Health-Related Quality of Life in Children with Neurofibromatosis Type 1: Contribution of Demographic Factors, Disease-Related Factors, and Behavior

作者：Krab Lianne C; Oostenbrink R; De Goede Bolder Arja; Aarsen Femke K; Elgersma Ype; Moll Henriette A^*

来源：Journal of Pediatrics, 2009, 154(3): 420-425.

DOI：10.1016/j.jpeds.2008.08.045

Objective To, investigate health-related quality of life (HR-QOL) in children with neurofibromatosis type 1 (NF1) with parental reports and children's self-reports, and to investigate the potential contribution of demographic factors, disease-specific factors, and problems in school performance or behavior.
Study design In a prospective observational study, parents of 58 children with NF1 (32 boys, 26 girls, age 12.2 /- 2.5 years) visiting a university clinic, and their 43 children 10 years or older were assessed with the Child Health Questionnaire (CHQ). Potential determinants of domain scores were assessed in 3 explorative regression models.
Results Parents reported a significant impact of NF1 on 9/13 CHQ scales, with moderate effect sizes on 8 (general health perceptions, physical functioning, general behavior. mental health, self esteem, family activities, role functioning emotional behavioral, and parent emotional impact). Children report an impact on bodily pain, and in above average general behavior. Multiple CHQ scales were sensitive to demographic factors and behavioral problems, and 1 to NF1 severity. NF1 visibility and school problems did not influence HR-QOL.
Conclusions Parents, but not the children with NF1, report a profound impact of NF1 on physical, social, behavioral, and emotional aspects of HR-QOL. Multiple HR-QOL domains were most sensitive to behavioral problems, which points to an exciting potential opportunity to improve HR-QOL in children with NF1 by addressing these behavioral. problems. (J Pediatr 2009;154:420-5)

• 出版日期2009-3