21-羟化酶缺乏症对婴幼儿先天性肾上腺增生的生理生化参数评价。

S Einaudi, R Lala, A Corrias, P Matarazzo, S Pagliardini, C de Sanctis
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引用次数: 0

摘要

我们研究了12例接受醋酸可的松治疗的21-羟化酶缺乏症患者第一年的身高速度(HV)、骨龄进展(δ BA/ δ CA)、尿妊娠三醇(PT)和血浆17-羟基孕酮(17-OH-P)。在控制良好的阶段,生长率正常(SDS在-1 ~ +1之间),骨龄进展令人满意(δ BA/ δ CA <或= 1),未发现治疗不良的临床症状;在治疗不足阶段,生长速度加快,骨龄进展迅速,在某些情况下,发现了阳刚化的迹象;在过度处理阶段,生长率降低是处理不良的唯一标志。激素值与治疗控制的相关性较弱。因此,生长速率评估可以代表非常年轻的21-羟化酶缺乏症患者监测治疗的最佳方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Auxological and biochemical parameters in assessing treatment of infants and toddlers with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

We studied height velocity (HV), bone age progression (delta BA/delta CA), urinary pregnanetriol (PT) and plasma 17-hydroxyprogesterone (17-OH-P) during the first years of life in 12 patients with 21-hydroxylase deficiency, treated by cortisone acetate. In the well-controlled phases normal growth rate (SDS between -1 and +1), satisfactory bone age progression (delta BA/delta CA < or = 1) and no clinical sign of poor treatment were found; in the undertreatment phases enhanced growth rate, rapid bone age progression and, in some instances, signs of virilization were found; in the overtreatment phases, reduced growth rate was the only sign of poor treatment. Hormonal values were only weakly correlated to therapeutic control. Therefore, growth rate evaluation can represent the best method of monitoring treatment in very young patients with 21-hydroxylase deficiency.

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