ABSTRACT

Congenital hypothyroidism (CH) is an important cause of preventable intellectual disability. Implementation of CH neonatal screening programs leading to early treatment has improved cognitive outcome. However, more subtle cognitive impairments are still reported, and there is lack of clarity regarding factors that impact long-term cognitive outcome. Research to better understand these factors can lead to further improvements in the cognitive prognosis for these patients. The current study aimed to evaluate the cognitive performance of adolescents who were early-treated for primary permanent CH and possible associated variables. Neurocognitive evaluation was carried out in 66 adolescents, 11 to 16 years old: 34 with CH and 29 paired controls. Intellectual quotient (IQ), verbal fluency, processing speed, executive functions, and memory were investigated. CH patients and control subjects were comparable regarding sex, age, schooling, family’s socioeconomic status and caregiver’s educational level. Both groups presented not only similar IQ scores but also equivalent performances regarding Perceptual Reasoning, Working Memory and Processing Speed index scores. Patients presenting different CH etiologies (dysgenesis and dyshormonogenesis) showed similar cognitive performance. Socioeconomic aspects along with the initial levothyroxine dose were the main variables to positively influence the cognitive performance, the family’s socioeconomic status having the strongest association with patients’ cognitive skills.

摘要

先天性甲状腺功能减退症 (CH) 是可预防的智力障碍的重要原因。实施导致早期治疗的 CH 新生儿筛查计划改善了认知结果。然而，仍报告了更细微的认知障碍，并且对影响长期认知结果的因素缺乏明确性。更好地了解这些因素的研究可以进一步改善这些患者的认知预后。目前的研究旨在评估早期接受原发性永久性 CH 治疗的青少年的认知能力和可能的相关变量。对 66 名 11 至 16 岁的青少年进行了神经认知评估：34 名患有 CH，29 名配对对照。智商 (IQ)、语言流畅度、处理速度、执行功能、和记忆被调查。CH 患者和对照受试者在性别、年龄、受教育程度、家庭社会经济地位和照顾者的教育水平方面具有可比性。两组不仅表现出相似的 IQ 分数，而且在感知推理、工作记忆和处理速度指数分数方面的表现也相当。呈现不同 CH 病因（发育障碍和激素异常）的患者表现出相似的认知表现。社会经济方面以及左旋甲状腺素的初始剂量是对认知表现产生积极影响的主要变量，家庭的社会经济地位与患者的认知技能有最强的关联。两组不仅表现出相似的 IQ 分数，而且在感知推理、工作记忆和处理速度指数分数方面的表现也相当。呈现不同 CH 病因（发育障碍和激素异常）的患者表现出相似的认知表现。社会经济方面以及左旋甲状腺素的初始剂量是对认知表现产生积极影响的主要变量，家庭的社会经济地位与患者的认知技能有最强的关联。两组不仅表现出相似的 IQ 分数，而且在感知推理、工作记忆和处理速度指数分数方面的表现也相当。呈现不同 CH 病因（发育障碍和激素异常）的患者表现出相似的认知表现。社会经济方面以及左旋甲状腺素的初始剂量是对认知表现产生积极影响的主要变量，家庭的社会经济地位与患者的认知技能有最强的关联。