Acromegaly Flashcards

1
Q

What is acromegaly?

A

S/S caused by hypersecretion of GH in adults

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2
Q

What is the term used for excess GH before puberty?

A

GIGANTISM

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3
Q

What causes acromegaly?

A

GH-secreting pituitary adenoma (95%)
RARELY caused by ectopic GH (or GHRH) production by tumours e.g. pancreatic, lung

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4
Q

What condition is associated with acromegaly?

A

6% have MEN-1

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5
Q

Describe the epidemiology of acromegaly

A

RARE
5/ 1,000,000
Age: 40-50y

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6
Q

Give 3 S/S caused by pituitary tumour in acromegaly

A

Headache
Visual field defect: Bitemporal hemianopia
Hypopituitarism

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7
Q

List 6 changes in appearance that may arise in acromegaly

A

Enlargement of hands (spade-like) + feet (rings + shoes becoming tight)
Frontal bossing
Enlarged tongue (Macroglossia)
Coarsening of facial features
Growth of jaw (Prognathism)

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8
Q

How do symptoms arise in acromegaly?

A

Very gradual progression of symptoms over many years

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9
Q

Give an example of a nerve compression syndrome seen in acromegaly

A

Carpal tunnel syndrome

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10
Q

Name 4 skin features of acromegaly

A

Excessive sweating
Thick, oily skin
Development of skin tags
+/- mild hirsuitism in females

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11
Q

What arises due to articular overgrowth of synovial tissue and arthropathy in acromegaly?

A

Arthralgia
Osteoarthritis
Back pain
Kyphosis

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12
Q

What does insulin resistance in acromegaly lead to?

A

T2DM or glucose intolerance

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13
Q

Acromegaly can give symptoms of hypopituitarism. Describe these

A

Hypogonadism
Hypothyroidism
Hypoadrenalism

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14
Q

What other hormone is secreted in 1/3 of adenomas causing acromegaly? What does this result in?

A

Prolactin
Galactorrhoea + Amenorrhoea

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15
Q

What investigations should be used in acromegaly?

A

Serum IGF-1
(GH stimulates IGF-1 secretion)
Oral Glucose Tolerance Test confirms dx if IGF-1 levels are raised

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16
Q

What is a positive result in a oral glucose tolerance test?

A

75mg glucose load
Normal: GH suppressed to <2 mu/L with hyperglycaemia
Acromegaly: No suppression of GH (may also demonstrate impaired glucose tolerance)

17
Q

Describe first line treatment for acromegaly

A

Trans-sphenoidal hypophysectomy
+/- adjunctive drug Tx
Only curative tx

18
Q

When is acromegaly managed medically?

A

if tumour is inoperable or surgery unsuccessful

19
Q

Describe the medical treatment of acromegaly

A

SC Somatostatin Analogues
Oral Dopamine Agonists
GH Antagonist (pegvisomant)

20
Q

What is the MOA of somatostatin analogues?

A

Directly inhibit release of GH

21
Q

Give an examples of subcutaneous somatostatin analogues. List 4 side effects of these medications

A

Octreotide
Abdominal pain
Steatorrhoea
Glucose intolerance
Gallstones

22
Q

What is the MOA of Pegvisomant? How is it administered?

A

GH receptor antagonist, prevents dimerisation of the GH receptor
Doesn’t reduce tumour volume (surgery still needed if mass effect)
SC OD

23
Q

Why have dopamine agonists been superseded by somatostatin analogues?

A

Dopamine agonists effective only in a minority of patients

24
Q

Give 2 examples of oral dopamine agonists. List 4 side effects of these medications

A

Bromocriptine, Cabergoline
N+V
Constipation
Postural hypotension
Psychosis (RARE)

25
Q

What is used to monitor acromegaly disease control?

A

Serum IGF1 levels

26
Q

List 6 complications of acromegaly

A

HTN
T2DM
Cardiomyopathy
Colorectal cancer
OSA

27
Q

What is the prognosis in acromegaly?

A

GOOD with early dx + tx
Physical changes are irreversible

28
Q

What 5 complications may arise due to surgery in acromegaly?

A

Nasoseptal perforation
Hypopituitarism
Adenoma recurrence
CSF leak
Infection

29
Q

Which investigations may be performed following diagnosis of acromegaly?

A

Pituitary Function Tests
MRI of Brain: visualise pituitary adenoma

30
Q

What is measured in pituitary function tests?

A

9am cortisol
Free T4 + TSH
LH + FSH
Testosterone
Prolactin

31
Q

What additional intervention may be used in older patients/ following failed medical/ surgical treatment for acromegaly?

A

External irradiation