Acromegaly Flashcards

1
Q

Acromegaly cause

A

Excess GH secretion
Most common - pituitary adenoma

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2
Q

Initial presentation of acromegaly

A

Middle age - starts young
T2DM + HPTN
Large fingers and toes
Visual field defect - bitemporal hemianopia or superiorquadrantopia

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3
Q

Clinical features of acromegaly

A

Coarse facial features, spade like hands, increase in shoe size
Increase inter dental spacing, large tongue, prognathism
Excessive sweating 9sweat glan hypertrophy)
Carpal tunnel syndrome
OSA - excess tissue in nose
CVD - HF, arrhythmias
Osteoporosis
Long term cancer risk
Raised prolactin -> galacotrrhea in 1/3
6% of patients MEN-1

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4
Q

Why does acromegaly cause visual field defect

A

Pituitary adenoma - pituitary crosses optic chiasm - adenoma

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5
Q

GH cycle

A

Hypothalamus -> GHRH -> pituitary -> GH -. bone, mucels, stomach, liver
Liver -> IGF-1, glucose -> Free fatty acids -> negative feedback to hypothalamus
SST from hypothalamus inhibits GH release from pituitary

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6
Q

What stimulates GHRH release from hypothalamus

A

Physical exercise
Amino acids
Hypoglycaemia
Gonadal steroids

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7
Q

Where does IGF-1 negatively feedback to

A

Hypothalamus AND pituitary

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8
Q

What is most accurate screening tool for acromegaly

A

IGF-1 - if high positive

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9
Q

When do an OGTT for acromegaly

A

When IGF-1 is moderately high but not high enough to diagnose outright

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10
Q

What is a positive OGTT

A

Non supression of GH (should be <1g/L) in response to glucose load

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11
Q

Ideal test for acromegaly once biochemically diagnosed

A

Pituitary MRI - look for enlarged pituitary or discrete adenoma

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12
Q

First line for acromegaly

A

Trans sphenoidal Surgery on pituiatry

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13
Q

2nd line for acromegaly post surgery

A

Continues GH after surgery -> pituitary radiotherapy (external beam radiotherapy), stereotactic radiosurgery, gamma radio therapy

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14
Q

3rd line treatment acromegaly

A

Medical - somatostatin analogues eg ocreotide, lanreotide LAR
Pegvisomant - high selective H receptor antagonist

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15
Q

Goal of acromegaly treatment

A

Reduce GH levels to normal range
Reduce pituitary size in case of risk of pressing on optic nerve

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16
Q

Long term managment of acromegaly

A

Cancer screening
Monitor end organ problems - ECHO, ECG, DEXA scan
Monitor IGF-1 level for recurrence

17
Q

What does ocreotide do

A

Supress GH - example of somatostatin
50-70% success

18
Q

What are a minority of acromegaly causes caused by

A

Ectopic GHRH or GH production by tumours eg pancreatic

19
Q

Complications acromegaly

A

HPTN
Diabetes >10%
Cardiomyopathy
Colorectal cancer

20
Q

What is normal in OGTT

A

GH supressed to <2mu/L with hyperglycaemia
Acromegaly - no supression

21
Q

Medical options for acromegaly

A

Somatostatin analogue
Pegvisomant
Doamine agonists

22
Q

What is pegvisomant

A

GH receptor antagonist
Once daily SC
V effective - 90%
Doesnt reduce tumour vol - still need surgery if mass effect

23
Q

Dopamine agonists example and how work

A

Bromocriptine
First med used, no largely replacced by somatostatin analogues