{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T21:40:52Z","timestamp":1760823652751,"version":"build-2065373602"},"reference-count":35,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2024,3,8]],"date-time":"2024-03-08T00:00:00Z","timestamp":1709856000000},"content-version":"unspecified","delay-in-days":7,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Cystic Fibrosis Trust Clinical Excellence and Innovation Award","award":["CEA010"],"award-info":[{"award-number":["CEA010"]}]},{"name":"UCL Partners award","award":["Not applicable"],"award-info":[{"award-number":["Not applicable"]}]},{"name":"Sir William Coxen Trust Fund","award":["(http:\/\/opencharities.org\/charities\/206936)"],"award-info":[{"award-number":["(http:\/\/opencharities.org\/charities\/206936)"]}]},{"name":"UCL Rosetrees Stoneygate prize","award":["M712"],"award-info":[{"award-number":["M712"]}]},{"name":"UCL, GOSH and Toronto SickKids studentship","award":["Not applicable"],"award-info":[{"award-number":["Not applicable"]}]},{"name":"CF Trust Youth Activity Unlimited SRC","award":["Not applicable"],"award-info":[{"award-number":["Not applicable"]}]},{"name":"NIHR GOSH BRC internship","award":["Not applicable"],"award-info":[{"award-number":["Not applicable"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Open"],"accepted":{"date-parts":[[2024,2,7]]},"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>The aim was to evaluate whether standardised exercise performance during the incremental shuttle walk test (ISWT) can be used to assess disease severity in children and young people (CYP) with chronic conditions, through (1) identifying the most appropriate paediatric normative reference equation for the ISWT, (2) assessing how well CYP with haemophilia and cystic fibrosis (CF) perform against the values predicted by the best fit reference equation and (3) evaluating the association between standardised ISWT performance and disease severity.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>A cross-sectional analysis was carried out using existing data from two independent studies (2018\u20132019) at paediatric hospitals in London,UK. CYP with haemophilia (n=35) and CF (n=134) aged 5\u201318 years were included. Published reference equations for standardising ISWT were evaluated through a comparison of populations, and Bland-Altman analysis was used to assess the level of agreement between distances predicted by each equation. Associations between ISWT and disease severity were assessed with linear regression.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Three relevant reference equations were identified for the ISWT that standardised performance based on age, sex and body mass index (Vardhan, Lanza, Pinho). A systematic proportional bias of standardised ISWT was observed in all equations, most pronounced with Vardhan and Lanza; the male Pinho equation was identified as most appropriate. On average, CYP with CF and haemophilia performed worse than predicted by the Pihno equation, although the range was wide. Standardised ISWT, and not ISWT distance alone, was significantly associated with forced expiratory volume in 1\u2009s in CYP with CF. Standardised ISWT in CYP with haemophilia was slightly associated with haemophilia joint health score, but this was not significant.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>ISWT performance may be useful in a clinic to identify those with worsening disease, but only when performance is standardised against a healthy reference population. The development of validated global reference equations is necessary for more robust assessment.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/bmjopen-2023-075733","type":"journal-article","created":{"date-parts":[[2024,3,8]],"date-time":"2024-03-08T21:55:29Z","timestamp":1709934929000},"page":"e075733","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Using reference equations to standardise incremental shuttle walk test performance in children and young people with chronic conditions and facilitate the evaluation of exercise capacity and disease severity"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3544-6136","authenticated-orcid":false,"given":"Nicole","family":"Filipow","sequence":"first","affiliation":[{"name":"Physiotherapy, Great Ormond Street Institute of Child Health, University College London, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melanie","family":"Bladen","sequence":"additional","affiliation":[{"name":"Physiotherapy, Great Ormond Street Institute of Child Health, University College London, London, UK"},{"name":"Physiotherapy, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0993-5115","authenticated-orcid":false,"given":"Emma","family":"Raywood","sequence":"additional","affiliation":[{"name":"Physiotherapy, Great Ormond Street Institute of Child