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庫欣氏症候群實驗室診斷之介紹

An Introduction to the Laboratory Diagnosis of Cushing's Syndrome

摘要


庫欣氏症候群依病因分爲ACTH-dependent,包括垂線性及異位性;及ACTH-independent,即腎上腺性。實驗室診斷分爲三步驟-(一)篩檢或確定步驟,利用單劑量迪皮醇抑制試驗及UFC來篩檢懷疑患者,並進一步利用LDDST及LDDST/CRH test來確定那些志者爲庫欣氏症候群。(二)鑑別診斷步驟,利用血漿ACTH測定,來區分庫欣氏症候群息者爲ACTH-dependent或ACTH-independent,並進一步利用HDDST、CRH test及desmopressin test將ACTH-dependent患者,區分爲垂腺性或異位性。(三)腫瘤定位,若患者爲腎上腺性,應施行腎上腺CT scan;若爲垂腺性,應施行蝶鞍MRI,當MRI與法確定腫瘤時,可施行BIPSS,進一步定位腫瘤所在;若爲異位性,應施行肺部、縱膈腔及腹部之CT scan,必要時可施行octreotide核子醫學掃瞄。

關鍵字

庫欣氏症候群

並列摘要


Cushing's syndrome, a status of chronic, sustained hypersecretion of cortisol, can be divided into ACTH-dependent, in which the excessive ACTH secretion comes from a pituitary tumor (Cushing's disease) or a neuroendocrine tumor (ectopic), and ACTH-independent, in which the excessive production of cortisol comes from adrenal tumors. The first step of laboratory diagnosis is to screen suspected patients using the single dose dexamethasone suppression test and urine free cortisol determination and then to confirm further hypersecretion of cortisol by LDDST and LDDST/CRH test. The second step of laboratory diagnosis is to differentiate proven patients with Cushing's syndrome into ACTH-dependent and -independent by plasma ACTH-IRMA, and then to distinguish further Cushing’s disease patients from those with ectopic ACTU syndrome using the HDDST, CR11 test and desmopressin test. In the third step of tumor localization, adrenal CT scan is sufficient for adrenal Cushing's syndrome; MRI of sella is better than CT scan for the localization of pituitary microadenoma causing Cushing's disease. If MRI of sella is negative, BIPSS is indicated. For patients with ectopic Cushing's syndrome, CT scan of lung, mediastium and abdomen should be performed. If bronchial carcinoid is suspected, octreotide radionuclectide scanning is helpful.

並列關鍵字

Cushing's syndrome

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