摘要
目的通过分析不同类型库欣综合征患者尿液中游离皮质醇（UFF）与游离皮质酮（UFE）的比值，反映体内11β羟基类固醇脱氢酶（11β-HSD2）的功能水平，进一步揭示促肾上腺皮质激素（ACTH）非依赖性库欣综合征患者低血钾的发病机制。方法运用液相色谱联合串联质谱分析（LC．MS／MS）方法，对北京协和医院2013年10至12月收治的异位ACTH综合征患者（n=6）、血钾正常肾上腺库欣腺瘤患者（n=6）、低血钾肾上腺库欣腺瘤患者（n=5），以及对照的健康人（n=6）的24h尿液中游离皮质醇（UFF）和皮质酮（UFE）的含量进行测算；对4组研究对象的UFF＋UFE总量以及UFF／UFE比值进行组间比较分析。结果通过LC—MS／MS对4组研究对象UFF以及UFE定量分析得出：异位ACTH综合征患者组UFF＋UFE为（2787±820）μg／L，UFF／UFE为2．98±0．35；血钾正常肾上腺库欣腺瘤患者组UFF＋UFE为（689±163）μ/L，UFF／UFE为1．03．±0．42；低血钾肾上腺库欣腺瘤患者UFF＋UFE为（697±120）μg／L，UFF／UFE为2．24±0．37；健康对照组UFF＋UFE为（70±27）μg／L，UFF／UFE为0．42±0．20；血钾正常肾上腺库欣腺瘤患者和低血钾肾上腺库欣腺瘤患者的UFF＋UFE差异无统计学意义，但低血钾肾上腺库欣腺瘤患者UFF／UFE比值显著高于血钾正常肾上腺库欣腺瘤患者（P〈0．001），提示低血钾。肾上腺库欣腺瘤患者存在11β．14SD2酶功能缺陷。结论部分ACTH非依赖性库欣综合征患者由于自身体内11β—HSD2酶功能缺陷，无法有效地将有活性的皮质醇转化为无活性的皮质酮出现典型低血钾等盐皮质激素过多表现。
Objective To explore the effects of different forms of glucocorticoid excess on cortisol/ cortisone ratio and to reveal the mechanisms of hypokalemia in Cushing＇s syndrome patients. Methods The levels of urinary free cortisol （UFF） and urinary free cortisone （UFE） were determined in 6 adult patients with ectopia adrenoeorticotropie hormone （ACTH） syndrome, 6 normal serum level of potassium adults with hypercortisolism due to adrenal tumor, 5 hypokalemie adults with hypercortisolism due to adrenal tumor and 6 healthy volunteers using high-performance liquid chromatography plus tandem mass spectrometry （LC-MS/ MS）. Results For 4 groups, the results of UFF ± UFE were （ 2 787 ± 820 ）, （ 689 ± 163 ）, （ 697 ±120 ） and （70±27）μg/L and UFF/UFE ratio 2. 98 ±0.35, 1.03 ±0.42, 2. 24 ±0.37 and 0.42 ±0.20 respectively. The values of UFF ± UFE had no statistical difference between normal and low levels of serum potassium adults with hypercortisolism. And the ratio of UFF/UFE of hypokalemic adults with hypercortisolism was significantly higher than that of normal serum level of potassium adults with hypercortisolism. It suggested that the deficiency of 11 β-hydroxysteroid dehydrogenase type 2 （ 11β -HSD2） was present in hypokalemic adults with hypercortisolism. Conclusion Due to a deficiency of 11 β-HSD2, active cortisol can not be transformed into inactive cortisone in some patients with ACTH-independent Cushing＇s syndrome.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2015年第12期929-932,共4页
National Medical Journal of China
