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先天性肾上腺增生症患儿的血清3α-雄烷二醇葡萄糖醛酸苷测定

Serum 3 alpha-androstanediol glucuronide measurements in children with congenital adrenal hyperplasia.

作者信息

Hatun S, Yordam N, Calikoğlu A S

机构信息

Division of Pediatric Endocrinology, Dr Sami Ulus Children's Hospital, Turkey.

出版信息

Eur J Endocrinol. 1994 Nov;131(5):504-8. doi: 10.1530/eje.0.1310504.

Abstract

To determine the value of 3 alpha-androstanediol glucuronide (3-AG) measurements in children with congenital adrenal hyperplasia, we compared serum 3AG, 17-hydroxyprogesterone (17-OHP), androstenedione (A), testosterone (T) and dihydrotestosterone (DHT) levels and 24-h urinary 17-ketosteroid (17-KS) excretion in 42 female children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, including 27 with the simple virilizing and 15 with the salt-losing form. Their mean age was 74.5 +/- 48.5 months (range, 6-194 months). Twenty-four-hour urinary 17-KS excretion and serum 3-AG, A, T, DHT and 17-OHP levels were measured in the patients. The values were less than the mean + 2 SD of the control group in 63%, 74%, 67%, 69%, 60% and 31% of the patients, respectively. Serum 3-AG levels correlated with 24-h urinary 17-KS excretion (r = 0.66) and plasma A (r = 0.80), 17-OHP (r = 0.56), T (r = 0.79) and DHT (r = 0.62) levels. We conclude that serum 3-AG is a useful metabolic index in the management of children with congenital adrenal hyperplasia.

摘要

为了确定3α-雄烷二醇葡糖苷酸(3-AG)检测在先天性肾上腺皮质增生症患儿中的价值,我们比较了42例因21-羟化酶缺乏导致先天性肾上腺皮质增生症的女童的血清3-AG、17-羟孕酮(17-OHP)、雄烯二酮(A)、睾酮(T)和双氢睾酮(DHT)水平以及24小时尿17-酮类固醇(17-KS)排泄量,其中包括27例单纯男性化型和15例失盐型。她们的平均年龄为74.5±48.5个月(范围6 - 194个月)。对患者进行了24小时尿17-KS排泄量以及血清3-AG、A、T、DHT和17-OHP水平的检测。患者中上述指标分别有63%、74%、67%、69%、60%和31%低于对照组均值 + 2标准差。血清3-AG水平与24小时尿17-KS排泄量(r = 0.66)以及血浆A(r = 0.80)、17-OHP(r = 0.56)、T(r = 0.79)和DHT(r = 0.62)水平相关。我们得出结论,血清3-AG是先天性肾上腺皮质增生症患儿管理中的一个有用的代谢指标。

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