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PNMT (Phenylethanolamine N-methyltransferase)

  • Converts norepinephrine to epinephrine.
  • Found only in the adrenal medulla.

Pheochromocytoma:

  • 10% are:
    1. In children
    2. Malignant
    3. Part of MEN
    4. Bilateral
    5. Extra-adrenal (Organ of Zuckerkandl at Aortic bifurcation è most common extra adrenal location)

Screen with:

  • Urine metanephrines, VMA
  • MIBG can localizes

Preop:

  • Alpha block first
  • Then beta block if tachycardic


Nelson syndrome: Post adrenalectomy (10%)

  • Increase ACTH
  • Pigmentation
  • Vision changes from increase pituitary response

Waterhouse Friedrickson:

  • Adrenal hemorrhage a/w meningococcal sepsis

Conn's syndrome:

  • Primary hyperaldosteronism
  • (éAldosterone, êRenin, HTN, êK, éNa)
  • 70% unilateral adenoma
  • 30% bilateral hyperplasia (idiopathic hyperaldosteronism)
  • See with postural stimulation test


Addison's disease:

  • Low aldosterone and glucocorticoids
  • Low Na, high K, hypoglycemia
  • Crisis presents similar to sepsis with hypo-tension, fever
  • Steroids are diagnostic and therapeutic

Congenital Adrenal Hyperplasia:

  • 21-hydroxylase deficiency = most common

Cushing's syndrome:

  • Excess steroids
  • Most commonly iatrogenic.


Pituitary (Cushing's Disease): 70% non-iatrogenic

  • High ACTH
  • Suppresses w/high dose steroid test


Adrenal Cushing's Syndrome: (15%)

  • Low ACTH
  • Independent steroid production
  • Does not suppress


Ectopic Cushing's Syndrome: ACTH produced elsewhere

  • Usually small cell CA
  • Does not suppress


Posterior pituitary: neurohypophysis -> ADH, Oxytocin

Anterior pituitary: adenohypphysis -> GH, ACTH, TSH, LH, FSH, Prolactin

Bitemporal hemianopsia: is classical vision change with pituitary mass effect

Chromophobe pituitary adenoma: non-functional, see decrease GH, FSH, LH, TSH, ACTH

Prolactinoma: #1 = pituitary adenoma

  • Galactorrhea, irregular menses
  • Tx: Bromocriptine or trans-sphenoidal resection


Sheehan syndrome: postpartum lack of lactation, persistent amenorrhea

Pages in category "Pituitary/Adrenal"

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