Health, University College London, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisabeth","family":"Robinson","sequence":"additional","affiliation":[{"name":"Physiotherapy, Great Ormond Street Institute of Child Health, University College London, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Deepti","family":"Chugh","sequence":"additional","affiliation":[{"name":"Physiotherapy, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helen","family":"Douglas","sequence":"additional","affiliation":[{"name":"Physiotherapy, Great Ormond Street Institute of Child Health, University College London, London, UK"},{"name":"Physiotherapy, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nikki","family":"Thorpe","sequence":"additional","affiliation":[{"name":"Physiotherapy, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rachel","family":"O'Connor","sequence":"additional","affiliation":[{"name":"Paediatric Cystic Fibrosis Centre, Barts Health NHS Trust, The Royal London Hospital, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicky","family":"Murray","sequence":"additional","affiliation":[{"name":"Paediatric Physiotherapy, Royal Brompton Hospital, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9739-3167","authenticated-orcid":false,"given":"Eleanor","family":"Main","sequence":"additional","affiliation":[{"name":"Physiotherapy, Great Ormond Street Institute of Child Health, University College London, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2024,3,8]]},"reference":[{"key":"2025101814074843000_14.3.e075733.1","unstructured":"NICE . Managing long-term conditions in the community. Available: https:\/\/www.nice.org.uk\/about\/what-we-do\/into-practice\/measuring-the-use-of-nice-guidance\/impact-of-our-guidance\/niceimpact-children-and-young-peoples-healthcare\/ch2-managing-long-term-conditions-in-children [Accessed 25 May 2022]."},{"key":"2025101814074843000_14.3.e075733.2","article-title":"Exercise for haemophilia","volume":"12","author":"Strike","year":"2016","journal-title":"Cochrane Database Syst Rev"},{"key":"2025101814074843000_14.3.e075733.3","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1159\/000439057","article-title":"Statement on exercise testing in cystic fibrosis","volume":"90","author":"Hebestreit","year":"2015","journal-title":"Respiration"},{"key":"2025101814074843000_14.3.e075733.4","doi-asserted-by":"crossref","first-page":"S123","DOI":"10.1513\/AnnalsATS.201611-912FR","article-title":"Cardiopulmonary exercise testing in pediatrics","volume":"14","author":"Takken","year":"2017","journal-title":"Ann Am Thorac Soc"},{"key":"2025101814074843000_14.3.e075733.5","unstructured":"Bull FC , Al-Ansari SS , Biddle S , et al . World Health Organization 2020 guidelines on physical activity and sedentary behaviour. Br J Sports Med"},{"key":"2025101814074843000_14.3.e075733.6","unstructured":"World Health Organization . Physical activity. 2022."},{"key":"2025101814074843000_14.3.e075733.7","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ijo.0803774"},{"key":"2025101814074843000_14.3.e075733.8","doi-asserted-by":"publisher","DOI":"10.1136\/thx.47.12.1019"},{"key":"2025101814074843000_14.3.e075733.9","doi-asserted-by":"crossref","DOI":"10.1186\/s40814-021-00831-1","article-title":"Feasibility, safety and acceptability of select outcome measures in a Physiotherapy study protocol for boys with Haemophilia","volume":"7","author":"Thorpe","year":"2021","journal-title":"Pilot Feasibility Stud"},{"key":"2025101814074843000_14.3.e075733.10","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00055513"},{"key":"2025101814074843000_14.3.e075733.11","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1164\/rccm.201806-1110OC","article-title":"Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis","volume":"199","author":"Hebestreit","year":"2019","journal-title":"Am J Respir Crit Care Med"},{"key":"2025101814074843000_14.3.e075733.12","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2007.12.016"},{"key":"2025101814074843000_14.3.e075733.13","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2011.07.023"},{"key":"2025101814074843000_14.3.e075733.14","doi-asserted-by":"crossref","DOI":"10.1136\/bmjopen-2020-039587","article-title":"Protocol for project Fizzyo, an analytic longitudinal observational cohort study of physiotherapy for children and young people with cystic fibrosis, with interrupted time-series design","volume":"10","author":"Raywood","year":"2020","journal-title":"BMJ Open"},{"key":"2025101814074843000_14.3.e075733.15","unstructured":"UK National Haemophilia Database . UKHCDO annual report 2020 & bleeding disorder statistics for the financial year 2019\/2020. 