title: Quality of life and psychological adjustment in children and adolescents with neurofibromatosis type 1.
abstract: OBJECTIVE
  To assess quality of life (QoL) and psychological adjustment in children and adolescents with neurofibromatosis type 1 (NF1).
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  QoL and psychological adjustment are impaired in children and adolescents with NF1. Illness-related variables and the quality of family relationships are important predictors.
authors.affiliation: Division of Psychosomatics and Psychiatry, University Children's Hospital, Steinwiesstrasse 75, CH-8032 Zurich, Switzerland.
authors.forename: Anna
authors.initials: A
authors.lastname: Graf
authors.forename: Markus A
authors.initials: MA
authors.lastname: Landolt
authors.forename: Andrea Capone
authors.initials: AC
authors.lastname: Mori
authors.forename: Eugen
authors.initials: E
authors.lastname: Boltshauser
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D005195:Family Relations; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000523:psychology*; D011788:Quality of Life; D012720:Severity of Illness Index; D012923:Social Class