pit

Question 1

name 3 familial syndromes that are strongly related to phaechromocytoma

Answer 1

• MEN type 2
• Von Hippel-Lindau syndrome
• Neurofibromatosis type 1

Question 2

what is MEN T2 characterised by (3)

Answer 2

• medullary thyroid cancer
• phaechromocytoma
• hyperparathyroidism

Question 3

inheritance of MEN T2

Answer 3

autosomal dominant

Question 4

causative mutation in MEN T2

Answer 4

RET proto-oncogene mutation

Question 5

derm features of Neurofibromatosis T1

Answer 5

cafe au lait spots
&
axillary and inguinal freckling

Question 6

neuroendocrine tumour of chromaffin cells

Answer 6

Phaechromocytoma

Question 7

phaechromocytoma triad?

Answer 7

• pounding episodic headache
• sweating
• tachycardia

Question 8

phaechromocytoma rule of 10

Answer 8

10% bilateral
10% cancerous
10% located outside adrenal

Question 9

metabolite of adrenaline?

Answer 9

metanephrines

Question 10

test for low probability phaechromocytoma

Answer 10

urinary fractionated metanephrines

Question 11

test for high probability phaechromocytoma

Answer 11

plasma free metanephrines

Question 12

why should beta blockers never be used as single therapy for phaechromocytoma?

Answer 12

can precipitate a hypertensive crisis from unopposed alpha-adrenergic stimulation… because peripheral beta-receptors have a vasodilatory effect

Question 13

alpha blockers for phaechromocytoma

Answer 13

• phenoxybenzamine

- doxazocin

Question 14

alternative to alpha blockers?

Answer 14

calcium channel blockers: amlodopine

Question 15

medication for patients that can not tolerate alpha or beta blockers

Answer 15

metyrosine

• inhibits catecholamine synthesis
• SE: depression, anxiety, nightmares

Question 16

condition caused by excess growth hormone?

Answer 16

Acromegaly

Question 17

physical appearance of individual w/ acromegaly

Answer 17

• frontal bossing (prominent forehead)
• Large nose
• Macroglossia
• large hands & feet
• prognathism (large protruding jaw)
• arthritis from imbalance of growth of joints

Question 18

GH excess prior to epiphyseal fusion? and what do untreated children become

Answer 18

Gigantism

very tall adult stature & rapid growth

Question 19

what cells & where does Growth hormone get produced

Answer 19

somatotrophic cells within anterior pituitary

Question 20

what does Growth hormone stimulate the release of? and where?

Answer 20

IGF-1 (pruced & released by liver)

Question 21

> 90% of acromegaly due to?

Answer 21

pituitary adenoma

Question 22

ectopic release of GHRH?

Answer 22

• carcinoid tumour

- small cell lung cancer

Question 23

High GH & IGF1 lead to increased risk of what condtions?

Answer 23

• CVD
• T2DM
• obstructive sleep apnoea
• organomegaly
• thyroid gland enlargement
• headache

Question 24

4 features of hyperprolactinaemia

Answer 24

• galactorrhoea
• dysmenorrhoea
• hypogonadism
• infertility

Question 25

1st line test for Acromegaly

Answer 25

IGF-1

Question 26

if 1st line test is not diagnostic what is 2nd line?

Answer 26

Oral glucose tolerant test glucose should suppress GH in healthy individuals

Question 27

1st line management of acromegaly?

Answer 27

Transphenoidam surgery

Question 28

medical treatment of acromegaly (3)

Answer 28

1) Somatostatin analogs (Octreotide) - monthly injection to reduce the levels of GH & shrink tumour 2) GH antagonists (Pegvisomant) - daily injection, lowers IGF1 levels 3) Dopamine agonists (Bromocriptine) - reduce release of GH, only effective for small proportion