Bals-Pratsch M, Hanker J P, Hellhammer D H, Lüdecke D K, Schlegel W, Schneider H P
Klinik und Poliklinik für Geburtshilfe und Frauenheilkunde der Universität Münster, Germany.
Horm Metab Res. 1996 Feb;28(2):105-10. doi: 10.1055/s-2007-979138.
Clinical symptoms of Cushing's syndrome were recognized in approximately 23 of 97 successive hirsute women attending the Endocrinological Department of the Women's Hospital. Endocrine and radiological examinations ultimately confirmed Cushing's syndrome caused by pituitary microadenomas (Cushing's disease) in only 2 women. In 17 of the remaining 21 patients suspected of having Cushing's disease hypercortisolism could not be diagnosed by classical endocrine tests. Thus further endocrinologial tests as corticotrophin releasing hormone (CRH)-tests (100 micrograms i.v.) and insulin tolerance tests were initiated including serum and stress-free salivary cortisol profiles during the day. Diurnal cortisol profiles showed higher values in the morning than during the night, but intermittent elevated cortisol concentrations could be detected. Finally, seven patients could be classified as suffering from a special form of pituitary ACTH-dependent hypercortisolism, named intermittent Cushing's disease (ICD). It is characterized by severe Cushingoid symptoms like marked central adiposity, purple striae, hirsutism and acne, intermittently increased cortisol concentrations, no cortisol response to hypoglycaemia, but preservation of some diurnal variation of serum or saliva cortisol. Three of these seven patients had diagnostic transsphenoidal neurosurgery and pituitary ACTH-producing microadenomas were removed in two women. Repeated determinations of the circadian rhythm of cortisol in saliva samples in combination with an overnight 1 mg dexamethasone suppression and an insulin stimulation test, are recommended to diagnose patients with suspicion on ICD even in an outpatient clinic.
在女子医院内分泌科就诊的97例连续多毛女性中，约23例出现了库欣综合征的临床症状。内分泌和放射学检查最终仅在2名女性中确诊为由垂体微腺瘤（库欣病）引起的库欣综合征。在其余21例疑似库欣病的患者中，有17例无法通过经典内分泌检查诊断出皮质醇增多症。因此，启动了进一步的内分泌检查，如促肾上腺皮质激素释放激素（CRH）试验（静脉注射100微克）和胰岛素耐量试验，包括白天的血清和无应激唾液皮质醇谱。昼夜皮质醇谱显示早晨的值高于夜间，但可检测到间歇性升高的皮质醇浓度。最后，7例患者可被归类为患有垂体促肾上腺皮质激素依赖性皮质醇增多症的一种特殊形式，称为间歇性库欣病（ICD）。其特征为严重的库欣样症状，如明显的向心性肥胖、紫纹、多毛和痤疮，皮质醇浓度间歇性升高，对低血糖无皮质醇反应，但血清或唾液皮质醇仍保留一些昼夜变化。这7例患者中有3例行诊断性经蝶窦神经外科手术，2名女性切除了产生垂体促肾上腺皮质激素的微腺瘤。建议对唾液样本中的皮质醇昼夜节律进行重复测定，并结合过夜1毫克地塞米松抑制试验和胰岛素刺激试验，即使在门诊也能诊断疑似ICD的患者。
