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评估21-羟化酶缺乏所致先天性肾上腺皮质增生症婴幼儿治疗效果的体格学和生化参数

Auxological and biochemical parameters in assessing treatment of infants and toddlers with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

作者信息

Einaudi S, Lala R, Corrias A, Matarazzo P, Pagliardini S, de Sanctis C

机构信息

Department of Pediatric Endocrinology, Regina Margherita Children's Hospital, Turin, Italy.

出版信息

J Pediatr Endocrinol. 1993 Apr-Jun;6(2):173-8.

PMID:8348221
Abstract

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.

摘要

我们研究了12例接受醋酸可的松治疗的21-羟化酶缺乏症患者在生命最初几年的身高增长速度(HV)、骨龄进展(ΔBA/ΔCA)、尿孕三醇(PT)和血浆17-羟孕酮(17-OH-P)。在病情控制良好的阶段,发现生长速度正常(标准差在-1至+1之间)、骨龄进展令人满意(ΔBA/ΔCA≤1)且无治疗效果不佳的临床迹象;在治疗不足阶段,发现生长速度加快、骨龄进展迅速,在某些情况下还有男性化迹象;在治疗过度阶段,生长速度降低是治疗效果不佳的唯一迹象。激素值与治疗控制的相关性较弱。因此,生长速度评估可能是监测21-羟化酶缺乏症幼儿治疗效果的最佳方法。

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