Acromegaly

Question 1

Define Acromegaly

Answer 1

Chronic, progressive multi organ disease, characterised by excessive circulation of Growth hormone and IGF1
This is most of the time caused by pituary somatotroph adenomas (micro-adenoma<10mm)

Acromegaly is when this occurs in adults
Children suffer of giantism

Question 2

Aetiology and risk factors of Acromegaly

Answer 2

99% of the cases are linked to pituitary somatotroph adenomas
In 30% of the cases, prolactin is co-secreted
The somatotrophin stimulate production of IGF1 which is the active component

Risk factors:
None really-multifactorial
Equal sex
Any age-most common middle age
MEN-1 syndrome

Question 3

Epidiemology of Acromegaly

Answer 3

Pit adenomas occur in 15 % of normal people and are discovered on autopsy or incidentally
about 20% of those are somatoroph ones
Acromegaly is still a rare disease-8 in 100000

Question 4

Signs and Sx of Acromegaly

Answer 4

patients very often present with complications already because it often goes unnoticed for years

Larger facial features-bigger nose, frontal bossing, Jaw enlargement
Separation of teeth, macroglossion
Usually unaware-very slow-compare old pictures but always present
-> increased Snoring

Increased skin thickness
Carpal tunnel syndrome

Long standing disease-Joint pain and dysfunction, with OA

Associated Hyperprolactin-loss of libido, erectile dysfunction, Galactorrhoae,

Other typical things:
FLAWS-
Increased appetite
Hypertension (cardiomyopathy)
Organomegaly (goitre/prostate most common)

Diabetes-Increased appetite, polyuria, polydipsia

Visual field-bitemporal hemianopia

Question 5

Investigations of Acromegaly

Answer 5

Serum IGF1-elevated (stable levels in day unlike GH)
Malnutrition, hypothydroisim can alter result

Oral Glucose tolerance test-give 75g oral glucose-should suppress GH levels
Failure to suppress in adenomas

Random serum GH-can be elevated

CT scan of pit
Serum glucose-diabetes
Prolactin-elevated

Question 6

Management of Acromegaly

Answer 6

1st line Mainstay if surgery-transphenoidal resection of tumour (through the nose)

2nd line-Medically-
somatostatin analogues-for if surgery isn’t enough, or reduce size before surgery
-octreotide/lanretotide
Resistance to those exist in 60% of pt

dopamine agonist
-Carbegoline

GH receptor antagonist-
-pegvisomant

3rd line-radiotherapy

In the long run-agressive treatment of complication (HTN, T2DM) and constant monitoring of IGF1 levels

Question 7

Complications of Acromegaly

Answer 7

Hypertension and arrythmias
Diabetes miellitus

Cardiac hypertrophy, valvular disease, MI-main cause of death

Sleep apnoea-and super tired

Osteoarticular complications

carpal tunnel syndrome

patients very often present with these because it often goes unnoticed for years

Question 8

Prognosis of Acromegaly

Answer 8

Untreated-serious heart complications and early death
3x normal pop of CVD death
Surgery and strict control of IGF1 after has nearly fully remedied that

need aggressive treatment of complications

QOL is nearly always lowered

SSA sensitive patients have a better time