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CONTENTS

Summary
Introduction
Epidemiology
Pathophysiology
Aetiology
Clinical features
Diagnosis
Clinical management
Pharmacoeconomics
Key points
References

SUMMARY

Acromegaly refers to an overproduction of growth hormone (GH; previously termed somatotrophin) by the pituitary gland and is most commonly the result of a pituitary tumour. It is a rare condition, with a global prevalence of about 60 cases per million people. The disease is usually diagnosed in middle-aged adults, who often present with altered features that result from the effects of excessive GH levels. Large or swollen hands and feet are common clinical features, as are a protruding brow and jaw, thickened nose and lips, increased sweating and other bone, joint and neuromuscular disorders. Mortality in patients with acromegaly is more than double that of the normal population and there is a strong association with cardiovascular and respiratory disease and diabetes. The biochemical diagnosis of acromegaly is confirmed by an excessive concentration of GH measured during an oral glucose tolerance test and/or levels of insulin-like growth factor 1 (IGF-I) above the normal age- and sex-adjusted reference range. Treatment options for the disease include surgical removal of the pituitary tumour, radiation therapy targeted at the tumour and pharmacological measures that either inhibit the secretion of GH from the pituitary (e.g. somatostatin analogues, dopamine receptor agonists) or block the peripheral effects of GH (e.g. the GH receptor antagonist, pegvisomant). Pegvisomant and somatostatin analogues are both effective in ameliorating the symptoms of acromegaly but acquisition costs are currently high.