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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

  1. 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/
  2. 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)
  3. 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
  4. 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)
  5. Melmed, Shlomo. “Acromegaly pathogenesis and treatment.” The Journal of clinical investigation vol. 119,11 (2009): 3189-202. doi:10.1172/JCI39375
  6. National Endocrine and Metabolic Diseases Information Service Acromegaly

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