Graf Anna, Landolt Markus A, Mori Andrea Capone, Boltshauser Eugen
Division of Psychosomatics and Psychiatry, University Children's Hospital, Steinwiesstrasse 75, CH-8032 Zurich, Switzerland.
J Pediatr. 2006 Sep;149(3):348-53. doi: 10.1016/j.jpeds.2006.04.025.
To assess quality of life (QoL) and psychological adjustment in children and adolescents with neurofibromatosis type 1 (NF1).
Forty-six patients with NF1 were investigated between the ages of 7 and 16 years (mean, 11.6 years), with children and parents used as informants. TNO-AZL Questionnaire for Children's Health-Related Quality of Life and Child Behavior Checklist scores were compared with healthy reference groups. Predictive values of sociodemographic variables, illness-related variables, and family-related variables for quality of life and psychological adjustment were assessed.
Most dimensions of QoL in NF1 children and adolescents were different from reference values. Deviations in the NF1 group were an impairment of motor, cognitive, and social functioning and a reduction of positive and negative emotions. Also, psychological adjustment in patients with NF1 was significantly impaired compared with normal subjects. Illness-related variables had a negative impact on the emotional domain of QoL. Good family relationships positively affected both QoL and psychological adjustment.
QoL and psychological adjustment are impaired in children and adolescents with NF1. Illness-related variables and the quality of family relationships are important predictors.
评估1型神经纤维瘤病（NF1）患儿及青少年的生活质量（QoL）和心理调适情况。
对46例年龄在7至16岁（平均11.6岁）的NF1患者进行了调查，以儿童及其父母作为信息提供者。将儿童健康相关生活质量的TNO-AZL问卷和儿童行为清单得分与健康对照组进行比较。评估了社会人口学变量、疾病相关变量和家庭相关变量对生活质量和心理调适的预测价值。
NF1患儿及青少年生活质量的大多数维度与参考值不同。NF1组的差异在于运动、认知和社会功能受损以及积极和消极情绪减少。此外，与正常受试者相比，NF1患者的心理调适明显受损。疾病相关变量对生活质量的情感领域有负面影响。良好的家庭关系对生活质量和心理调适均有积极影响。
NF1患儿及青少年的生活质量和心理调适受损。疾病相关变量和家庭关系质量是重要的预测因素。
