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本文报告用高效液相色谱法同时分离和测定人血清中皮质素(E)、皮质醇(F)、皮质酮、11-去氧皮质醇、雄烯二酮+11-去氧皮质酮(A+DOC)、17α-羟孕酮和孕酮等8种主要类固醇激素的方法以及用本方法测得的6例先天性肾上腺增生症(CAH)病人血清类固醇激素谱。单纯男性化型21-羟化酶缺乏症的典型变化为F和E显著减低,17α-羟孕酮和A+DOC显著增高,21-羟化酶指数显著增高,色谱图上出现阶梯式“三连峰群”;17α-羟化酶缺乏症的典型变化为F和E显著减低,皮质酮显著增高,17α-羟化酶指数显著增高。初步临床观察显示,本方法在CAH的诊断、分型、治疗监测及变异型研究等方面有较高的实用价值。
This paper reports the simultaneous separation and determination of human serum cortisol (E), cortisol (F), corticosterone, 11-deoxycortisol, androstenedione + 11-deoxycorticosterone by high performance liquid chromatography + DOC), 17α-hydroxyprogesterone and progesterone, as well as 6 patients with congenital adrenal hyperplasia (CAH) measured by this method serum steroid hormone profile. Typical changes in simple masculinic 21-hydroxylase deficiency were a marked decrease in F and E, a significant increase in 17α-hydroxyprogesterone and A + DOC, a significant increase in 21-hydroxylase index and a stepped “tris Lianfeng group ”. The typical changes of 17α-hydroxylase deficiency were marked decrease of F and E, significant increase of corticosterone, and significant increase of 17α-hydroxylase index. Preliminary clinical observation shows that this method has high practical value in the diagnosis, classification, treatment monitoring and mutation study of CAH.