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Application of Adrenal Vein Sampling in Diagnosing Adrenocorticotropic Hormone-independent Cushing's Syndrome with Bilateral Adrenal Masses
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摘要:
目的 研究肾上腺静脉插管取血术在非促肾上腺皮质激素(adrenocorticotropic hormone, ACTH)依赖性库欣综合征合并双侧肾上腺占位患者中的诊断价值。 方法 回顾性分析北京协和医院诊治的4例非ACTH依赖性库欣综合征合并双侧肾上腺占位患者的临床资料。空腹状态下取外周静脉及双侧肾上腺静脉血, 分别测定血浆总皮质醇及醛固酮水平, 分别计算双侧肾上腺静脉与外周静脉血皮质醇的比值及双侧肾上腺静脉的血皮质醇与醛固酮的比值比。 结果 4例经肾上腺静脉插管取血后计算出双侧肾上腺静脉的血皮质醇与醛固酮的比值比平均为8.4(3.6~16.2), 其中3例为单侧分泌皮质醇的高功能肾上腺腺瘤所致的显性库欣综合征, 对侧腺瘤则为无功能皮质腺瘤; 1例为单侧高功能肾上腺腺瘤所致的亚临床库欣综合征, 对侧则为肾上腺结节样增生。定位明确后行单侧肾上腺及腺瘤切除术, 病情均缓解, 平均随访36个月(6~75个月)均无复发。 结论 成功的肾上腺静脉取血可以帮助判断非ACTH依赖性库欣综合征合并双侧肾上腺占位的功能状态, 对指导下一步治疗措施有重要的诊断价值, 而成功的关键在于肾上腺静脉置管到位和排除内源性干扰因素。 -
关键词:
- 双侧肾上腺占位 /
- 非ACTH依赖性库欣综合征 /
- 肾上腺静脉插管取血
Abstract:Objective To investigate the diagnostic value of adrenal vein sampling (AVS) in adrenocorticotropic hormone (ACTH)-independent Cushing's syndrome with bilateral adrenal masses. Methods A retrospective analysis of 4 cases of ACTH-independent Cushing's syndrome with bilateral adrenal masses treated in Peking Union Medical College Hospital was performed. Bilateral adrenal venous blood and peripheral venous blood samples were collected under fasting state for measurement of cortisol and aldosterone concentrations. The ratio of cortisol level in adrenal venous blood to that in peripheral venous blood was calculated, as well as the cortisol-to-aldosterone ratios in bilateral adrenal veins. Results Based on the results of AVS, the average odds ratio of bilateral cortisol/aldosterone of the 4 cases was 8.4 (3.6-16.2). Among these patients, 3 cases were diagnosedas overt Cushing's syndrome caused by unilateral cortisol-secreting hyperfunctioning adrenal adenoma with contralateral nonfunctioning cortical adenoma; the other case was subclinical Cushing's syndrome caused by unilateral cortisol-secreting hyperfunctioning adrenal adenoma with contralateral nodular adrenal hyperplasia. After unilateral adrenalectomy guided by localization diagnosis, all of these 4 patients had complete remission. During a mean follow-up of 36 months, Cushing's syndrome or clinically important cortisol secretory autonomy did not recur. Conclusions AVS can contribute to measure the adrenal function in ACTH-independent Cushing's syndrome with bilateral adrenal masses, and is of important value to guide the decision on treatment. The key factors for successful AVS are catheterization into the adrenal vein and exclusion of endogenous interference factors. -
表 1 4例患者肾上腺静脉取血结果
病例 性别 年龄
(岁)肾上腺静脉取血结果 左肾上腺
静脉血F
(nmol/L)右肾上腺
静脉血F
(nmol/L)外周静脉
血F
(nmol/L)左肾上腺静脉
血F与外周血
F比值右肾上腺静脉
血F与外周血
F比值左肾上腺
静脉血ALD
(pmol/L)右肾上腺
静脉血ALD
(pmol/L)双侧肾上腺静脉
血F/肾上腺静脉
血ALD的比值比1 男 14 792 18 796 761 1.0 24.7 589 862 16.2(右/左) 2 女 35 10 438 4444 574 18.2 7.7 1133 1742 3.6(左/右) 3 女 40 1344 4115 345 3.9 11.9 601 487 3.8(右/左) 4 女 51 7239 1863 420 17.3 4.4 1571 3989 9.9(左/右) F:皮质醇；ALD:醛固醇 
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