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通过频繁测量唾液17-羟孕酮监测先天性肾上腺皮质增生症的病情控制情况。

Control in congenital adrenal hyperplasia monitored by frequent saliva 17OH-progesterone measurements.

作者信息

Hughes I A, Read G F

出版信息

Horm Res. 1984;19(2):77-85. doi: 10.1159/000179870.

Abstract

Treatment in a group of 19 patients with congenital adrenal hyperplasia (CAH) has been monitored by frequent, serial measurements of saliva 17OH-progesterone (17OHP) concentrations. Detailed 17OHP profiles were obtained during consecutive weekend days and every 1-2 h over a separate 24-hour period. Patients showed a marked diurnal rhythm in 17OHP levels, particularly when treated with hydrocortisone. In some patients, 10 mg/m2/day of hydrocortisone was sufficient glucocorticoid replacement to produce adequate control, although there was considerable individual variation. Saliva 17OHP profiles provided valuable information on the efficacy of hydrocortisone, cortisone acetate, prednisolone and dexamethasone as glucocorticoid suppressive regimes in the treatment of CAH. Preliminary results suggest that hydrocortisone given in two divided doses during the day, supplemented by a small dose of prednisolone at bedtime, is suitable treatment for CAH patients who are still growing. In the patient who has completed statural growth, single daily dose dexamethasone therapy ensures adequate adrenal suppression and is convenient in the longterm.

摘要

通过频繁、连续测量唾液中17-羟孕酮(17OHP)浓度,对一组19例先天性肾上腺皮质增生症(CAH)患者的治疗情况进行了监测。在连续的周末以及另外一个单独的24小时期间内,每1 - 2小时获取详细的17OHP曲线。患者的17OHP水平呈现明显的昼夜节律,尤其是在接受氢化可的松治疗时。在一些患者中,每天10 mg/m²的氢化可的松剂量足以进行充分的糖皮质激素替代治疗以实现有效控制,尽管个体差异较大。唾液17OHP曲线为氢化可的松、醋酸可的松、泼尼松龙和地塞米松作为糖皮质激素抑制方案治疗CAH的疗效提供了有价值的信息。初步结果表明,对于仍在生长的CAH患者,白天分两次给予氢化可的松,并在睡前补充小剂量泼尼松龙是合适的治疗方法。对于已经完成身高生长的患者,每日单次剂量的地塞米松治疗可确保充分抑制肾上腺,且长期来看较为方便。

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