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正常儿童及青春期前先天性肾上腺皮质增生症患者体内的17-羟孕酮、雄烯二酮和睾酮

17-hydroxyprogesterone, androstenedione, and testosterone in normal children and in prepubertal patients with congenital adrenal hyperplasia.

作者信息

von Schnakenburg K, Bidlingmaier F, Knorr D

出版信息

Eur J Pediatr. 1980 May;133(3):259-67. doi: 10.1007/BF00496086.

Abstract

To determine maximal plasma levels of androstenedione (A) and testosterone (T) which still can be considered non virilizing in 21-hydroxylase deficiency, we investigated plasma levels of these steroids in unaffected children and in adults. For T we found an upper limit of the prepubertal normal range of 16 ng/dl in girls and of 20 ng/dl in boys, with the exception of boys in the first half-year of life in which T is elevated up to the low adult range with peak values in the 2nd and 3rd month. During puberty T values show a significant difference between pubic hair stage 1 and stage 2. T levels below 20 ng/dl can be considered to be non virilizing. For A we found a plasma concentration of 86 ng/dl to be the upper normal level in both sexes before the onset of puberty. A values below this limit are expected to be non virilizing. To evaluate the usefulness of 17-hydroxyprogesterone (OHP) for the prediction of A and T and to define "acceptable" OHP levels in CAH we performed simultaneous determinations of OHP, T, and A in prepubertal patients treated for CAH. From these values we calculated the 95% confidence interval for prediction of T and A on known OHP levels. On an OHP value of 1.000 ng/dl, T can be expected to be between 6 and 60 ng/dl and A between 25 and 320 ng/dl. Because of these wide ranges, OHP has to be considered an unreliable parameter for predicting androgen levels in CAH.

摘要

为了确定在21-羟化酶缺乏症中仍可被视为无男性化作用的雄烯二酮(A)和睾酮(T)的最大血浆水平,我们研究了未受影响儿童和成人中这些类固醇的血浆水平。对于T,我们发现女孩青春期前正常范围的上限为16 ng/dl,男孩为20 ng/dl,但一岁以内的男孩除外,在此期间T升高至低成人范围,在第2和第3个月达到峰值。在青春期,T值在阴毛1期和2期之间存在显著差异。T水平低于20 ng/dl可被视为无男性化作用。对于A,我们发现青春期开始前男女的血浆浓度正常上限均为86 ng/dl。低于此限值的A值预计无男性化作用。为了评估17-羟孕酮(OHP)对预测A和T的有用性,并确定先天性肾上腺皮质增生症(CAH)中“可接受”的OHP水平,我们对接受CAH治疗的青春期前患者同时测定了OHP、T和A。根据这些值,我们计算了已知OHP水平下预测T和A的95%置信区间。当OHP值为1000 ng/dl时,预计T在6至60 ng/dl之间,A在25至320 ng/dl之间。由于范围较宽,OHP必须被视为预测CAH中雄激素水平的不可靠参数。

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