Acromegaly- quick review card
Acromegaly Rare disorders due to excessive production of growth hormone ( GH ) from the anterior pituitary Benign tumour or adenoma ( usually macroadenoma ie > 1 cm ) of the pituitary somatotroph cells ( ant pituitary ) either purely GH secreting or mixed , ectopic – produced by other tumours familial syndromes as MEN type 1
slow insidious disease usually diagnosed aged 4th or 5th decade of life delay in diagnosis is common ( 5-10 yrs ) clinical manifestations depend upon ○ levels of GH and IGF-1 ○ sensitivity of the different tissues & organs to the increased levels of GH & IGF-1 ○ tumor size ○ age
disproportionate skeletal , tissue and organ growth dysmorphic syndrome ( new bone formation ) ○ extremities – hands and feet are broadened , fingers are widened , thickened and stubby , soft tissue is thickened ( soft tissue hypertrophy , excess growth of bone & cartilage and also bone formation ) ○ nose is widened & thickened , cheek bones are obvious , forehead bulges , thick lips , mandibular overgrowth with prognathism , maxillary widening , teeth separation & jaw malocclusion symptoms as malodorous sweating , skin tags , fatigue , lethargy , headache , sweaty oily skin
IGF-1 ( Insulin like growth factor ) excess leads to the characteristic overgrowth of certain tissues leading to the typical facial features , enlarged hands and feet and the affects on multiple systems throughout the body tumoral mass effect (headache , visual defects , hydrocephaly )
clinical suspicion and biochemical confirmation GH excess which is not suppressed by following an oral glucose load normal suppression of GH levels following a load of 75 to 100 gms of glucose would not happen in people with acromegaly Increase in IGF-1 level MRI of the pituitary
surgery is 1st line treatment – tumour removal radiotherapy medical management as ○ dopamine agonists ( cabergoline ) ○ somatostatin receptor ligands ( octreotide ) ○ GH receptor antagonists
REFERENCES
- Adigun OO, Nguyen M, Fox TJ, et al. Acromegaly. [Updated 2021 Dec 21]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK431086/
- Chanson, Philippe, and Sylvie Salenave. “Acromegaly.” Orphanet journal of rare diseases vol. 3 17. 25 Jun. 2008, doi:10.1186/1750-1172-3-17 Acromegaly (nih.gov)
- AlDallal, Salma. “Acromegaly: a challenging condition to diagnose.” International journal of general medicine vol. 11 337-343. 24 Aug. 2018, doi:10.2147/IJGM.S169611
- Ben-Shlomo, Anat, and Shlomo Melmed. “Acromegaly.” Endocrinology and metabolism clinics of North America vol. 37,1 (2008): 101-22, viii. doi:10.1016/j.ecl.2007.10.002 Acromegaly (nih.gov)
- Melmed, Shlomo. “Acromegaly pathogenesis and treatment.” The Journal of clinical investigation vol. 119,11 (2009): 3189-202. doi:10.1172/JCI39375
- National Endocrine and Metabolic Diseases Information Service Acromegaly
