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库欣病:临床表现与诊断评估

Cushing's disease: clinical manifestations and diagnostic evaluation.

作者信息

Kirk L F, Hash R B, Katner H P, Jones T

机构信息

Lamar Family Medicine and Occupational Health Center, Barnsville, Georgia, USA.

出版信息

Am Fam Physician. 2000 Sep 1;62(5):1119-27, 1133-4.

PMID:10997535
Abstract

The most common endogenous cause of Cushing's syndrome is Cushing's disease. Frequent clinical findings include weight gain, truncal obesity, striae, hypertension, glucose intolerance and infections. Cranial nerve II may be affected by enlarging pituitary adenomas in Cushing's disease; cranial nerves III, IV and VI may also be affected. The evaluation of patients with suspected Cushing's disease and syndrome requires an understanding of the proper use and limitations of the tests commonly included in the diagnostic work-up. The best screening test for Cushing's syndrome is a 24-hour urine collection with analysis for urinary free cortisol excretion. Low-dose and high-dose dexamethasone suppression tests, corticotropin assays, a corticotropin-releasing hormone stimulation test and inferior petrosal sinus catheterization may be required for a definitive diagnosis. Magnetic resonance imaging is useful in localizing the lesion. Surgical removal of the lesion by a transphenoidal approach is usually successful, but long-term follow-up is required. Some patients require lifetime glucocorticoid replacement therapy.

摘要

库欣综合征最常见的内源性病因是库欣病。常见的临床症状包括体重增加、躯干肥胖、皮肤紫纹、高血压、糖耐量异常和感染。库欣病中,垂体腺瘤增大可能会影响Ⅱ脑神经;Ⅲ、Ⅳ和Ⅵ脑神经也可能受到影响。对疑似库欣病和综合征患者的评估需要了解诊断检查中常用检测方法的正确使用及局限性。库欣综合征最佳的筛查试验是收集24小时尿液并分析尿游离皮质醇排泄量。明确诊断可能需要进行低剂量和高剂量地塞米松抑制试验、促肾上腺皮质激素测定、促肾上腺皮质激素释放激素刺激试验以及岩下窦插管。磁共振成像有助于病变定位。经蝶窦入路手术切除病变通常是成功的,但需要长期随访。一些患者需要终身糖皮质激素替代治疗。

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1
Cushing's disease: clinical manifestations and diagnostic evaluation.库欣病:临床表现与诊断评估
Am Fam Physician. 2000 Sep 1;62(5):1119-27, 1133-4.
2
The dexamethasone-suppressed corticotropin-releasing hormone stimulation test and the desmopressin test to distinguish Cushing's syndrome from pseudo-Cushing's states.地塞米松抑制促肾上腺皮质激素释放激素刺激试验及去氨加压素试验用于鉴别库欣综合征与假性库欣状态。
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[Use of catheterization of the inferior petrosal sinus in the diagnosis of Cushing's disease].[岩下窦插管术在库欣病诊断中的应用]
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Inferior petrosal sinus sampling in the diagnostic evaluation of Cushing's syndrome: K.E.M. experience.岩下窦采血在库欣综合征诊断评估中的应用:K.E.M.的经验
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Cavernous and inferior petrosal sinus sampling in the evaluation of ACTH-dependent Cushing's syndrome.海绵窦及岩下窦采血在促肾上腺皮质激素(ACTH)依赖性库欣综合征评估中的应用
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[Differential diagnosis and medical treatment in Cushing's disease].[库欣病的鉴别诊断与医学治疗]
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