Endocrine II Pathology: Adrenals

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Endocrine II Pathology Adrenals

• Amy Toscano-Zukor, MD
• Based upon a presentation prepared by
• Anne Marie Van Hoven, MD
• UMDNJ Robert Wood Johnson Medical School

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Adrenal Diseases

• Cushings Syndrome
• Addisons Disease
• Conns (primary hyperaldo)
• Pheochromocytoma

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Adrenal Gland
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Hormone Synthesis
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Cushings Syndrome
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Cushings Signs and Symptoms

• Moon facies
• Buffalo hump
• Purple striae
• Hypertension
• Proximal muscle weakness
• Central obesity

• Acne
• Easy Bruisability
• Gonadal dysfxn
• Hirsutism
• Osteopenia

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Work up for Cushings

• 24 hour urine free cortisol
• Morning and afternoon cortisol
• Low dose dexamethasone suppression test (give 1
  mg at midnight and check an early AM cortisol)
• High dose dexa supp test

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Treatment

• Surgery
• Radiation
• Medical - ketoconazole

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Addisons Disease etiologies

• Autoimmune (75)
• Metastasis lung, breast
• Hemorrhage anti coagulant, Waterhouse-Friderichs
  en, trauma
• Infectious (TB, CMV, Histo)
• HIV
• amyloidosis

• Hemachromatosis
• Adrenal Hypoplasia
• Drugs ketoconazole, metyrapone, phenytoin,
  barbiturates, rifampin

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Signs and symptoms

• Hyperpigmentation
• Hyponatremia
• Hyperkalemia
• Hypotension
• Hypoglycemia
• Postural sx
• Weakness/fatigue
• Vitiligo
• Eosinophilia
• Hypercalcemia

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Work up

• Am Cortisol
• ACTH stimulation test
• Use ACTH level to distinguish primary from
  secondary

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Treatment

• Hydrocortisone or any steroid replacement
• IV Fluids if is hypotension is a concern
• Mineralocorticoid if secondary (fludrocortisone)

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Endocrine Hypertension
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Actions of Aldosterone

• Increase Na resorption, increase volume, increase
  blood pressure
• Increase K excretion
• Decrease renin activity by negative feedback
• Decreased K means hydrogen moves into the cells
  which leads to metabolic alkalosis

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Conns Syndrome Primary Hyperaldosteronism

• One etiology of Endocrine Hypertension
• Consider in setting of resistant HTN, HTN
  hypokalemia, adrenal incidentaloma and HTN, HTN
  onset at age lt20, severe HTN (gtor 160 SBP or 100
  DBP), or whenever considering sec. HTN.
• Could be due to adenoma, bilateral adrenal
  hyperplasia or carcinoma

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Work Up

• Get AM blood sample in seated ambulent pt for
• Plasma aldosterone concentration (PAC)
• Plasma renin activity (PRA or PRC)
• IF ? PAC (15 ng/dL) ?PRA or ?PRC AND
• PAC/PRA ratio of 20 THEN you should investigate
  for Primary Aldosteronism by doing a saline
  suppression test
• Saline suppression- Measure Plasma aldo level
  before and after 2L normal saline bolus or
  measure 24 hour urine excretion of aldosterone
  after an oral sodium loading test.
• Elevated aldo levels are confirmatoyr of primary
  Aldosteronism because in the normal person, a
  sodium load should suppress aldosterone levels.

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Dx and Rx

• CT imaging
• Venous sampling
• Surgery
• Spironolactone or epleronone to suppress
  aldosterone

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Pheochromocytoma

• Paroxysmal
• HTN
• HA
• Sweating
• Palpitations
• Anxiety
• N/V
• fatigue

• Visual disturbances
• Facial pallor or flushing
• Cold hands/feet
• hyperglycemia

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Rule of 10 for describimg characteristics of
Pheos

• 10 are extra-adrenal
• 10 occur in children
• 10 are multiple or bilateral
• 10 recur after surgical resection
• 10 are malignant
• 10 are familial
• 10 of benign sporadic adrenal pheos are found as
  incidentalomas

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Dx and Rx

• 24 hour urine for catecholamines, metanephrine,
  normetanephrines
• Plasma free metanephrines
• Localize tumor by CT scan, MRI or MIBG
• Rx Alpha blockade, Beta blockade in preparation
  for surgery

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MEN

• MEN Type I
• parathyroid tumors
• pituitary tumors
• pancreatic tumors
• MEN Type IIa
• medullary thyroid carcinoma
• pheochromocytoma
• hyperparathyroidism
• MEN Type IIb
• medullary thyroid carcinoma
• pheochromocytoma
• mucosal neuromas