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
What is used to monitor acromegaly disease control?
Serum IGF1 levels
26
List 6 complications of acromegaly
HTN T2DM Cardiomyopathy Colorectal cancer OSA
27
What is the prognosis in acromegaly?
GOOD with early dx + tx Physical changes are irreversible
28
What 5 complications may arise due to surgery in acromegaly?
Nasoseptal perforation Hypopituitarism Adenoma recurrence CSF leak Infection
29
Which investigations may be performed following diagnosis of acromegaly?
Pituitary Function Tests MRI of Brain: visualise pituitary adenoma
30
What is measured in pituitary function tests?
9am cortisol Free T4 + TSH LH + FSH Testosterone Prolactin
31
What additional intervention may be used in older patients/ following failed medical/ surgical treatment for acromegaly?
External irradiation