Cognitive function in children with classic congenital adrenal hyperplasia
Cognitive function in children with classic congenital adrenal hyperplasia
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DOI：
10.1007/s00431-018-3226-7
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发表时间：
2018-11-01
影响因子：
3.6
通讯作者：
Farghaly, Hekma Saad
中科院分区：
文献类型：
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作者：
Hamed, Sherifa Ahmed;Metwalley, Kotb Abbass;Farghaly, Hekma Saad
关于先天性肾上腺皮质增生症（CAH）患者认知功能的研究很少且存在争议。本研究旨在探讨盐耗型CAH儿童的一般智力和特定认知功能及其与人口统计学、临床和实验室变量的关系。本研究纳入了36名患有经典21羟化酶缺乏症SW型CAH的儿童（男性=12;女性=24;平均年龄= 15.62.3岁）。采用韦氏儿童智力量表第3版（WISC-3）和斯坦福大学比奈亚组测验第4版（SBST 4）评定智商和认知功能。与对照组相比，患者的平均全量表（FS）IQ（P=0.01）评分较低，特别是操作IQ评分（P=0.001），以及理解、模式分析、定量、珠记忆和SBST 4句子记忆（分别为P=0.05、P= 0.014、P=0.001、P=0.002和P=0.05）。与接受药物治疗的控制良好的患者相比，在控制不良的患者中观察到较低的智商。FSIQ与年龄呈显著相关（r=-0.810; P=0.001）、疗程（r=-0.887; P=0.01），糖皮质激素剂量（r=-0.463; P=0.01）、17-OHP（r=-0.543; P=0.01）和睾酮（r=-0.462; P=-0.006）水平与低钠血症发作次数（r=-0.350; P=0.05）的相关性。在多变量分析中，低FSIQ的独立风险是糖皮质激素的剂量（OR=1.14; 95%CI =1.08-1.23，P=0.0001），17-OHP水平（OR=2.25; 95%CI =1.19-2.85，P=0.01），低钠血症发作次数（OR=4.34; 95%CI =2.05-5.15，P=0.01）。SW型CAH患者可能有较低的智商和认知缺陷，这可能与糖皮质激素的剂量、雄激素过多和低钠血症发作次数有关。
Studies of cognitive function in patients with congenital adrenal hyperplasia (CAH) are few and controversial. This study aimed to investigate general intelligence and specific cognitive functions in children with salt wasting (SW) form of CAH and their relationship to demographic, clinical, and laboratory variables. This study included 36 children with classic 21 hydroxylase deficiency SW type of CAH (males=12; females=24; mean age=15.62.3years). Intelligence quotient (IQ) and cognition were assessed using Wechsler Intelligence Scale for Children 3rd edition (WISC-III) and Stanford Binet Subsets Test version 4 (SBST4). Compared to controls, patients had lower mean full-scale (FS) IQ (P=0.01) score, particularly performance IQ score (P=0.001), and comprehension, pattern analysis, quantitation, bead memory, and memory for sentences of SBST4 (P=0.05, P=0.014, P=0.001, P=0.002, and P=0.05, respectively). Lower IQ was observed in poorly controlled compared with well-controlled patients on medical treatment. Significant correlations were observed between FSIQ with age (r=-0.810; P=0.001), duration of treatment (r=-0.887; P=0.01), dose of glucocorticoids (r=-0.463; P=0.01), 17-OHP (r=-0.543; P=0.01) and testosterone (r=-0.462; P=-0.006) levels, and number of hyponatremic episodes (r=-0.350; P=0.05). In multivariate analysis, the independent risks of low FSIQ were the dose of glucocorticoids (OR=1.14; 95% CI=1.08-1.23, P=0.0001), 17-OHP levels (OR=2.25; 95% CI=1.19-2.85, P=0.01), and number of hyponatremic episodes (OR=4.34; 95% CI=2.05-5.15, P=0.01).p id=Par Conclusion: Patients with SW form of CAH may have lower IQ and cognitive deficits which may be related to the dose of glucocorticoids, androgen excess, and number of hyponatremic episodes.