acromegaly
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Etiology
Pathology
- hypersecretion of growth hormone (GH) by the pituitary after closure of epiphyses.
- presumably, hypersecretion of somatomedin C would result in a similar disorder
* GH acts via somatomedin C for most, if not all, its activity
Clinical manifestations
- headache
- visual loss
- muscular weakness
- pain
- back pain
- pain in extremities
- bone pain
- arthralgia: severe, premature osteoarthritis
- calcium pyrophosphate deposition (CPPD)
- sweating
- amenorrhea
- endocrine manifestations
- facial edema[3]
- exophthalmos
- enlargement of:
- hands & feet
- skull
- accentuated supraorbital ridges
- mandible with separation of teeth
- nose
- lips
- tongue
- cardiomegaly
- hepatomegaly
- hypertrichosis
- hypertension
- oily skin
* images[7]
Laboratory
- serum growth hormone (GH)
- random
- carbohydrate growth hormone (GH) suppression test
- elevated serum GH 1-2 hours after 100 g of glucose (failure to suppress to < 1 ng/mL)[2]
- elevated serum GH 30, 60 & 90 minutes after a glucose load (failure to suppress to < 5 ng/mL)[3]
- elevated serum GH 30, 60,90, 120 & 150 minutes after 75 glucose load (failure to suppress to < 1 ng/mL)[3]
- most specific test[3]
- serum somatomedin C (IGF-1) is increased*
- good sensitivity, poor specificity[3]
- single best test for making diagnosis of acromegaly[3]
- serum growth hormone-releasing hormone
- serum chemistries
- elevated serum phosphate
- elevated serum alkaline phosphatase
- serum glucose: hyperglycemia may be present
- urinalysis
- increased urinary 17-ketosteroids
- glycosuria may be present
- oral glucose tolerance test to confirm elevated serum IGF-1[3]
- see ARUP consult[5]
* initial diagnostic test[3]
Radiology
- plain films
- overgrowth of cancellous bone
- enlarged sella turcica
- osteoporosis
- tufted phalangeal tips
- signs of osteoarthritis
- MRI of the sella turcica to determine tumor size
Complications
- increased incidence of tubular adenomas
- 2-3 fold increase in mortality when plasma growth hormone & IGF-1 levels are not normalized
Differential diagnosis
Management
- transphenoidal surgical excision of tumor
- primary treatment of choice[3]
- radiation therapy
- primary ablative therapy
- post-operatively perisistent disease
- cure rate of 70% in 10 years
- long latent period (months to years) before disease is activity controlled
- used in conjunction with pharmacologic therapy
- pharmacologic therapy for residual disease[3]
- octreotide 100-200 mg SC every 8 hours ( 20 mg/28 day) had been preferred agent[3]
- pasireotide 40 mg/28 days may be superior to octreotide[9]
- dopamine agonists not as effective as octreotide
- cabergoline is the most effective dopamine agonist[3]
- lanreotide
- pegvisomant
- periodic colonoscopy
More general terms
Additional terms
References
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 861
- ↑ 2.0 2.1 Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 195-96
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018, 2022.
- ↑ AACE medical guidelines for clinical practice for the diagnosis and treatment of acromegaly. Endocr Pract 2004; 10(3):213 PMID: https://www.ncbi.nlm.nih.gov/pubmed/15382339 (Corresponding guideline updated Dec 2012)
Jellinger PS AACE Task Force for Management of Dyslipidemia and Prevention of Atherosclerosis. American Association of Clinical Endocrinologists' guidelines for management of dyslipidemia and prevention of atherosclerosis. Endocr Pract 2012 Mar-Apr;18(Suppl 1):1-78 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22522068 - ↑ 5.0 5.1 ARUP Consult: Acromegaly The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/acromegaly
ARUP Consult: Acromegaly Testing Algorithm https://arupconsult.com/algorithm/acromegaly-testing-algorithm - ↑ Katznelson L, Atkinson JL, Cook DM et al American Association of Clinical Endocrinologists medical guidelines for clinical practice for the diagnosis and treatment of acromegaly - 2011 update. Endocr Pract. 2011 Jul-Aug;17 Suppl 4:1-44. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21846616 (corresponding NGC guideline withdrawn Nov 2016)
- ↑ 7.0 7.1 DermNet NZ. Acromegaly (images) http://www.dermnetnz.org/systemic/acromegaly.html
- ↑ Diaz-Thomas A, Kemp A Medscape: Gigantism and Acromegaly http://emedicine.medscape.com/article/925446-overview
- ↑ 9.0 9.1 Colao A, Bronstein MD, Freda P et al Pasireotide versus octreotide in acromegaly: a head-to-head superiority study. J Clin Endocrinol Metab. 2014 Mar;99(3):791-9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24423324 Free PMC Article
- ↑ Katznelson L, Laws ER Jr, Melmed S et al Acromegaly: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014 Nov;99(11):3933-51. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25356808
- ↑ Melmed S Acromegaly pathogenesis and treatment. J Clin Invest. 2009 Nov;119(11):3189-202 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19884662 Free PMC Article
- ↑ National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) web page 'Acromegaly' http://www.niddk.nih.gov/health/endo/pubs/acro/acro.htm