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Clinical features and management of children with primary ciliary dyskinesia in England

Clinical features and management of children with primary ciliary dyskinesia in England
Clinical features and management of children with primary ciliary dyskinesia in England
Objective In England, the National Health Service commissioned a National Management Service for children with primary ciliary dyskinesia (PCD). The aims of this study were to describe the health of children seen in this Service and compare lung function to children with cystic fibrosis (CF). Design Multi-centre service evaluation of the English National Management PCD Service. Setting Four nationally commissioned PCD centres in England. Patients 333 children with PCD reviewed in the Service in 2015; lung function data were also compared to 2970 children with CF. Results Median age at diagnosis for PCD was 2.6 years, significantly lower in children with situs inversus (1.0 vs 6.0 years, p<0.0001). Compared with national data from the CF Registry, mean (SD) %predicted FEV1 76.8% in PCD (n=240) and 85.0% in CF, and FEV1 was lower in children with PCD up to the age of 15 years. Approximately half of children had some hearing impairment, with 26% requiring hearing aids. Children with a lower BMI had lower FEV1 (p<0.001). One third of children had positive respiratory cultures at review, 54% of these grew Haemophilus influenzae. Conclusions We provide evidence that children with PCD in England have worse lung function than those with CF. Nutritional status should be considered in PCD management, as those with a lower BMI have significantly lower FEV1. Hearing impairment is common but seems to improve with age. Well designed and powered randomised controlled trials on management of PCD are needed to inform best clinical practice.
cystic Fibrosis, lung function, management, primary ciliary dyskinesia
0003-9888
1-6
Rubbo, B.
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Best, S.
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Hirst, R.
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Shoemark, A.
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Goggin, P.
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Carr, S.B.
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Chetcuti, P.
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Hogg, C.
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Kenia, P.
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Lucas, J.S.
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Moya, E.
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Narayanan, M.
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O’Callaghan, C.
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Williamson, M.
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Walker, W.T.
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Rubbo, B.
dc31cd48-3d84-41ab-a8b8-351c9914dca4
Best, S.
4a192d35-d137-417a-a0fb-05e3d83cef2f
Hirst, R.
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Shoemark, A.
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Goggin, P.
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Carr, S.B.
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Chetcuti, P.
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Hogg, C.
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Kenia, P.
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Lucas, J.S.
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Moya, E.
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Narayanan, M.
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O’Callaghan, C.
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Williamson, M.
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Walker, W.T.
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Rubbo, B., Best, S., Hirst, R., Shoemark, A., Goggin, P., Carr, S.B., Chetcuti, P., Hogg, C., Kenia, P., Lucas, J.S., Moya, E., Narayanan, M., O’Callaghan, C., Williamson, M. and Walker, W.T. (2020) Clinical features and management of children with primary ciliary dyskinesia in England. Archives of Disease in Childhood, 105 (8), 1-6. (doi:10.1136/archdischild-2019-317687).

Record type: Article

Abstract

Objective In England, the National Health Service commissioned a National Management Service for children with primary ciliary dyskinesia (PCD). The aims of this study were to describe the health of children seen in this Service and compare lung function to children with cystic fibrosis (CF). Design Multi-centre service evaluation of the English National Management PCD Service. Setting Four nationally commissioned PCD centres in England. Patients 333 children with PCD reviewed in the Service in 2015; lung function data were also compared to 2970 children with CF. Results Median age at diagnosis for PCD was 2.6 years, significantly lower in children with situs inversus (1.0 vs 6.0 years, p<0.0001). Compared with national data from the CF Registry, mean (SD) %predicted FEV1 76.8% in PCD (n=240) and 85.0% in CF, and FEV1 was lower in children with PCD up to the age of 15 years. Approximately half of children had some hearing impairment, with 26% requiring hearing aids. Children with a lower BMI had lower FEV1 (p<0.001). One third of children had positive respiratory cultures at review, 54% of these grew Haemophilus influenzae. Conclusions We provide evidence that children with PCD in England have worse lung function than those with CF. Nutritional status should be considered in PCD management, as those with a lower BMI have significantly lower FEV1. Hearing impairment is common but seems to improve with age. Well designed and powered randomised controlled trials on management of PCD are needed to inform best clinical practice.

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More information

Accepted/In Press date: 3 February 2020
e-pub ahead of print date: 10 March 2020
Keywords: cystic Fibrosis, lung function, management, primary ciliary dyskinesia

Identifiers

Local EPrints ID: 437842
URI: http://eprints.soton.ac.uk/id/eprint/437842
ISSN: 0003-9888
PURE UUID: a028f81c-77c3-48ec-92dd-218ec1e5ae87
ORCID for B. Rubbo: ORCID iD orcid.org/0000-0002-1629-8601

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Date deposited: 19 Feb 2020 17:32
Last modified: 28 Jul 2022 04:01

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Contributors

Author: B. Rubbo ORCID iD
Author: S. Best
Author: R. Hirst
Author: A. Shoemark
Author: P. Goggin
Author: S.B. Carr
Author: P. Chetcuti
Author: C. Hogg
Author: P. Kenia
Author: J.S. Lucas
Author: E. Moya
Author: M. Narayanan
Author: C. O’Callaghan
Author: M. Williamson
Author: W.T. Walker

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