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Lung function and nutritional status in children with cystic fibrosis and primary ciliary dyskinesia

Woolf Walker, Amanda Harris, Bruna Rubbo, Victoria Keenan, Amanda Friend, Sarah Payne, Judi Maddison, Caroline Yonge, Catherine Crocker, Tricia McGinnity, Teresa Curbishley, Kerry Gove, Sian Phillips, Julian Legg, Hazel Evans, Jane Lucas, Gary Connett
European Respiratory Journal 2016 48: PA3128; DOI: 10.1183/13993003.congress-2016.PA3128
Woolf Walker
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2Clincal and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
3NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Amanda Harris
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
3NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Bruna Rubbo
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2Clincal and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
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Victoria Keenan
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Amanda Friend
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Sarah Payne
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Judi Maddison
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Caroline Yonge
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Catherine Crocker
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Tricia McGinnity
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Teresa Curbishley
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Kerry Gove
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2Clincal and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
3NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Sian Phillips
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Julian Legg
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2Clincal and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
3NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Hazel Evans
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2Clincal and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
3NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Jane Lucas
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2Clincal and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
3NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Gary Connett
1Primary Ciliary Dykinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2Clincal and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom
3NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
4Children's Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
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Abstract

Introduction: Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) both cause recurrent pulmonary infections resulting in bronchiectasis. CF is considered the more severe condition. Nutritional status is fundamental to outcome in CF but there is little evidence about its importance in PCD.

Aim: To compare BMI and lung function of pancreatic sufficient (PS) and insufficient (PI) CF children to those with PCD.

Methods: Cross sectional data (2015) from 204 CF (189 PI, 15 PS) and 46 PCD children, obtained at annual review at our Paediatric Respiratory Centre were compared.

Results: Age, sex and mean BMI centile were comparable across the three groups. Mean age (SD) at diagnosis was significantly older in the PCD compared to the PI-CF cohort (4.8yrs (4.6) vs 0.6yrs (1.3) P<0.001). While FEV1% did not vary significantly with BMI centile in PS-CF, a lower BMI centile was associated with a lower FEV1% in both PI-CF and PCD cohorts, even when adjusted by age. FEV1 declined similarly in the PS-CF and PCD groups with increasing age although the rate of decline was greater in the PI-CF cohort. Of note, the mean (SD) FEV1% was significantly lower in the PCD cohort than both PI-CF and PS-CF children (73.8% (19.4), 84.9% (21.2), 96.1% (15.9) P<0.05 respectively).

Conclusion: Similar to PI-CF, a lower BMI centile was associated with a lower FEV1% in our PCD cohort. Whilst a greater decline in FEV1% with increasing age was observed in the PI-CF patients, the mean FEV1% in the PCD patients was significantly lower and they were significantly older at diagnosis. Optimising nutritional status and earlier diagnosis might be of importance in improving lung function amongst PCD patients.

  • Spirometry
  • Epidemiology
  • Copyright ©the authors 2016
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Lung function and nutritional status in children with cystic fibrosis and primary ciliary dyskinesia
Woolf Walker, Amanda Harris, Bruna Rubbo, Victoria Keenan, Amanda Friend, Sarah Payne, Judi Maddison, Caroline Yonge, Catherine Crocker, Tricia McGinnity, Teresa Curbishley, Kerry Gove, Sian Phillips, Julian Legg, Hazel Evans, Jane Lucas, Gary Connett
European Respiratory Journal Sep 2016, 48 (suppl 60) PA3128; DOI: 10.1183/13993003.congress-2016.PA3128

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Lung function and nutritional status in children with cystic fibrosis and primary ciliary dyskinesia
Woolf Walker, Amanda Harris, Bruna Rubbo, Victoria Keenan, Amanda Friend, Sarah Payne, Judi Maddison, Caroline Yonge, Catherine Crocker, Tricia McGinnity, Teresa Curbishley, Kerry Gove, Sian Phillips, Julian Legg, Hazel Evans, Jane Lucas, Gary Connett
European Respiratory Journal Sep 2016, 48 (suppl 60) PA3128; DOI: 10.1183/13993003.congress-2016.PA3128
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More in this TOC Section

  • Measuring lung function in asthmatic children: A spirometry and forced oscillation technique (FOT) comparison
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