• Anna Graf, Markus A Landolt, Mori Andrea Capone AC, and Eugen Boltshauser.
• Division of Psychosomatics and Psychiatry, University Children's Hospital, Steinwiesstrasse 75, CH-8032 Zurich, Switzerland.
• J. Pediatr. 2006 Sep 1; 149 (3): 348-53.

ObjectiveTo assess quality of life (QoL) and psychological adjustment in children and adolescents with neurofibromatosis type 1 (NF1).Study DesignForty-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.ResultsMost 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.ConclusionsQoL and psychological adjustment are impaired in children and adolescents with NF1. Illness-related variables and the quality of family relationships are important predictors.

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