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先天性肾上腺皮质增生症患儿的学习障碍

Learning disabilities in children with congenital adrenal hyperplasia.

作者信息

Nass R, Baker S

机构信息

Department of Pediatrics, New York Hospital Cornell University Medical Center, NY.

出版信息

J Child Neurol. 1991 Oct;6(4):306-12. doi: 10.1177/088307389100600404.

DOI:10.1177/088307389100600404
PMID:1940131
Abstract

Early exposure to androgens may be a cause of the higher incidence of learning disabilities among males. To test this hypothesis, we determined the frequency of learning disabilities, as measured by extent of Wechsler Verbal-Performance IQ discrepancy, among female patients with congenital adrenal hyperplasia, who are exposed in utero to excess androgens for their sex. A significantly larger Verbal-Performance IQ discrepancy (10.1 vs 7.7) was found for the 18 female patients than for 27 unaffected female siblings (P = .045, one-tailed). The Verbal-Performance IQ discrepancy of the females with congenital adrenal hyperplasia is in the male range (10.4 to 13.4), similar to males with congenital adrenal hyperplasia (n = 20) and unaffected male siblings (n = 31). In addition to the sex effect, there is an effect of the type of congenital adrenal hyperplasia on the frequency of learning disabilities. Simple virilizers are more likely to be learning disabled than salt-wasters (P = .04, one-tailed). However, salt-waster patients have a lower IQ (104 vs 117) than simple virilizer patients (P = .005, one-tailed). Presumably, this is because some salt-waster patients suffer brain injury from episodes of hypotension and hyponatremia. The diffuse insult to the central nervous system may overide the androgen effect and result in a general cognitive impairment. Because of this confounding effect on IQ in the salt-waster form of congenital adrenal hyperplasia, the simple virilizer female versus unaffected female siblings reprsents the best test of the hypothesis.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

早期接触雄激素可能是男性学习障碍发病率较高的一个原因。为了验证这一假设,我们测定了先天性肾上腺皮质增生症女性患者中学习障碍的发生率,该发生率通过韦氏言语-操作智商差异程度来衡量,这些女性患者在子宫内就因自身性别而接触到过量雄激素。结果发现,18名先天性肾上腺皮质增生症女性患者的言语-操作智商差异(10.1)显著大于27名未受影响的女性同胞(7.7)(P = 0.045,单尾检验)。先天性肾上腺皮质增生症女性的言语-操作智商差异处于男性范围内(10.4至13.4),与先天性肾上腺皮质增生症男性患者(n = 20)和未受影响的男性同胞(n = 31)相似。除了性别影响外,先天性肾上腺皮质增生症的类型对学习障碍的发生率也有影响。单纯男性化型患者比失盐型患者更易出现学习障碍(P = 0.04,单尾检验)。然而,失盐型患者的智商(104)低于单纯男性化型患者(117)(P = 0.005,单尾检验)。据推测,这是因为一些失盐型患者因低血压和低钠血症发作而遭受脑损伤。对中枢神经系统的弥漫性损害可能会掩盖雄激素的作用,导致一般性认知障碍。由于失盐型先天性肾上腺皮质增生症对智商有这种混杂效应,单纯男性化型女性与未受影响的女性同胞的比较是对该假设的最佳检验。(摘要截短至250字)

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