Acromegaly : Epidemiology, Symptoms, and Diagnosis(<SPECIAL ISSUES>Update on Growth Hormone Secreting Pituitary Adenomas)

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  • Arita Kazunori
    Department of Neurosurgery, Graduate School of Medical and Dental Sciences, Kagoshima University
  • Yunoue Shunji
    Department of Neurosurgery, Graduate School of Medical and Dental Sciences, Kagoshima University
  • Tominaga Atsushi
    Department of Neurosurgery, Graduate School of Biomedical Science, Hiroshima University
  • Kurisu Kaoru
    Department of Neurosurgery, Graduate School of Biomedical Science, Hiroshima University

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  • 先端巨大症 : 疫学・病態・診断(<特集>GH産生下垂体腺腫のupdate)

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Abstract

The reported annual incidence of acromegaly is 3-4 cases/one-million people and prevalence is 38-69/one-million. The incidence in Japan is estimated around 500 cases/year. Despite the fact that almost all of cases of the acromegaly were caused by GH producing pituitary adenoma, some rare pathologies such as ectopic GH secreting tumors and GH-RH producing tumors may also be causative factors of this disease. In general, picking up on the characteristic features of acromegaly leads to the correct diagnostic process. Recently, symptoms other than the features, such as impaired glucose tolerance, carpal tunnel syndrome, malocclusion of jaws, or sleep apnea syndrome occasionally becomes clue to the diagnosis. For the definitive diagnosis, verification of excessive growth hormone secretion, high blood IGF-1 level, and the existence of pituitary adenoma on magnetic resonance imaging is essential. Because of its pulsatile nature of secretion, the blood GH level in the healthy population briskly changes. Therefore, the random measurement of GH level sometimes fails to differentiate the normal physiological diurnal rhythm of GH secretion from GH hypersecretion. The definition of GH hypersecretion should be based on a nadir GH level being under 1 ng/mL during a 75 g glucose tolerance test. It is important to know that blood GH level may increase in some physiological or pathologic conditions, such as pain, puberty, stress, prolonged fasting, chronic renal failure, heart failure, diabetes mellitus, and malnutrition. IGF-1 is produced in the liver under the control of GH. Its blood concentration minimally fluctuates and represents total GH secretion for the previous several days. Blood IGF-1 levels in the normal population, however, vary according to gender and age. Therefore, the validation of the IGF-1 level should be based on the standard range of IGF-1 of a gender and age-matched control.

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