title: Health-related quality of life, cognitive functioning and behaviour problems in children with Langerhans cell histiocytosis.
abstract: BACKGROUND
  This study was designed to evaluate generic and disease-specific health-related quality of life (HRQoL), cognitive functioning and behaviour problems of children with Langerhans Cell Histiocytosis (LCH). Furthermore, we investigated which medical determinants and social demographic factors were predictive for HRQoL, cognitive functioning and behavioural problems.
  
  
  METHODS
  In this cross-sectional case-control study 24 children ranging from 7 to 17 years of age were administered a HRQoL questionnaire, cognitive tests and behaviour ratings. In addition, a disease-specific HRQoL measure was developed and tested. Results were compared to a reference group consisting of healthy peers and to proxy-ratings by parents and teachers.
  
  
  RESULTS
  Children with LCH reported a lower physical HRQoL than the reference group (P <or= 0.05). Children older than 12 reported lower HRQoL scores. Scores on the disease-specific HRQoL questionnaire were lower than on the generic measure used. Performances on cognitive tests varied widely, short term visual memory was most affected. Twenty-five percent of the children follow special education. According to parents and teachers, children with LCH had more internalising behaviour problems (i.e., anxiety and depression), compared to the instrument norms. Children with Diabetes Insipidus, other CNS involvement and children who have had chemotherapy had more cognitive and behaviour problems than the other children with LCH.
  
  
  CONCLUSIONS
  HRQoL is affected in children with LCH, especially in older children. Children with LCH show more internalising problem behaviour than their peers. Teachers are important additional informants about behaviour problems.
authors.affiliation: Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: C M Jantien
authors.initials: CM
authors.lastname: Vrijmoet-Wiersma
authors.forename: Vicky M
authors.initials: VM
authors.lastname: Kooloos
authors.forename: Hendrik M
authors.initials: HM
authors.lastname: Koopman
authors.forename: Annemarie M
authors.initials: AM
authors.lastname: Kolk
authors.forename: Ingrid
authors.initials: I
authors.lastname: van der Laan
authors.forename: Martha A
authors.initials: MA
authors.lastname: Grootenhuis
authors.forename: R Maarten
authors.initials: RM
authors.lastname: Egeler
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002653:Child Behavior Disorders / Q000209:etiology*; D003072:Cognition Disorders / Q000209:etiology*; D003430:Cross-Sectional Studies; D006646:Histiocytosis, Langerhans-Cell / Q000523:psychology*; D006801:Humans; D011788:Quality of Life / Q000523:psychology*; D012959:Socioeconomic Factors; D011795:Surveys and Questionnaires