Acromegaly Tổng quan về bệnh

Giới thiệu

Acromegaly is a chronic, slowly developing disease with progressive disfigurement and disability.

Dịch tễ học

Acromegaly is a rare disease with an estimated prevalence of 70 to 80 cases per million population (cpmp) and an incidence of 3 to 11 cpmp per year based on existing data from western countries. The mean age of diagnosis is early to mid-40s in both males and females. The condition equally affects males and females. The diagnosis is often delayed by up to 4 to 7 years due to its insidious onset. There is limited epidemiological data in Asia, however, based on the data from some Asian countries, the approximated prevalence of acromegaly is at 28 cpmp and its incidence is at 4 cpmp per year. 

Sinh lý bệnh

More than 95% of patients with acromegaly have hypersecretion of growth hormone (GH) and insulin-like growth factor 1 (IGF-1) secondary to a GH-secreting pituitary adenoma that arises from somatotroph cells. In response to inducing signals including hypothalamic growth hormone-releasing hormone (GHRH), GH is synthesized and stored in somatotroph cells. The production of GH is suppressed by somatostatin signaling primarily through the somatostatin receptor subtype 2. Additionally, peripheral signals such as IGF-1, steroids, and paracrine growth factors regulate the production of GH. However, in less than 5% of patients with acromegaly, excess GH secretion is caused by a hypothalamic tumor or a neuroendocrine tumor that leads to somatotroph hyperplasia and acromegaly.