Acromegaly Flashcards

1
Q

Definition

A

· Constellation of signs and symptoms caused by hypersecretion of GH in adults

o Excess GH before puberty results in GIGANTISM

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2
Q

Aetiology/Risk factors

A

· Most cases are caused by a GH-secreting pituitary adenoma

· RARELY caused by excess GHRH causing somatotroph hyperplasia from hypothalamic ganglioneuroma, bronchial carcinoid or pancreatic tumours

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3
Q

Epidemiology

A

· RARE
· 5/1,000,000
· Age affected: 40-50 yrs

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4
Q

Presenting symptoms

A

· Very gradual progression of symptoms over many years

· Rings and shoes becoming tight

· Increased sweating

· Headaches

· Carpal tunnel syndrome

· Hypopituitary symptoms:
o Hypogonadism
o Hypothyroidism
o Hypoadrenalism

· Visual disturbances (due to compression of optic chiasm by tumour)

· Hyperprolactinaemia leading to:
o Irregular periods
o Decreased libido
o Impotence

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5
Q

Signs on physical examination (hands)

A

Hands

o Large spade-like hands
o Thick greasy skin
o Carpel tunnel syndrome signs
o Premature osteoarthritis

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6
Q

Signs on physical examination (face)

A

Face

o Prominent eyebrow ridge
o Prominent cheeks
o Broad nose bridge
o Prominent nasolabial folds
o Thick lips
o Increased gap between teeth
o Large tongue
o Prognathism
o Husky resonant voice (due to thickening of vocal cords)
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7
Q

Signs on physical examination (other)

A

· Visual Field Loss
o Bitemporal superior quadrantopia progressing to bitemporal hemianopia

· Neck
o Multinodular goitre

· Feet
o Enlarged

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8
Q

Investigations

A

· Serum IGF-1 - useful screening test
o GH stimulates IGF-1 secretion

· Oral Glucose Tolerance Test (OGTT)
o Positive result: failure of suppression of GH after 75 g oral glucose load

· Pituitary Function Tests
o 9am cortisol
o Free T4 and TSH
o LH and FSH
o Testosterone
o Prolactin

· MRI of Brain - visualise the pituitary adenoma

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9
Q

Management plan (medical)

A

· Medical - if surgery is contraindicated or refused

o Subcutaneous Somatostatin Analogues
· Examples: octreotide, lanreotide
· Side-effects: abdominal pain, steatorrhoea, glucose intolerance, gallstones

o Oral Dopamine Agonists
· Examples: bromocriptine, cabergoline
· Side-effects: nausea, vomiting, constipation, postural hypotension, psychosis (RARE)

o GH Antagonist (pegvisomant)

o Monitor
· GH and IGF1 levels can be used to monitor disease control

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10
Q

Management plan (other)

A

· Surgical - trans-sphenoidal hypophysectomy

· Radiotherapy - adjunctive to surgery

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11
Q

Possible complications (of surgery)

A

· Complications of Surgery

o Nasoseptal perforation
o Hypopituitarism
o Adenoma recurrence
o CSF leak
o Infection
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12
Q

Possible complications (CVS)

A

o Cardiomegaly

o Hypertension

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13
Q

Possible complications (respiratory)

A

o Obstructive sleep apnoea

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14
Q

Possible complications (GI)

A

o Colonic polyps

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15
Q

Possible complications (reproductive)

A

o Hyperprolactinaemia (in 30% of cases)

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16
Q

Possible complications (metabolic)

A

o Hypercalcaemia

o Hyperphosphataemia

o Renal stones

o Diabetes mellitus

o Hypertriglyceridaemia

17
Q

Possible complications (physiological)

A

o Depression

o Psychosis (from dopamine agonists)

18
Q

Prognosis

A

· GOOD with early diagnosis and treatment

· Physical changes are irreversible