Clinical features and management of children with primary ciliary dyskinesia in England.
cystic Fibrosis
lung function
management
primary ciliary dyskinesia
Journal
Archives of disease in childhood
ISSN: 1468-2044
Titre abrégé: Arch Dis Child
Pays: England
ID NLM: 0372434
Informations de publication
Date de publication:
08 2020
08 2020
Historique:
received:
14
06
2019
revised:
26
11
2019
accepted:
03
02
2020
pubmed:
12
3
2020
medline:
21
10
2020
entrez:
12
3
2020
Statut:
ppublish
Résumé
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). Multi-centre service evaluation of the English National Management PCD Service. Four nationally commissioned PCD centres in England. 333 children with PCD reviewed in the Service in 2015; lung function data were also compared with 2970 children with CF. Median age at diagnosis for PCD was 2.6 years, significantly lower in children with situs inversus (1.0 vs 6.0 years, p<0.001). Compared with national data from the CF Registry, mean (SD) %predicted forced expiratory volume in one second (FEV 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 FEV
Identifiants
pubmed: 32156696
pii: archdischild-2019-317687
doi: 10.1136/archdischild-2019-317687
doi:
Types de publication
Comparative Study
Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
724-729Investigateurs
Amanda Harris
(A)
Amanda Friend
(A)
Victoria Keenan
(V)
Hannah Wilkins
(H)
Hasnaa Ismail-Koch
(H)
Andrea Burgess
(A)
Samantha Packham
(S)
Patricia Goggin
(P)
Gemma Marsh
(G)
Laura Baynton
(L)
Michael Leshen
(M)
Sarah Ollosson
(S)
Nicola Collins
(N)
Peter Rea
(P)
Ann-Louise McDermott
(AL)
Amanda Parsons
(A)
Rajinder Kang
(R)
Nicola Lloyd
(N)
Alison Truscott
(A)
Lynne Schofield
(L)
L Knight
(L)
Informations de copyright
© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.
Déclaration de conflit d'intérêts
Competing interests: JL reports grants, personal fees and non-financial support from Aerocrine/ Circassia, grants and personal fees from Vertex, grants from Parion, outside the submitted work. SC reports grants, personal fees and non-financial support from Vertex Pharmaceuticals, grants and other from Chiesi Pharmaceuticals, other from Pharmaxis Pharmaceuticals, personal fees from Actavis Pharmaceuticals, other from Profile Pharma, outside the submitted work.