Ayet-Roger Aitana, Joga-Elvira Lorena, Caixàs Assumpta, Corripio Raquel
Clinical and Health Psychology Department, Universitat Autònoma de Barcelona, 08193 Bellaterra, Spain.
Pediatric Neuropediatric Department, Parc Tauli Sabadell Hospital Universitari, 08208 Sabadell, Spain.
J Clin Med. 2022 Mar 14;11(6):1592. doi: 10.3390/jcm11061592.
Prader-Willi Syndrome (PWS) is a genetically based neurodevelopmental disease characterized by obesity, hyperphagia, and mild to moderate intellectual disability. Treatment with growth hormone (GH) could provide cognitive benefits. The objective of the present study was to compare the cognitive and adaptive performance of 31 patients with genetically confirmed PWS grouped in two cohorts, one treated with GH before 2 years old (Group 1) and the other receiving the treatment later (Group 2).
We compared two variables necessary to diagnose intellectual disability: intellectual performance, using the Weschler scales, and adaptive behavior, using the DABS scale. The scores were analyzed by means of non-parametric statistical tests.
Group 1 ( = 10) obtained higher and statistically significant scores in Total Intelligence Quotient (TIQ), General Ability Index (GAI), and General Adaptive Behavior (GAB), implying better cognitive and adaptive performance compared to Group 2.
Treatment with GH should be administered in the early stage of development (before 2 years old) to obtain greater benefits at the cognitive and adaptive levels.
普拉德-威利综合征（PWS）是一种基于基因的神经发育疾病，其特征为肥胖、食欲亢进以及轻度至中度智力障碍。生长激素（GH）治疗可能带来认知方面的益处。本研究的目的是比较31例基因确诊的PWS患者在两个队列中的认知和适应性表现，一个队列在2岁前接受GH治疗（第1组），另一个队列在2岁后接受治疗（第2组）。
我们比较了诊断智力障碍所需的两个变量：使用韦氏智力量表评估智力表现，使用DABS量表评估适应性行为。通过非参数统计检验分析得分。
第1组（n = 10）在总智商（TIQ）、一般能力指数（GAI）和一般适应性行为（GAB）方面获得了更高且具有统计学意义的分数，这意味着与第2组相比，其认知和适应性表现更好。
GH治疗应在发育早期（2岁前）进行，以便在认知和适应水平上获得更大益处。
