肢端肥大症

什么是肢端肥大症?

定义:
肢端肥大症 is a chronic metabolic disorder caused by the presence of too much growth hormone. It results in gradual enlargement of body tissues including the bones of the face, 下巴, 手, 脚, 和头骨.

替代的名字: Somatotroph adenoma; Growth hormone excess

Causes, incidence, and risk factors:
肢端肥大症 occurs in about 6 of every 100,000 adults. It is caused by abnormal production of growth hormone after normal growth of the skeleton and other organs is complete. Excessive production of growth hormone in children causes gigantism rather than acromegaly.

The cause of the increased hormone secretion is usually a benign tumor of the pituitary gland. 脑下垂体, which is located just below the brain, controls the production and release of several different hormones including growth hormone.

There are no known risk factors for acromegaly other than a prior history of a pituitary tumor.

症状:

• 扩大的手
• 扩大脚
• Widened fingers or toes due to skin overgrowth with swelling, redness, and pain
• Enlarged 下巴 (prognathism) and tongue
• 面部骨骼增大
• Thickening of the skin, skin tags
• Enlarged sebaceous glands
• 声音沙哑
• 头疼
• 容易疲劳
• 过度出汗
• Decreased muscle strength (weakness)
• 关节活动受限
• 关节疼痛
• 腕管综合症
• 宽间距齿
• Swelling of the bony areas around a joint

Additional symptoms that may be associated with this disease:

• Weight gain (unintentional)
• Hair, excessive on females

体征和测试:

• The level of growth hormone is high.
• The level of IGF-1 (insulin-like growth factor 1) is high.
• A spine X-ray shows abnormal bone growth.
• A cranial MRI or cranial CT scan may show a pituitary tumor.
• An echocardiogram may show a leaky mitral valve or a leaky aortic valve.

This disease may also alter the results of the following tests:

• Fasting plasma glucose (high)
• Glucose tolerance test (too high at 2 hours)
• 肌酸酐
• 肌酐-尿
• 17-ketosteroids
• 17-hydroxycorticosteroids

治疗:
Microsurgery to remove the pituitary tumor causing this condition corrects the abnormal growth hormone secretion in most patients. This surgery may not be available to patients in isolated geographic locations, so travel to a larger metropolitan area may be necessary for treatment.

Radiation of the pituitary gland is used for people who do not respond to the surgical treatment. However, the reduction in growth hormone levels after radiation is very slow.

Treatment with the medications cabergoline or octreotide may control growth hormone secretions in some people. Pegvisomant, a new drug that directly blocks the effects of growth hormone, has been shown to improve symptoms of acromegaly in recent studies. These medications may be used as initial treatment if surgery is unavailable or if the person is unable to tolerate surgery.

治疗后, periodic evaluation is necessary to ensure the normal functioning of the pituitary gland. Yearly evaluations are recommended.

支持组:

Expectations (prognosis):
Pituitary surgery is successful in up to 80% of patients, depending on the size of the tumor and the experience of the surgeon.

Without treatment the symptoms are progressive, and the risk of 心血管病 increases.

并发症:

• hypopituitarism (too little secretion of other pituitary hormones)
• 高血压
• glucose intolerance or diabetes
• 心血管病
• 腕管综合症
• 睡眠呼吸暂停
• 关节炎
• 结肠息肉
• 子宫肌瘤
• spinal cord compression
• 视觉异常

呼叫ing your health care provider:
呼叫 your health care provider if symptoms of acromegaly are present or if symptoms do not improve with treatment.

预防:
No measures exist to prevent the initial condition, but early treatment may prevent any worsening of complications associated with this disease.