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双侧肾上腺切除术后库欣病患者尼尔森综合征的发生能否预测?

[Is it possible to predict the development of Nelson's syndrome in Cushing's disease after bilateral adrenalectomy?].

作者信息

Horký K, Kanka J, Gregorová I, Tomsová Z, Dvoráková J

机构信息

III. interní katedra fakulty vseobecného lékarství Univerzity Karlovy, Praha.

出版信息

Cas Lek Cesk. 1990 May 25;129(21):645-9.

PMID:2164450
Abstract

A detailed analysis of clinical and laboratory findings in a group of 66 patients with Cushing's disease treated with bilateral adrenalectomy, out of whom 15 developed Nelson's syndrome, gave evidence that Nelson's syndrome mostly affects children and young patients compared to older persons. Bilateral adrenalectomy performed in patients aged over 40 years was never accompanied by Nelson's syndrome. Basal plasma ACTH values prior to adrenalectomy (30.9 +/- 4.53 pmol/l in the absence of Nelson's syndrome and 31.3 +/- 5.41 pmol/l in patients with Nelson's syndrome) as well as the degree of their suppressibility with 8 mg dexamethane (to 18.8 +/- 3.43 pmol/l in Nelson's syndrome and 19.0 +/- 3.44 pmol/l in patients without it) did not provide sufficient evidence for the prediction of later development of Nelson's syndrome. The same is true of the plasma cortisol concentrations. A tendency to a significant plasma ACTH increase after adrenalectomy within a 6-month period, and especially its lesser suppressibility raises a strong suspicion of an incipient development of Nelson's syndrome. These findings will have to be taken into account when deciding on the surgical treatment of Cushing's disease.

摘要

对一组66例接受双侧肾上腺切除术治疗的库欣病患者的临床和实验室检查结果进行了详细分析,其中15例发生了尼尔森综合征,结果表明,与老年人相比,尼尔森综合征主要影响儿童和年轻患者。40岁以上患者进行双侧肾上腺切除术从未伴有尼尔森综合征。肾上腺切除术之前的基础血浆促肾上腺皮质激素(ACTH)值(无尼尔森综合征者为30.9±4.53 pmol/L,有尼尔森综合征者为31.3±5.41 pmol/L)以及其对8 mg地塞米松的抑制程度(尼尔森综合征患者为18.8±3.43 pmol/L,无尼尔森综合征患者为19.0±3.44 pmol/L)均未提供足够证据来预测尼尔森综合征的后期发生。血浆皮质醇浓度情况亦是如此。肾上腺切除术后6个月内血浆ACTH有显著升高的趋势,尤其是其抑制性较低,这强烈提示尼尔森综合征开始发生。在决定库欣病的手术治疗时,必须考虑这些发现。

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