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頁籤選單縮合
題 名 | 庫欣氏症候群=Cushing's Syndrome |
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作 者 | 林興中; | 書刊名 | 內科學誌 |
卷 期 | 10:4 1999.08[民88.08] |
頁 次 | 頁144-152 |
分類號 | 415.934 |
關鍵詞 | 庫欣氏症候群; 醫源性; 異位性; 皮促素; Cushing's syndrome; Latrogenic; Ectopic; ACTH; |
語 文 | 中文(Chinese) |
中文摘要 | William Osler首先描述慢性皮質醇過量的臨床表徵,Harvey Cushing 則最早 提出腦下垂腺腫瘤可能是導致此症的重要原因(庫欣氏病),其後醫界陸續發現腎上腺腫 瘤、異位性皮促素分泌症候群也是「內源性庫欣氏症候群」的原因。其實臨床上以長期使 用過量合成的皮質醇造成「外源性庫欣氏症候群」最常見。庫欣氏症候群(CS)的症狀包 括體重增加、中心性肥胖、水牛肩、鎖骨上脂肪墊、月亮臉、多毛症、痤瘡、月經失調、 皮膚變薄、容易瘀血、骨質疏鬆、消化性潰瘍、肌病變、高血壓、葡萄糖耐量異常、低血 鉀、水腫、和色素過度沉著等症狀。臨床上被懷疑患有庫欣氏症候群者都應接受「內分泌 生化學檢查」來確認。目前臨床上最簡單可靠的篩選檢查為測量患者24小時尿液游離皮質 醇的排泄量,或測量其晚上11pm至清晨1am血漿或唾液皮質醇濃度。門診病患則以「單一 隔夜低劑量(1mg)迪皮醇抑制試驗較方便。一旦CS被確定後,下一步應區分患者是「AC H依賴性(ACTH>15pg/ml)」或「非ACTH依賴性(ACTH<5pg/ml)」。如果患者確定為「 ACTH依賴性」則應追查這些異常分泌的ACTH來源。臨床上最簡單的方法為進行「高劑量迪 皮醇抑制試驗」以便區分其為「庫欣氏病」或「異位性ACTH症候群」。庫欣氏病的定位以 高解析度核磁共振掃描(MRI)較電腦斷層掃描(CT)為佳,腎上腺腫瘤的定位則以上兩 者差不多,至於異位性ACTH腫瘤的定位,一般採用CT或MRI來掃描胸腔或腹部,對於特別 難以確定位置的腫瘤,則以□In-Octreotide掃描來加以定位。庫欣氏症候群的治療還是 以手術切除腫瘤為優先考慮,但18歲以下的「庫欣氏病」患者以放射治療效果亦佳。至於 那些不能接受手術治療患者則應給以藥物治療以便控制症狀,這些藥物作用機轉包括抑制 ACTH的分泌、抑制腎上腺皮質醇的合成、分解並破壞腎上腺皮質層、及抑制皮質醇的週邊 作用等。但不管採用何種治療方式,每一位病患都應該定期至醫院接受追蹤檢查。 |
英文摘要 | Cushing's syndrome is caused by chronic exposure of either endogenous or exogenous glucocorticoids (so called iatrogenic Cushing's syndrome). Cushing's syndrome may be either ACTH-dependent or ACTH- independent. The former includes Cushing's disease (primary pituitary ACTH hypersecretion), which is the most common cause of endogenous Cushing's syndrome, the ectopic ACTH or CRH syndrome, and the rare exogenous ACTH administration. The latter includes adrenal adenoma or carcinoma, the more rare bilateral micronodular or macronodular hyperplasia, and the most commonly seen iatrogenic or factitious Cushing's syndrome. During the past decade, new diagnostic tests and procedures have emerged and improved the ability to recognize even mild hypercortisolism and have provided the means to secure an accurate differential diagnosis. Furthermore, various drugs have been employed in the management of Cushing's syndrome if surgical removal of tumors causing the syndrome is failure or incomplete. This article reviews new tests in the evaluation of patients with suspected Cushing's syndrome, and outlines the major therapies available nowadays for the management of Cushing's syndrome. (J Intern Med Taiwan 1999;10: 144-152) |
本系統中英文摘要資訊取自各篇刊載內容。