库兴氏综合征的治疗trilostane(赢得24540),肾上腺类固醇生物合成的抑制剂。

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Komanicky P,射频火花,梅尔JC

库兴氏综合征的治疗trilostane(赢得24540),肾上腺类固醇生物合成的抑制剂。

中国性金属底座。1978年11月,47 (5):1042 - 51。

PubMed ID

233687年(在PubMed

]

文摘

7例库欣综合征患者接受trilostane(赢得24540)4α,5-epoxy-17 beta-hydroxy-3-oxo-5 alpha-androstane-2 alpha-carbonitrile),肾上腺类固醇生物合成的抑制剂。Trilostane治疗类固醇生物合成减少,也改善了疾病的生化表现所有的患者。平均皮质醇分泌率显著降低,治疗,从47.1到23.4毫克/ 24小时(P小于0.005),和泌尿17-hydroxycorticosteroids从15.7下降到8.7毫克/ 24小时(P小于0.01)。尿皮质醇分泌自由从277减少到88微克/ 24小时(P小于0.01),和0800 h血浆皮质醇水平下降从25.0到12.0微克/分升(P小于0.05)。相反,硫酸脱氢表雄酮排泄尿液从1.3增加到5.8毫克/ 24 h (P小于0.0025)和等离子体从162毫克/ 24小时增加(P小于0.025)。血浆和尿自由脱氢表雄酮增加2倍。尿17-ketosteroid排泄从18岁增加到43个毫克/ 24小时(P小于0.001)。tetrahydroaldosterone显著减少尿排泄,tetrahydrodeoxycorticosterone, 18-hydroxytetrahydrodeoxycorticosterone观察与治疗。类固醇生物合成的抑制是伴随着PRA的患病率增加,血清胆固醇水平没有变化。平均动脉血压降低治疗从109到97毫米汞柱(P小于0.005),和空腹血糖下降从117到98 mg / dl (P小于0.005),伴有血浆钾水平上升从3.8到4.3毫当量/升(P小于0.025)。 Two patients on long term therapy also showed an improvement in clinical features of their disease. There were no significant treatment-related carcinoma, simultaneously producing both an excessive amount of cortisol and ACTH, is described. It is concluded that trilostane is an effective inhibitor of 3 beta-hydroxysteroid dehydrogenase enzyme system in human adrenal gland; it inhibits biosynthesis of cortisol and it is useful in the treatment of Cushing's syndrome.

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药物

Trilostane