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FUNCTIONAL CAPACITY and quality of life IN CHILDREN AND ADOLESCENTS WITH SICkLE CELL ANEMIA
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  • Jonathan Luiz da Silva,
  • Bruno Alvarenga Soares,
  • Jaksoel Silva,
  • Patrícia Belintani Blum,
  • Mariana Mazzuca Reimberg,
  • Simone Dal Corso,
  • Fernanda Lanza
Jonathan Luiz da Silva
Universidade Nove de Julho - Campus Vergueiro
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Bruno Alvarenga Soares
Universidade Federal de Minas Gerais
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Jaksoel Silva
Universidade Nove de Julho - Campus Vergueiro
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Patrícia Belintani Blum
Darcy Vargas Children's Hospital – São Paulo – SP – Brazil
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Mariana Mazzuca Reimberg
Universidade Nove de Julho - Campus Vergueiro
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Simone Dal Corso
Universidade Nove de Julho - Campus Vergueiro
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Fernanda Lanza
Universidade Nove de Julho - Campus Vergueiro
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Abstract

Objective: To evaluate the functional capacity, pulmonary function and quality of life of children and adolescents with sickle cell anemia (SCA) and to test the reproducibility of functional capacity tests in this population. Method: Cross-sectional study with  volunteers with SCA genotype Hb-SS (SCAG), aged 6 to 18 years matched in age and  gender to the control group (CG). Spirometry, 5-repetition sit-to-stand test (5STS-test),  modified shuttle walk test (MSWT), and Pediatric Quality of Life Questionnaire (PedsQL) were performed. The reproducibility of 5STS-test and MSWT was evaluated:   Results: 48 volunteers of SCAG and 48 of CG were evaluated. Pulmonary function of SCAG (FVC: 92 ± 15% pred.; FEV 1 /FVC: 84 ± 8% pred.) was worse than the CG (104 ± 15% pred.; FEV 1 /FVC: 90 ± 6% pred.) p < 0.05. SCAG had worse functional capacity registered by distance walked: 576m (515-672m) and 5STS-test: 8 seconds (7.4-8.9seconds) compared to the CG who showed distance walked: 1010m (887- 1219m) and 5STS-test: 7 seconds (7.0-8.1seconds), p < 0.001. SCAG had worse quality compared to CG, p < 0.05. The reproducibility was good of MSWT (ICC 0.99 (0.98-0.99 IC-95%)) and 5STS-test (ICC 0.80 (0.69 – 0.88), p < 0.001 . Conclusion: Children and adolescents with sickle cell anemia showed worse capacity to walk or run, and to perform sit-to-stand test. Additionally, they have poor quality of life when compared with their control peers. The MSWT and 5STS-test showed reproducible to be applied in pediatric individual with SCA.
24 Jun 2022Submitted to Pediatric Pulmonology
24 Jun 2022Assigned to Editor
24 Jun 2022Submission Checks Completed
25 Jun 2022Reviewer(s) Assigned
30 Aug 2022Review(s) Completed, Editorial Evaluation Pending
02 Sep 2022Editorial Decision: Revise Major