Berenbaum Sheri A, Bryk Kristina Korman, Duck Stephen C
Departments of Psychology and Pediatrics, The Pennsylvania State University, University Park, PA 16803, USA.
Int J Pediatr Endocrinol. 2010;2010:853103. doi: 10.1155/2010/853103. Epub 2010 Oct 7.
We provide evidence regarding the nature, causes, and consequences of intelligence in patients with 21-hydroxylase deficient congenital adrenal hyperplasia (CAH). Intelligence and quality of life (psychological adjustment) were measured on multiple occasions from childhood to young adulthood in 104 patients with CAH (62 females, 42 males) and 88 unaffected relatives (31 females, 57 males). Information on disease severity (CAH type, age at diagnosis, genital virilization for girls) and salt-wasting crises was obtained from medical records. There was no evidence of intellectual deficit in either female or male patients with CAH. Intelligence was not significantly associated with psychological adjustment or disease characteristics. CAH itself does not appear to increase risk for poor intellectual function. In a sample of patients with generally good disease control, intelligence is not related to adjustment problems, disease severity, or salt-wasting crises.
我们提供了关于21-羟化酶缺乏型先天性肾上腺皮质增生症（CAH）患者智力的性质、成因及后果的证据。对104例CAH患者（62例女性，42例男性）和88名未受影响的亲属（31例女性，57例男性）从童年到青年期多次测量了智力和生活质量（心理适应情况）。从医疗记录中获取了疾病严重程度（CAH类型、诊断年龄、女孩生殖器男性化情况）和失盐危象的信息。无论是CAH女性患者还是男性患者均没有智力缺陷的证据。智力与心理适应情况或疾病特征无显著关联。CAH本身似乎不会增加智力功能不良的风险。在疾病总体控制良好的患者样本中，智力与适应问题、疾病严重程度或失盐危象无关。
