Adrenal Hypertension

Question 1

Phaechromocytoma?

Answer 1

Tumour of adrenal medulla, chromaffin cells

Question 2

Pharmacology drugs for aldosterone pathway?

Answer 2

Renin inhibitors, ace inhibitors, aldosterone antagonists, a2 receptor blockers

Question 3

Who to screen for conn’s syndrome?

Answer 3

Hypokalaemia, resistant hypertension need 4 drugs, younger people

Question 4

Conns tests?

Answer 4

Initial screening- low renin high aldosterone

Confirmatory- oral/Iv na suppression test- in normal people their aldosterone levels will decrease.

Question 5

Why do adrenal venous sampling?

Answer 5

To assess whether autonomous hormone production is unilateral or bilateral

Question 6

Scan to lateralise aldosterone secretion?

Answer 6

Metomidate PET CT

Question 7

How to treat unilateral adenoma?

Answer 7

Laparoscopic adrenalectomy

Question 8

Bilateral hyperplasia treatment?

Answer 8

Aldosterone antagonists: spironolactone, eplerinone

Question 9

Effect of noradrenalin?

Answer 9

Alpha 1 and 2

Vasoconstriction, so high bp, pallor, glycogenolysis

Question 10

Role of adrenaline?

Answer 10

Alpha 1, beta 1 and 2

Vasoconstriction, increased heart rate, sweating, vasodilation in muscle

Question 11

Presentation of phaechromocytoma

A?

Answer 11

Spells; headaches, sweating, pallor, palpitation and anxiety

Hypertension, permanent or intermittent.

Family history

Question 12

Associated conditions with phaechromocytoma?

Answer 12

1. Neurofibromatosis type 1- tumours growing along nerves
2. Multiple endocrine neoplasia type 2
3. Von hippel- Lindau syndrome- formation of tumours and cysts around body

Question 13

Axillary freckling?

Answer 13

Neurofibromatosis type 1 - dark spots in armpits due to melanin deposition

Question 14

Biochemical diagnosis of phaechromocytoma?

Answer 14

24 hour urine: normetanephrines and methanephrine’s elevated x4
3 methoxytyromine (metabolite of dopamine)

Plasma high noradrenaline and adrenaline

Question 15

What else can cause high levels of catecholamines?

Answer 15

Obstructive sleep apnoea
Amphetamine like drugs
Ldopa
Labetalol (inhibits clearnace of adrenaline)

Urine dopamine doesnt come from adrenal medulla so measure urine methoxytyramine

Question 16

Phaechromocytoma management?

Answer 16

Alpha blockers: phenoxybenzamine, doxazocin

Beta blockers, propranolol

Laparoscopic adrenalectomy

Question 17

What percentage are genetic?

Answer 17

30%

Also check annually for metanephrines

Question 18

Adrenal causes of hypertension?

Answer 18

Hyperaldosteronism- zona glomerulosa (adenoma, hyperplasia, genetic cause)
Phaechromocytoma
Congenital adrenal hyperplasia

Question 19

How does glycyrrhetinic acid cause hypertension?

Answer 19

It inhibits renal 11-beta hydroxysteroid dehydrogenase, which allows cortisol to stimulate mineralocorticoid receptors.

Question 20

What percent of phaechromocytomas are diagnosed iin mortuary?

Answer 20

20

Question 21

In which condition do you get cafe au lait marks?

Answer 21

Neurofibromatosis type 1

Question 22

Multiple endocrine neoplasia type 2?

Answer 22

3 primary cancers: medullary thyroid, parathyroid tumour and phaechromocytoma

Question 23

What do you find in von-hippel landau?

Answer 23

Retinal hamangioglioblastoma (tumour due to growth of leaky irregular blood cells)

Cerebellar hameangioglioblastoma

Question 24

When would you do an MIBG scan?

Answer 24

Known or suspected neuroectodermally derived tumour

Question 25

When would you use PET?

Answer 25

Extra-adrenal (paragangliomas) or metastases

Question 26

What percent of phaechromocytomas are malignant?

Answer 26

10%

Question 27

What is essential hyperaldosteronism?

Answer 27

High blood pressure but not due to any medical conditions

Question 28

What percent of pheochromocytoma are diagnosed in mortuary?

Answer 28

20

Question 29

What do you see in von hippel landau syndrome?

Answer 29

Retinal hemangioglioblastoma

Cerebellar haemangioglioblastoma

Question 30

Which scan would you use for phaecytochroma?

Answer 30

MIBG scan

Pet ct- extra adrenal phaeos i.e paragangliomas or metastasis