Hiroko Matsumoto1,2, Gerard Marciano3, Gregory Redding4, June Ha3, Scott Luhmann5, Sumeet Garg6, David Roye3, Klane White7. 1. Division of Pediatric Orthopaedic Surgery, Department of Orthopaedic Surgery, Columbia University, New York, NY, USA. hm2174@cumc.columbia.edu. 2. Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, USA. hm2174@cumc.columbia.edu. 3. Division of Pediatric Orthopaedic Surgery, Department of Orthopaedic Surgery, Columbia University, New York, NY, USA. 4. Pulmonary and Sleep Medicine Division, Department of Pediatrics, University of Washington, Seattle, WA, USA. 5. Department of Orthopedic Surgery, Washington University School of Medicine, St. Louis, MO, USA. 6. Division of Orthopaedic Surgery, Children's Hospital of Colorado, Aurora, CO, USA. 7. Department of Orthopedics and Sports Medicine, University of Washington, Seattle, WA, USA.
Abstract
INTRODUCTION: Investigations in associations between subjective health-related quality of life (HRQoL) measures and objective clinical assessments in patients with early-onset scoliosis (EOS) are limited. The purpose of this study is to investigate the association between pulmonary function rated by parents and pulmonary function testing (PFT) in patients with EOS. MATERIALS/ METHODS: In this cross-sectional study, patients with EOS at any stage of treatment from 2011 to 2018 were identified in 2 registries including 33 centers. Parents' perception of pulmonary function was evaluated using pulmonary function (PF) domain in the Early-Onset Scoliosis 24 item Questionnaire (EOSQ-24). PFT measures included FVC% predicted, FEV1/FVC, and TLC% predicted. All PFT predicted values utilized arm span. PFT and EOSQ-24 questionnaire were completed within 180 days of each other with an average day difference of 26 days. RESULTS: 176 patients (mean age: 10.4 years old, female: 56%) were identified. 33% of patients were of congenital/structural etiology, 27% neuromuscular, 26% syndromic, and 14% idiopathic. Wide variance and lower scores of PF domain were reported by parents at lower FVC% predicted values (< 50%). As FVC% predicted values increased, PFD scores increased with simultaneous decreases in variance with few exceptions. CONCLUSION: More variability and frequent lower pulmonary function values are reported by parents when percent forced vital capacity (FVC%) is < 50%. This likely reflects the degree to which children adapt to restrictive lung disease and the limits on adaptation that occur increasingly as lung function falls below 50% predicted. As a direct linear association with high correlation was expected, more research into the character of what the PF domain is measuring is necessary. LEVEL OF EVIDENCE: IV.
INTRODUCTION: Investigations in associations between subjective health-related quality of life (HRQoL) measures and objective clinical assessments in patients with early-onset scoliosis (EOS) are limited. The purpose of this study is to investigate the association between pulmonary function rated by parents and pulmonary function testing (PFT) in patients with EOS. MATERIALS/ METHODS: In this cross-sectional study, patients with EOS at any stage of treatment from 2011 to 2018 were identified in 2 registries including 33 centers. Parents' perception of pulmonary function was evaluated using pulmonary function (PF) domain in the Early-Onset Scoliosis 24 item Questionnaire (EOSQ-24). PFT measures included FVC% predicted, FEV1/FVC, and TLC% predicted. All PFT predicted values utilized arm span. PFT and EOSQ-24 questionnaire were completed within 180 days of each other with an average day difference of 26 days. RESULTS: 176 patients (mean age: 10.4 years old, female: 56%) were identified. 33% of patients were of congenital/structural etiology, 27% neuromuscular, 26% syndromic, and 14% idiopathic. Wide variance and lower scores of PF domain were reported by parents at lower FVC% predicted values (< 50%). As FVC% predicted values increased, PFD scores increased with simultaneous decreases in variance with few exceptions. CONCLUSION: More variability and frequent lower pulmonary function values are reported by parents when percent forced vital capacity (FVC%) is < 50%. This likely reflects the degree to which children adapt to restrictive lung disease and the limits on adaptation that occur increasingly as lung function falls below 50% predicted. As a direct linear association with high correlation was expected, more research into the character of what the PF domain is measuring is necessary. LEVEL OF EVIDENCE: IV.
Entities:
Keywords:
Early-onset scoliosis (EOS); Early-onset scoliosis 24 item questionnaire (EOSQ-24); Forced vitale capacity (FVC); Health related quality of life (HRQoL); Pulmonary function; Pulmonary function testing (PFT)
Authors: Philip H Quanjer; André Capderou; Mumtaz M Mazicioglu; Ashutosh N Aggarwal; Sudip Datta Banik; Stevo Popovic; Francis A K Tayie; Mohammad Golshan; Mary S M Ip; Marc Zelter Journal: Eur Respir J Date: 2014-07-25 Impact factor: 16.671
Authors: Brendan A Williams; Hiroko Matsumoto; Daren J McCalla; Behrooz A Akbarnia; Laurel C Blakemore; Randal R Betz; John M Flynn; Charles E Johnston; Richard E McCarthy; David P Roye; David L Skaggs; John T Smith; Brian D Snyder; Paul D Sponseller; Peter F Sturm; George H Thompson; Muharrem Yazici; Michael G Vitale Journal: J Bone Joint Surg Am Date: 2014-08-20 Impact factor: 5.284