Endocrine: 15.11: Adrenal Medulla Flashcards

1
Q

The adrenal medulla is composed of _____ derived from _____.

A
  • chromaffin cells
  • from neural crest cells
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2
Q

How does pheochromocytoma look grossly?

A

a brown tumor of the adrenal medulla

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3
Q

Is pheochromocytoma benign or malignant?

A

usu. benign (90% of the time)

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4
Q

Name 2 neurocutaneous disorders assoc. with pheochromocytoma.

A
  1. Neurofibromatosis type 1
  2. von Hippel-Lindau disease
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5
Q

What is phentolamine? What is it used to treat?

A
  • a reversible nonselective alpha blocker
  • used to treat pts on MAOIs that have tyramine reaction
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6
Q

This drug is a reversible nonselective alpha blocker used to treat pts on MAOIs that have tyramine reaction.

A

phentolamine

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7
Q

What are the s/s of pheochromocytoma?

A
  • episodic HTN
  • HA
  • palpitations
  • tachycardia
  • sweating
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8
Q

This drug is a nonselective, irreversible alpha blocker used pre-op in pheochromocytoma to prevent catecholamine (hypertensive) crisis.

A

phenoxybenzamine

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9
Q

Name a selective alpha-2 blocker. What is it used to treat?

A
  • Mirtazapine
  • it’s an atypical antidepressant
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10
Q

Other than the kidney, where else can pheochromocytomas occur?

A

the bladder wall

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11
Q

What is a pheochromocytoma?

A

a tumor of the chromaffin cells of the adrenal medulla

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12
Q

Mutation causing this disease?

  • café-au-lait spots
  • cutaneous neurofibromas
  • optic gliomas
  • pheochromocytomas
  • Lisch nodules
A
  • mutation of NF1 gene on Ch. 17
  • disease = Neurofibromatosis Type 1
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13
Q

What is phenoxybenzamine? What is it used to treat?

A
  • a nonselective, irreversible alpha blocker
  • used pre-op in pheochromocytoma to prevent catecholamine (hypertensive) crisis
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14
Q

Which MEN subtypes are assoc. with pheochromocytoma?

A
  • MEN 2A
  • MEN 2B
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15
Q

This is a tumor of the chromaffin cells of the adrenal medulla.

A

pheochromocytoma

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16
Q

Name 2 nonselective alpha blockers.

A
  1. phenoxybenzamine
  2. phentolamine
17
Q

What causes von Hippel-Lindau disease?

A

deletion of VHL gene on Ch. 3

18
Q

What do pheochromocytomas secrete?

A
  • Epi
  • NE
  • dopamine
19
Q

What enzyme breaks down the catecholamines?

A

monoamine oxidase

20
Q

Dx?

  • café-au-lait spots
  • cutaneous neurofibromas
  • optic gliomas
  • pheochromocytomas
  • Lisch nodules
A

Neurofibromatosis type 1

21
Q

How is pheochromocytoma diagnosed?

A
  • increased serum metanephrines
  • increased 24 urine metanephrines and VMA
  • *** all breakdown products of catecholamines
22
Q

What do the alpha-1 selective blockers end in?

A
  • -osin
  • ex:
    • prazosin
    • terazosin
    • doxazosin
    • tamsulosin (Flomax)
23
Q

What is the tx for pheochromocytoma?

A
  1. phenoxybenzamine first
  2. then beta-blockers
  3. then surgery
  • *** phenoxybenzamine and pheochromocytoma both have 16 letters ***