Acromegaly and gigantism
AbstractAcromegaly (‘acron’ = extremity, ‘megale’ = great) is a rare clinical syndrome with only 3 to 4 cases per million seen annually. The paediatric form is called gigantism and is mainly due to mutations of aryl hydrocarbon receptor-interacting proteins (AIP) or abnormalities in the gene GPR101 resulting in pituitary adenomas.1Gigantism is extremely rare2 but the diagnosis is usually easier than in acromegaly.3 Other causes of gigantism may be MEN1, Carney complex and McCune-Albright syndrome.3 These children usually present 2 standard deviations taller than their peers. Pituitary adenomas in children also tend to be locally aggressive and often break through the sella turcica. The other manifestations are very similar to acromegaly and will be discussed below.3 Acromegaly is a chronic progressive multisystem disease seen in adults and is mainly caused by anterior pituitary tumours (macroadenomas, i.e. a tumour greater than 1 cm in size) that secretes excessive growth hormone (GH) from the pars distalis.4 It is a disease of middle age (4th to 6th decade.)5 The onset is insidious and is usually advanced before the diagnosis is made.3 The average time for diagnosis is 6–8–13,4 years.3,6
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