title: Health-related quality of life among children, young people and adults with esophageal atresia: a review of the literature and recommendations for future research.
abstract: OBJECTIVE
  Esophageal atresia (EA) is a rare congenital malformation requisite of surgical treatment. Survival rates have significantly increased in recent decades, but treated children are at risk of chronic morbidity. This study aims to review the literature on health-related quality of life (HRQOL) in patients with EA and describe the questionnaires used.
  
  
  METHODS
  A literature search was conducted in Pubmed, Cinahl and PsycINFO, from inception to January 2015. A meta-analysis of studies investigating HRQOL in patients with EA compared to healthy references was performed. The effect size was calculated as Cohen's d.
  
  
  RESULTS
  Twelve articles (published 1995-2014) describing HRQOL among children, young people and adults were identified. Only European studies were found, and these had variable sample sizes (8-128). Overall HRQOL was reported to be reduced in five of the seven studies that compared overall HRQOL with a healthy reference population. Impaired physical or general health was described in eight articles. In the meta-analysis, eight eligible studies provided 16 estimates of the effect of EA, six of which reached statistical significance for worse HRQOL (p < 0.05). Using Cohen's criteria, the pooled estimate of the effect of EA was small for overall and physical HRQOL, and <0.2 for the mental and social HRQOL components. Altogether, 15 different questionnaires were used, and none were condition specific for EA.
  
  
  CONCLUSIONS
  Different findings are reported; however, this study suggests that patients with EA may have a reduced HRQOL. Moreover, HRQOL is not adequately measured in this group. Additional research is required.
authors.affiliation: Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, 416 85, Gothenburg, Sweden. michaela.m.blom@vgregion.se.
authors.forename: Michaela
authors.initials: M
authors.lastname: Dellenmark-Blom
authors.affiliation: Gothenburg Pediatric Growth Research Center (GPGRC), Department of Pediatrics, Institute of Clinical Sciences, Gothenburg University, 416 85, Gothenburg, Sweden.
authors.forename: John Eric
authors.initials: JE
authors.lastname: Chaplin
authors.affiliation: Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, 416 85, Gothenburg, Sweden.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Gatzinsky
authors.affiliation: Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, 416 85, Gothenburg, Sweden.
authors.forename: Linus
authors.initials: L
authors.lastname: Jönsson
authors.affiliation: Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, 416 85, Gothenburg, Sweden.
authors.forename: Kate
authors.initials: K
authors.lastname: Abrahamson
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004933:Esophageal Atresia / Q000453:epidemiology* / Q000401:mortality; D006801:Humans; D008297:Male; D011788:Quality of Life; D011795:Surveys and Questionnaires
keywords: Congenital malformation; Esophageal atresia; Health-related quality of life; Quality of life; Rare medical health condition; Review
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