Impaired cognitive function in women with congenital adrenal hyperplasia

Impaired cognitive function in women with congenital adrenal hyperplasia
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DOI:
10.1210/jc.2005-1959
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发表时间:
2006-04-01
影响因子:
5.8
通讯作者:
Main, KM
Main, KM
中科院分区:
医学2区
文献类型:
--
作者:
Johannsen, TH;Ripa, CPL;Main, KM

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内容:先天性肾上腺皮质增生症(CAH)是一种严重程度广泛的疾病。目的:本研究的目的是调查CAH妇女的认知功能。设计:这是一项病例对照研究。设置:本研究在哥本哈根大学医院的儿科内分泌学三级中心进行。参与者:35名丹麦CAH妇女(年龄17 - 51岁),参与率为84%。通过丹麦民事登记系统招募对照组妇女,并在年龄和教育程度上进行匹配。主要结果测量:使用韦氏成人智力量表的缩写形式,即全量表智商IQ; 11个分测验中的5个),其中包括6个言语IQ分测验中的3个和5个操作IQ分测验中的2个。与对照组相比,CAH患者的平均全量表智商显著降低(84.5 vs. 99.1; P < 0.001),平均言语智商(86.6 vs. 97.3; P < 0.001),平均操作智商(85.7 vs. 101.3; P < 0.001)。盐耗型CAH组的智商低于单纯男性化CAH组,且在平均总智商上差异有显著性(81.2 vs. 92.8,P = 0.04)和平均语言智商(84.7 vs. 95.5,P = 0.05),此外,得分低于晚发型CAH组,这在操作智商方面达到了显著性结论:CAH患者存在认知功能损害,尤其是盐耗性CAH患者。这些有趣的发现可能反映了低钠血症发作的不良影响,次优的产后激素替代治疗或产前肾上腺雄激素过多,以及该疾病的潜在心理社会后果。
Context: Congenital adrenal hyperplasia (CAH) is a disorder with a wide spectrum of severity.Objective: The objective of this study was to investigate cognitive function in CAH women.Design: This was a case-control study.Setting: This study was conducted at a tertiary center for pediatric endocrinology at the University Hospital of Copenhagen.Participants: Thirty-five Danish CAH women ( age 17 - 51 yr) were included, and participation rate was 84%. Control women were recruited through the Danish Civil Registration System and matched on age and education.Main Outcome Measures: An abbreviated form of the Wechsler Adult Intelligence Scale was used, i.e. full-scale intelligence quotient IQ; five of 11 subtests), which included three of six verbal IQ subtests and two of five performance IQ subtests.Results: A significantly lower IQ was found in CAH patients compared with controls with respect to mean full-scale IQ ( 84.5 vs. 99.1; P < 0.001), mean verbal IQ (86.6 vs. 97.3; P < 0.001), and mean performance IQ (85.7 vs. 101.3; P < 0.001). The salt-wasting CAH group had lower IQ scores than the simple-virilizing CAH group, which reached significance for mean total IQ (81.2 vs. 92.8, P = 0.04) and mean verbal IQ (84.7 vs. 95.5, P = 0.05), and additionally, lower scores than the late-onset CAH group, which reached significance for performance IQ ( mean 81.5 vs. 96.2, P = 0.02).Conclusions: Impaired cognitive function was observed in patients with CAH, especially in salt-wasting CAH. These intriguing findings may reflect adverse effects of hyponatremic episodes, suboptimal postnatal hormone replacement therapy or prenatal adrenal androgen excess, and the potential psychosocial consequences of the disorder.