| Autor: |
Michael Williamson, Manjith Narayanan, Sunayna Best, Siobhán B. Carr, Priti Kenia, Claire Hogg, Robert A. Hirst, Bruna Rubbo, Florian Gahleitner, Eduardo Moya, Christopher O'Callaghan, Philip Chetcuti, Woolf T. Walker, Jane S. Lucas |
| Rok vydání: |
2018 |
| Předmět: |
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| Zdroj: |
Paediatric respiratory epidemiology. |
| Popis: |
Introduction: Primary ciliary dyskinesia (PCD) is a rare condition where impaired mucociliary clearance leads to recurrent sinopulmonary infection & bronchiectasis. A National Children9s PCD management service was commissioned in England in 2012 across 4 Centres. The aim of this study was to describe the characteristics of all children in England within this new service. Methods: Detailed diagnostic, phenotypic & management data were collected at annual review assessments for all children seen in 2014. Results: 334 children underwent annual review. Median age at review was 9.8yrs. Median age at diagnosis was 2.6yrs, however was significantly lower in patients with dextrocardia (1.0yr vs 6.2yrs, p Conclusion We provide evidence that, far from being a mild respiratory condition, children with PCD have worse lung function than those with CF. This highlights the need for intensive management of PCD by a multi-professional specialist team. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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