title: Quality of life in caregivers of children and adolescents with Osteogenesis Imperfecta.
abstract: BACKGROUND
  Osteogenesis imperfecta (OI) is a group of genetic disorders of collagen biosynthesis, characterized by low bone density leading to fractures. Most patients exhibit functional impairment and require the aid of a caregiver. The aim of this study is to assess the quality of life (QoL) of caregivers of patients with OI.
  
  
  METHODS
  In this cross-sectional study, a convenience sampling strategy was used to enroll adult caregivers of children and adolescents with OI who attended a referral center in southern Brazil. The WHOQOL-BREF instrument was used to assess QoL.
  
  
  RESULTS
  Twenty-four caregivers of 27 patients (10 with type I, 4 with type III, and 13 with type IV OI) were included in the study. Eighteen caregivers were the patients' mothers, two had OI, and 22 cared for only one patient. Mean WHOQOL-BREF scores were 14.59 for the physical health domain, 13.80 for the psychological domain, 15.19 for the social relationships domain, and 12.87 for the environmental domain; the mean total QoL score was 14.16. QoL scores did not differ significantly according to patients' OI type or number of fractures. Economic status was not correlated significantly with QoL scores.
  
  
  CONCLUSIONS
  QoL appears to be impaired in caregivers of patients with OI. Additional studies are required to confirm these findings and to ascertain which factors account for this phenomenon.
authors.affiliation: Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil. avanz@hcpa.edu.br.|Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. avanz@hcpa.edu.br.
authors.forename: Ana Paula
authors.initials: AP
authors.lastname: Vanz
authors.affiliation: Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. tfelix@hcpa.edu.br.
authors.forename: Têmis M
authors.initials: TM
authors.lastname: Félix
authors.affiliation: Postgraduate Program in Medical Sciences: Psychiatry, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil. neusa-rocha@via-rs.net.
authors.forename: Neusa Sica
authors.initials: NS
authors.lastname: da Rocha
authors.affiliation: Postgraduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil. ischwartz@hcpa.edu.br.|Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. ischwartz@hcpa.edu.br.|Department of Genetics, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil. ischwartz@hcpa.edu.br.
authors.forename: Ida V D
authors.initials: IV
authors.lastname: Schwartz
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D001938:Brazil; D017028:Caregivers / Q000523:psychology*; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D010013:Osteogenesis Imperfecta / Q000451:nursing* / Q000523:psychology*; D010287:Parent-Child Relations; D011788:Quality of Life / Q000523:psychology*