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Handb Clin Neurol. 2014;124:197-219. doi: 10.1016/B978-0-444-59602-4.00014-9.

Acromegaly.

Author information

1
Department of Endocrinology and Disorders of Reproduction, Hôpital Bicêtre and Reference Center for Rare Endocrine Disorders of Growth, Le Kremlin-Bicêtre, Paris, France; Faculty of Medicine, Université Paris-Sud 11, Le Kremlin-Bicêtre, Paris, France; INSERM U693, Le Kremlin-Bicêtre, Paris, France. Electronic address: philippe.chanson@bct.aphp.fr.
2
Department of Endocrinology and Disorders of Reproduction, Hôpital Bicêtre and Reference Center for Rare Endocrine Disorders of Growth, Le Kremlin-Bicêtre, Paris, France.
3
Department of Endocrinology and Disorders of Reproduction, Hôpital Bicêtre and Reference Center for Rare Endocrine Disorders of Growth, Le Kremlin-Bicêtre, Paris, France; Faculty of Medicine, Université Paris-Sud 11, Le Kremlin-Bicêtre, Paris, France; INSERM U693, Le Kremlin-Bicêtre, Paris, France.

Abstract

Acromegaly is due to excessive production of growth hormone (GH), generally by a pituitary GH-secreting adenoma. Its prevalence is estimated at 40-130 cases per million inhabitants. Acromegaly is characterized by slowly progressive acquired somatic disfigurement (mainly involving the face and extremities) and systemic manifestations. The rheumatologic, cardiovascular, respiratory, and metabolic consequences of acromegaly determine the prognosis. The diagnosis is confirmed by elevated serum GH concentrations that cannot be suppressed by an oral glucose load, and by increased levels of insulin-like growth factor 1 (IGF-1). Treatment is aimed at correcting (or preventing) tumor compression of surrounding tissues by excising the disease-causing lesion, and at reducing GH and IGF-1 levels to normal values. When surgery (the usual first-line treatment) fails to correct GH/IGF-1 hypersecretion, medical treatment with dopamine agonists (particularly cabergoline), somatostatin analogs, and/or radiotherapy can be used. The GH receptor antagonist pegvisomant is helpful in patients who are resistant to somatostatin analogs. Thanks to this multistep therapeutic strategy, adequate hormonal disease control is achieved in most patients, giving them a normal life expectancy. Comorbidities associated with acromegaly generally improve after treatment, but persistent sequelae may nonetheless impair quality of life.

KEYWORDS:

Acromegaly; growth hormone; insulin-like growth factor 1; pegvisomant; pituitary; somatostatin analogs

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