[A gentle giant...].
Acromegaly is a rare disease with insiduous onset of clinical, endocrine and local, manifestations, and therefore often delayed diagnosis. Acral enlargement like prognathism and a bulky sweaty handshake, hyperhidrosis and thickening of the skin are almost always present, and, because of the cardiorespiratory comorbidities mortality is threefold increased compared to the normal population. Measurement of hGH level > 1ng/mL after an oral glucose load and of elevated IGF-1 confirm the diagnosis. A pituitary macroadenoma can be shown by MRI in over 90 % of the patients, in less than 1 % an ectopic source of hGH or GHRH may be the cause. The aim of the treatment is to stop the hGH overproduction and to prevent complications. The treatment of choice is transsphenoidal surgery, often combined with pharmacological therapy with long-acting somatostatin analogs, pegvisomant and dopamine agonists, or gamma knife radiosurgery.
Abteilung Endokrinologie/Diabetologie, Departement Medizin, Luzerner Kantonsspital, Luzern. christoph.henzen@ksl.ch
Christoph
C
Henzen
D000172:Acromegaly / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D000236:Adenoma / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D003131:Combined Modality Therapy; D005260:Female; D049912:Growth Hormone-Secreting Pituitary Adenoma / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D019382:Human Growth Hormone / Q000097:blood; D006801:Humans; D007016:Hypophysectomy; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male; D018365:Neoplasm, Residual / Q000601:surgery; D010901:Pituitary Function Tests; D016634:Radiosurgery; D055815:Young Adult
D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I