Acromegaly/Gigantism • Causes: • Proliferation of somatotroph cells in anterior pituitary • Benign tumor • Increased GH stimulates increased IGF-1 in liver • IGF-1 causes growth of tissues and metabolic abnormalities

  12. Acromegaly/Gigantism • Epidemiology: • Prevalence – 70/1,000,000 • Incidence – 3-4/1,000,000 per year • Neither disorder has strong genetic ties, although some genetic diseases can predispose to gigantism • Carney Complex, Multiple Endocrine Neoplasia

  13. Acromegaly/Gigantism • Clinical Manifestations: • Opthalmological • Vision blurring • Pulmonary • Sleep Apnea, Orthopnea • Dermatological • Thickening of skin and hair • Hyperhydrosis (sweating) • Muskuloskeletal • Skeletal growth and thickening, especially hand and foot • Widening of jaw and overbite • Joint pain • Abnormally heavy brow and prominent forehead • Husky voice due to larynx thickening

  14. Acromegaly/Gigantism • Diagnostic Testing: • IGF-1 levels • Growth Hormone levels • Pulsatile secretion – one high value is not indicative • Glucose tolerance test (glucose should suppress GH) • Brain MRI to visualize adenoma • Other Testing: • Pituitary hormone levels to assess tumor effect • TSH, FSH, LH, ACTH, Prolactin

  15. Acromegaly/Gigantism • Complications: • Cardiovascular • Cardiomyopathy, CHF • Valve dysfunction • Arrhythmia • Hypertension • Atherosclerosis • Pulmonary • Sleep apnea • Metabolic • Impaired glucose tolerance • Impaired lipid metabolism

  16. Metabolic Effects

  17. Acromegaly/Gigantism • Treatment: Goal is to lower levels of IGF-1, increase life expectancy • Physical Therapy • Surgery • Remove tumor • Medication • Somatostatin analogues • GH receptor antagonists • Dopamine agonists • Radiation Therapy • Gamma knife

  18. Cushing’s Disease • Overview • Caused by an extended increase in levels of ACTH, stimulating the adrenal glands to produce too much cortisol(a glucocorticoid) • Caused by a benign pituitary tumor • “Stress hormone” – Affects many organ systems, leading to metabolic, cardiovascular, and immune dysfunction

  19. Cushing’s Disease • Causes: • All symptoms caused by prolonged exposure to high levels of cortisol • Cushing’s Syndrome includes many causes most commonly iatrogenic • ACTH-independent • Cushing’s Disease is caused by pituitary adenoma • ACTH-dependent

  20. Cushing’s Disease • Epidemiology: • Prevalence – 13/1,000,000 • Incidence – 2/1,000,000 per year • 8x more common in women than men • Most common in ages 20-40 years

  21. Cushing’s Disease • Clinical Manifestation

  22. Cushing’s Disease • Diagnostic Testing: • Urinary Free Cortisol • Not specific unless 3x normal values • Dexamethasone suppression test • Negative feedback test – should be negative in pituitary adenoma • ACTH levels • Determine if adrenal or pituitary problem • Overnight Cortisollevels • Cortisollevels should fall throughout the day, lowest overnight • MRI to visualize tumor

  23. Cushing’s Disease • Complications: • Demineralization of bones causing osteoporosis • May progress to bone pain and even osteonecrosis • Generalized and sometimes extensive muscle weakness • Often unable to rise from chair unassisted • Cardiac hypertrophy, hypertension • May lead to CHF if untreated • Renal insufficiency • Renal stones due to excretion of calcium

  24. Cushing’s Disease • Treatment: goal is to restore hormone balance and reverse Cushing’s Syndrome • Surgery • Removal of pituitary adenoma • Removal of adrenal glands in refractory disease • Medication • Cortisol synthesis inhibitors (Ketoconazole) • Glucocorticoid type II receptor antagonist (Mifepristone) • Radiation • Gamma knife • Lifestyle modification • High protein diet for those with muscle wasting • Regular exercise • Clean and inspect all wounds

  25. Any Questions?

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