Acromegaly.JPEG
Facial Features of Acromegaly

Definition

[1]
Acromegaly is a condition where there is an excess secretion of Growth hormone resulting in enlarged growth.

Incidence

[2]
5 per million per year

Age

40-50
[3]

Gender

Equal incidence in both males and females

Aetiology

[4]
  • Pituitary Adenoma (99%)
  • Pituitary Hyperplasia

Risk Factors

[5]
  • Family History of Acromegaly

Signs and Symptoms

[6]

Pathophysiology

[7]
Growth hormone (GH) is produced and released in the Anterior Pituitary. It stimulates the production of Insulin-like Growth Factor 1 (IGF-1) in the liver which promotes organ growth. If the homeostatic mechanism is dysregulated, for example in a GH secreting pituitary adenoma then excessive IGF-1 is produced and excessive body growth occurs.

Investigations

[8]

Bloods

  • Serum IGF-1 - elevated
  • Random Serum Growth Hormone - >0.4 micrograms/L
  • Oral Glucose Tolerance Test - lowest GH value >0.4 micrograms/L

Imaging

  • MRI head (if acromegaly suspected) - pituitary adenoma

Diagnosis

[9]
Diagnosis is made on biochemical evidence of Growth Hormone or IGF-1 hypersecretion. An oral glucose tolerance test can be used to show that Growth Hormone is not suppressed by glucose intake.

Differentials to rule out

Treatment

[10]

Non-Pharmacological

  • Radiotherapy

Pharmacological

  • Somatostatin Analogue (Octreotide or lanreotide)
  • Dopamine agonist (Cabergoline)
  • Growth Hormone receptor antagonist (pegvisomant)

Surgical

  • Transsphenoid Pituitary Tumour resection (1st line)

Management

[11]
Patients with acromegaly should have lifelong follow up to monitor GH and IGF-1 levels. IGF-1 levels should be monitored every 6 months.

Complications

[12]

Prognosis

[13]
Patients with untreated acromegaly are likely to have a premature death and reduced quality of life due to complications. At present 70-90% of patients with small pituitary adenomas will have remission following surgical and biochemical treatment.

See Also

BMJ Best Practice - Acromegaly
Oxford Handbook of Clinical Medicine - 9th edition p230

Citation

Acromegaly photo: "Acromegaly facial features" by Philippe Chanson and Sylvie Salenave - Acromegaly. Orphanet Journal of Rare Diseases 2008, 3:17. doi:10.1186/1750-1172-3-17. Licensed under CC BY 2.0 via Commons - https://commons.wikimedia.org/wiki/File:Acromegaly_facial_features.JPEG#/media/File:Acromegaly_facial_features.JPEG
  1. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  2. ^ Oxford Handbook of Clinical Medicine - 9th edition p230
  3. ^ https://www.clinicalkey.com/topics/endocrinology/acromegaly.html
  4. ^ Oxford Handbook of Clinical Medicine - 9th edition p230
  5. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  6. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  7. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  8. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  9. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  10. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  11. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  12. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html
  13. ^ http://bestpractice.bmj.com.ezp.lib.unimelb.edu.au/best-practice/monograph/522.html