Cushing’s disease and Addison’s disease 库欣病和艾迪森氏病

It is sufficient for most gynecologists to be familiar with the presentation and screening of Cushing’s and Addison’s disease in order to make appropriate and timely referrals.

对于大多数妇科医生来说，只要熟悉库欣病和艾迪森氏病的表现和检测方法就足够他们及时且恰当地给病人转诊。

Cushing’s disease is the result of oversecretion of cortisol. It may arise centrally from pituitary over-activity (the eponymous Cushing’s disease), or peripherally from the adrenal gland or elsewhere. Peripheral causes are much less common, and include paraneoplastic secretion of adrenocorticotropic hormone (ACTH) and autonomous secretion of cortisol by the pituitary.

库欣病是皮质醇分泌过多导致的疾病，皮质醇有可能来自垂体过度活动(同名库欣病)的中枢，或肾上腺及其他地方的外周。外周激素包括副肿瘤分泌的促肾上腺皮质激素(ACTH)和垂体自主分泌的皮质醇。

Presentation is variable, but classically includes moon facies and a “buffalo hump” of fat between the shoulders. Patients have truncal obesity with leg muscle atrophy. Women commonly have hirsutism. Other findings include fatigue, irritatility, elevated serum glucose, and depression (1).

库欣病的症状有多种表现，典型症状包括满月脸和肩部脂肪堆积成的“水牛驼峰”。患者有躯干肥胖伴腿部肌肉萎缩。女性通常患有多毛症。其他表现包括疲劳、烦躁、血清葡萄糖升高和抑郁(1)。

Screening is done with serum cortisol. An overnight, 1 mg dexamethasone suppression test can be done, with an 0800 fasting cortisol drawn. Values less than 5mcg/dL exclude Cushing’s Disease of any source. Confirmation of disease is done with 24-hour urinary cortisol testing. Differentiation of peripheral and central disease is done with two- to six-day dexamethasone suppression testing. It is important to remember that although Cushing’s Disease is a common reason for referral to an endocrinologist, it is one of the least common of diagnoses. It is thus important to be familiar not only with its presentation but with the evaluation of hirsutism, to avoid unnecessary tests and referrals.

库欣病通过血清皮质醇检测进行筛查。可以在早上8点空腹抽血用1mg地塞米松进行隔夜抑制试验。结果小于5mcg/dL可以排除库欣病。确诊试验需要通过尿液检测24小时皮质醇水平。区分外周疾病和中枢疾病，则需要2至6天地塞米松抑制试验来完成。不过，虽然库欣病是需要转给内分泌学家的常见疾病，但它确诊率很低。因此，医生不仅需要熟悉多毛症的表现，还要熟悉多毛症的评估方法，以避免不必要的检查和转诊，这点非常重要。

Addison’s disease is the result of adrenal insufficiency. Cushing’s disease has substantial implications for long-term health, but Addison’s disease can lead to death abruptly from Addisionian crisis unless appropriately diagnosed and treated. Of note, auto-immune disease can be a cause of Addison’s disease as well as of premature ovarian failure and thyroid disease. Consideration should be given to multiple endocrine failures when one such failure is found (2).

艾迪森氏病是肾上腺皮质功能减退症。库欣病对长期健康有重大影响，但艾迪森氏病如果没有及时诊断和适当治疗，可能会因急性肾上腺皮质危象而导致猝死。需要关注的是，自身免疫性疾病可能是艾迪森氏病以及卵巢早衰和甲状腺疾病的病因。当出现这类疾病时，应当考虑是否存在多种内分泌失调(2)。

Presentation is usually insidious. Common symptoms include orthostatic hypotension, hypoglycemia, fatigue, and muscle weakness. In an Addisonian crisis, patients present acutely ill, with nausea, vomiting, confusion to the point of psychotic symptoms, syncope, and multiple electrolyte disturbances (3).

艾迪森氏病的表现通常是潜伏性的，常见症状包括体位性低血压、低血糖、疲劳和肌肉无力。 在艾迪生病危象中，患者会出现急性病状，伴有恶心、呕吐、精神错乱到精神病症状、晕厥和多种电解质紊乱的症状(3)。

Addison’s disease is not in the purview of any gynecology provider. If the diagnosis is suspected, patients should be sent to an endocrinologist or for emergency care, as appropriate.

艾迪森氏病不属于妇科范围。如有疑似案例，应酌情将患者送往内分泌科或接受紧急护理。

References

参考文献

1. Newell-Price J, Grossman A. Diagonisis and management of Cushing’s syndrome. Lancet 1999 June 19;353: 2068-2079.

2. Eisenbarth GS, Gottlieb PA. Medical progress-autoimmune polyendocrine syndromes. New Engl J Med 2004; 350(20): 2068-2079.

3. Baker SJ, Wass JA. Addison’s disease. BMJ 2009 July 2; 339: NIL_0007-NIL_0010.