2020. Available: http:\/\/www.ukhcdo.org\/wp-content\/uploads\/2021\/03\/UKHCDO-Annual-Report-2020-2019-20-Data_FINAL.pdf"},{"key":"2025101814074843000_14.3.e075733.16","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1111\/j.1365-2516.2007.01504.x","article-title":"Enhancing lifestyle for individuals with Haemophilia through physical activity and exercise: the role of physiotherapy","volume":"13 Suppl 2","author":"Wittmeier","year":"2007","journal-title":"Haemophilia"},{"key":"2025101814074843000_14.3.e075733.17","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1177\/2040620718784834","article-title":"Recent advances in musculoskeletal physiotherapy for haemophilia","volume":"9","author":"Stephensen","year":"2018","journal-title":"Ther Adv Hematol"},{"key":"2025101814074843000_14.3.e075733.18","doi-asserted-by":"publisher","DOI":"10.1016\/s0140-6736(16)00576-6"},{"key":"2025101814074843000_14.3.e075733.19","first-page":"28","article-title":"Exercise testing in cystic fibrosis: who and why","volume":"27","author":"Urquhart","year":"2018","journal-title":"Paediatr Respir Rev"},{"key":"2025101814074843000_14.3.e075733.20","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1111\/j.1651-2227.2006.tb02378.x","article-title":"WHO child growth standards based on length\/height, weight and age","volume":"95","author":"de Onis","year":"2006","journal-title":"Acta Paediatrica"},{"key":"2025101814074843000_14.3.e075733.21","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1002\/acr.20353","article-title":"Validation of a new pediatric joint scoring system from the international hemophilia prophylaxis study group: validity of the hemophilia joint health score","volume":"63","author":"Feldman","year":"2011","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"2025101814074843000_14.3.e075733.22","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01312.x"},{"key":"2025101814074843000_14.3.e075733.23","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00080312"},{"key":"2025101814074843000_14.3.e075733.24","first-page":"307","article-title":"Measurement in medicine: the analysis of method comparison studies","volume":"32","author":"Altman","year":"1983","journal-title":"The Stat"},{"key":"2025101814074843000_14.3.e075733.25","doi-asserted-by":"crossref","unstructured":"Galton F . Regression towards mediocrity in hereditary stature. 1886.","DOI":"10.2307\/2841583"},{"key":"2025101814074843000_14.3.e075733.26","doi-asserted-by":"crossref","first-page":"478","DOI":"10.22376\/ijpbs.2017.8.4.b478-483","article-title":"Normative values of incremental shuttle walk test in children and adolescents: an observational study","volume":"8","author":"Vardhan","year":"2017","journal-title":"Int J Pharma Bio Sci"},{"key":"2025101814074843000_14.3.e075733.27","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/j.pulmoe.2019.02.009","article-title":"Reference equation for the incremental shuttle walk test in Portuguese children and adolescents","volume":"25","author":"Pinho","year":"2019","journal-title":"Pulmonology"},{"key":"2025101814074843000_14.3.e075733.28","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1016\/j.jpeds.2015.07.068","article-title":"Reference equation for the incremental shuttle walk test in children and adolescents","volume":"167","author":"Lanza","year":"2015","journal-title":"J Pediatr"},{"key":"2025101814074843000_14.3.e075733.29","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12108"},{"key":"2025101814074843000_14.3.e075733.30","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01928.x"},{"key":"2025101814074843000_14.3.e075733.31","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02096.x"},{"key":"2025101814074843000_14.3.e075733.32","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01307.x"},{"key":"2025101814074843000_14.3.e075733.33","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2003.034249"},{"key":"2025101814074843000_14.3.e075733.34","doi-asserted-by":"publisher","DOI":"10.1038\/ijo.2014.136"},{"key":"2025101814074843000_14.3.e075733.35","article-title":"Physical exercise training for cystic fibrosis","volume":"11","author":"Radtke","year":"2017","journal-title":"Cochrane Database Syst Rev"}],"container-title":["BMJ Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bmjopen-2023-075733","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T21:07:55Z","timestamp":1760821675000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmjopen.bmj.com\/lookup\/doi\/10.1136\/bmjopen-2023-075733"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3]]},"references-count":35,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2024,3,8]]},"published-print":{"date-parts":[[2024,3]]}},"alternative-id":["10.1136\/bmjopen-2023-075733"],"URL":"https:\/\/doi.org\/10.1136\/bmjopen-2023-075733","relation":{},"ISSN":["2044-6055","2044-6055"],"issn-type":[{"type":"print","value":"2044-6055"},{"type":"electronic","value":"2044-6055"}],"subject":[],"published":{"date-parts":[[2024,3]]}}}