Cushing's Syndrome
Overview
库欣综合征是一种罕见的激素紊乱，当身体组织长时间暴露于过量的激素皮质醇时，就会发生这种疾病. 皮质醇由肾上腺产生，帮助身体对压力做出反应, such as surgery and illness, and recover from infections. 这种激素还有助于维持血压和心血管功能，调节蛋白质代谢, carbohydrates and fats.
Benign, or non-cancerous, 垂体瘤称为垂体腺瘤，分泌过量的促肾上腺皮质激素(ACTH)，导致大约70%的成人库欣综合征病例，被称为库欣病. Other causes include tumors of the adrenal glands, which are located above the kidneys, as well as tumors occurring elsewhere in the body, such as the lungs, that secrete ACTH. This condition is known as ectopic ACTH syndrome. Rarely, 有些人可能遗传了一个或多个内分泌腺的肿瘤，从而导致库欣综合征.
Cushing's syndrome is relatively rare, 每年每百万人中只有大约10到15人受到影响. 它最常见于20至50岁的成年人, with women accounting for over 70 percent of those diagnosed.
Our approach to Cushing's syndrome
UCSF is an international leader in endocrinology care. Our team provides comprehensive consultations, 广泛的肾上腺激素紊乱的评估和治疗, such as Cushing's syndrome.
库欣综合征的治疗取决于你的身体产生过多皮质醇的原因. 如果使用糖皮质激素治疗另一种疾病会升高你的皮质醇水平, your doctor will adjust the dosage. When an adrenal tumor is the cause, surgery is the first option, 如果手术不能解决症状，就进行药物治疗或放射治疗. 身体其他部位的癌变也会导致库欣综合征. In these cases, the treatment choice – surgery, radiation, chemotherapy, 免疫疗法或这两种疗法的结合将取决于癌症的类型和扩散程度.
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Signs & symptoms
Symptoms of Cushing's syndrome may include:
- Change in body form, 尤其是面部的体重增加，即所谓的月相，以及锁骨上方的脂肪沉积, called supraclavicular fat, and at the back of neck, called a buffalo hump.
- 皮肤变化容易出现瘀伤、紫色妊娠纹(称为纹)和脸颊发红
- Excess hair growth on face, neck, chest, abdomen and thighs
- Overall weakness and fatigue
- Loss of muscle bulk
- Menstrual disorders in women
- Decreased fertility and/or sex drive
- Hypertension
- Diabetes mellitus
- Depression with wide mood swings
Diagnosis
库欣综合征的诊断是基于对病史的回顾, physical examination and laboratory tests, 哪些有助于确定皮质醇水平是否过高. 肾上腺或垂体的x线检查通常对定位肿瘤有用. In addition, 比较老照片和近照往往会突出库欣综合征患者面部和身体外观的变化. 诊断库欣病的第一步是确认皮质醇分泌过多的存在. This diagnosis is most easily made by the following tests:
- 低剂量地塞米松抑制试验，包括在晚上11点服用小剂量的可的松样药物地塞米松(1mg).m. and having blood drawn for cortisol the following morning. 在这些样本中，正常人的皮质醇水平通常很低, indicating that ACTH secretion is suppressed, 而皮质醇很容易被检测到，而且在库欣病患者中往往会增加.
- 24-hour urine collection to measure cortisol levels.
Once the diagnosis of Cushing's syndrome is established, the source of the excess cortisol needs to be determined. The source may be from an adrenal gland tumor, ACTH-producing tumor or a pituitary ACTH-producing adenoma. 岩窦取样是一种用于区分ACTH分泌来源的测试，在确定库欣综合征的诊断之前不应进行.
如果实验室检查显示垂体腺瘤是库欣综合征的病因, 进行垂体磁共振成像(MRI)以确认诊断.
Treatments
库欣综合征的治疗取决于皮质醇过量的具体原因，可能包括手术, radiation, chemotherapy or the use of cortisol-inhibiting drugs.
- Surgery. 手术通常包括经蝶窦切除腺瘤. 这是实现库欣综合征长期治愈的主要治疗方法之一.
- Medical therapy. In patients who fail to recover after surgery, 有几种药物可以帮助控制皮质醇水平. 这些药物包括针对肾上腺素的药物，如酮康唑, aminoglutethemide and metyrapone, 这些都有助于抑制肾上腺中皮质醇的产生. 它们都相对有效，但都有副作用，它们只对纠正激素问题起作用, not the cause of Cushing's syndrome.
- Radiotherapy. When Cushing's syndrome is not controlled with surgery, 常规外束或立体定向放射手术在控制皮质醇水平和肿瘤生长方面是有效的. However, 与立体定向放疗相比，外束放疗通常需要更长的时间来降低皮质醇水平. Also, 治疗后5至10年，外部放射束确实会导致正常垂体功能的丧失. Neurologic complications such as vision loss, 外束和立体定向放射治疗很少有虚弱和记忆障碍的报道.
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