Your browser doesn't support javascript.
loading
Health-Related Quality of Life in Children and Young Adults with Marfan Syndrome.
Handisides, Jill C; Hollenbeck-Pringle, Danielle; Uzark, Karen; Trachtenberg, Felicia L; Pemberton, Victoria L; Atz, Teresa W; Bradley, Timothy J; Cappella, Elizabeth; De Nobele, Sylvia; Groh, Georgeann Keh-Teng; Hamstra, Michelle S; Korsin, Rosalind; Levine, Jami C; Lindauer, Bergen; Liou, Aimee; Neal, Meghan K Mac; Markham, Larry W; Morrison, Tonia; Mussatto, Kathleen A; Olson, Aaron K; Pierpont, Mary Ella M; Pyeritz, Reed E; Radojewski, Elizabeth A; Roman, Mary J; Xu, Mingfen; Lacro, Ronald V.
Afiliação
  • Handisides JC; Boston Children's Hospital, Harvard Medical School, Boston, MA.
  • Hollenbeck-Pringle D; New England Research Institutes Inc., Watertown, MA.
  • Uzark K; C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI.
  • Trachtenberg FL; New England Research Institutes Inc., Watertown, MA.
  • Pemberton VL; National Heart, Lung, and Blood Institute, Bethesda, MD.
  • Atz TW; Medical University of South Carolina, Charleston, SC.
  • Bradley TJ; The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
  • Cappella E; Ann and Robert H. Lurie Children's Hospital, Chicago, IL.
  • De Nobele S; Ghent University Hospital, Ghent, Belgium.
  • Groh GK; Washington University School of Medicine, St. Louis, MO.
  • Hamstra MS; Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
  • Korsin R; Children's Hospital of New York, New York, NY.
  • Levine JC; Boston Children's Hospital, Harvard Medical School, Boston, MA.
  • Lindauer B; Primary Children's Hospital, University of Utah, Salt Lake City, UT.
  • Liou A; Texas Children's Hospital, Houston, TX.
  • Neal MKM; Icahn School of Medicine at Mount Sinai, New York, NY.
  • Markham LW; The Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN.
  • Morrison T; Children's Hospital of Philadelphia, Philadelphia, PA.
  • Mussatto KA; Children's Hospital of Wisconsin, Milwaukee, WI.
  • Olson AK; Seattle Children's Hospital, Seattle, WA.
  • Pierpont MEM; Children's Hospital and Clinic of Minnesota, St. Paul, MN.
  • Pyeritz RE; The Perlman School of Medicine, University of Pennsylvania, Philadelphia, PA.
  • Radojewski EA; The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
  • Roman MJ; Weill Cornell Medical Center, New York, NY.
  • Xu M; Duke University School of Medicine, Durham, NC.
  • Lacro RV; Boston Children's Hospital, Harvard Medical School, Boston, MA. Electronic address: ron.lacro@cardio.chboston.org.
J Pediatr ; 204: 250-255.e1, 2019 01.
Article em En | MEDLINE | ID: mdl-30270167
OBJECTIVE: To assess health-related quality of life (HRQOL) in a large multicenter cohort of children and young adults with Marfan syndrome participating in the Pediatric Heart Network Marfan Trial. STUDY DESIGN: The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales were administered to 321 subjects with Marfan syndrome (5-25 years). PedsQL scores were compared with healthy population norms. The impact of treatment arm (atenolol vs losartan), severity of clinical features, and number of patient-reported symptoms on HRQOL was assessed by general linear models. RESULTS: Mean PedsQL scores in children (5-18 years) with Marfan syndrome were lower than healthy population norms for physical (P ≤ .003) and psychosocial (P < .001) domains; mean psychosocial scores for adults (19-25 years) were greater than healthy norms (P < .001). HRQOL across multiple domains correlated inversely with frequency of patient-reported symptoms (r = 0.30-0.38, P < .0001). Those <18 years of age with neurodevelopmental disorders (mainly learning disability, attention-deficit/hyperactivity disorder) had lower mean PedsQL scores (5.5-7.4 lower, P < .04). A multivariable model found age, sex, patient-reported symptoms, and neurodevelopmental disorder to be independent predictors of HRQOL. There were no differences in HRQOL scores by treatment arm, aortic root z score, number of skeletal features, or presence of ectopia lentis. CONCLUSIONS: Children and adolescents with Marfan syndrome were at high risk for impaired HRQOL. Patient-reported symptoms and neurodevelopmental disorder, but not treatment arm or severity of Marfan syndrome-related physical findings, were associated with lower HRQOL.
Assuntos
Palavras-chave
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Qualidade de Vida / Atenolol / Losartan / Síndrome de Marfan / Anti-Hipertensivos Tipo de estudo: Clinical_trials / Etiology_studies / Prognostic_studies / Risk_factors_studies Aspecto: Patient_preference Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: J Pediatr Ano de publicação: 2019 Tipo de documento: Article País de publicação: Estados Unidos
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Qualidade de Vida / Atenolol / Losartan / Síndrome de Marfan / Anti-Hipertensivos Tipo de estudo: Clinical_trials / Etiology_studies / Prognostic_studies / Risk_factors_studies Aspecto: Patient_preference Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: J Pediatr Ano de publicação: 2019 Tipo de documento: Article País de publicação: Estados Unidos