Acromegaly

Question 1

Definition of acromegaly

Answer 1

Rare chronic disease caused by excessive secretion of growth hormone, usually due to a pituitary somatotropin adenoma
• Gigantisism: disease onset in childhood (prior to epiphyseal growth plate closure)

Question 2

Aetiology of acromegaly

Answer 2

• Majority of cases due to somatotroph adenoma
• In rare cases, there is somatotroph hyperplasia
• An excess of growth hormone would also stimulate an increase in insulin-like growth factor 1 (IGF1), contributes to manifestations of the disease

Question 3

History and examination of acromegaly

Answer 3

• Insidious presentation: 10 years
• Headache
• Sweatiness
• Coarsening of facial features:
◦ Macroglossia
◦ Prominent nose
• Jaw enlargement: prognathism
• Increased hand and feet size: rings and shoes dont fit
• Snoring and obstructive sleep apnoea: larynx increases in size
• Hypertension
• Impaired glucose tolerance/diabetes
• Soft tissue and skin changes: increased skin thickness and soft tissue hypertrophy
• Joint pain and dysfunction
• Carpal tunnel syndrome: due to soft tissue hypertrophy

Question 4

Investigations for acromegaly

Answer 4

• Serum insulin-like growth factor 1 (IGF-1): recommended screening test for acromegaly, needed to confirm diagnosis (elevated)
• Oral glucose tolerance test: Give oral glucose load, there would be a PARADOXICAL rise of GH, can confirm diagnosis of acromegaly
• Prolactin can be raised

Question 5

Treatment for acromegaly

Answer 5

1) Trans-sphenoidal surgery: For a resectable tumour, surgery is 1st line treatment

If unresectable:
1) Somatostatin analogue (SSA): Octreotide can improve symptoms (taken via subcutaneous), reduce GH levels and reduce tumour size.
Or dopamine agonists

Question 6

Complications and prognosis of acromegaly

Answer 6

• Cardiac complications: increased risk of left ventricular hypertrophy, arrhythmias, valvular heat disease, cardiomyopathies
• Hypertension
• Sleep apnoea
• Osteoarticular complications
• Impaired glucose intolerance and diabetes
• Carpal tunnel syndrome

With treatment, the life expectancy and quality of life has been very much improved, nearly normal
However, without adequate treatment, acromegaly is associated with serious complications and premature death