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Description
| - Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, resulting in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility. The diagnosis of PCD requires presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by electron microscopy or evidence of abnormal ciliary function. Although the management of children with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage. This ERS consensus statement on the management of children with PCD formulates recommendations for diagnostic and therapeutic approaches with more accurate approach in these patients. Large well-designed randomised controlled trials are required to improve these recommendations.
- Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, resulting in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility. The diagnosis of PCD requires presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by electron microscopy or evidence of abnormal ciliary function. Although the management of children with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage. This ERS consensus statement on the management of children with PCD formulates recommendations for diagnostic and therapeutic approaches with more accurate approach in these patients. Large well-designed randomised controlled trials are required to improve these recommendations. (en)
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Title
| - Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children
- Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children (en)
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skos:prefLabel
| - Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children
- Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children (en)
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skos:notation
| - RIV/00216208:11130/09:5728!RIV10-MZ0-11130___
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http://linked.open...avai/riv/aktivita
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http://linked.open...avai/riv/aktivity
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http://linked.open...iv/cisloPeriodika
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http://linked.open...vai/riv/dodaniDat
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http://linked.open...aciTvurceVysledku
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http://linked.open.../riv/druhVysledku
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http://linked.open...iv/duvernostUdaju
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http://linked.open...titaPredkladatele
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http://linked.open...dnocenehoVysledku
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http://linked.open...ai/riv/idVysledku
| - RIV/00216208:11130/09:5728
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http://linked.open...riv/jazykVysledku
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http://linked.open.../riv/klicovaSlova
| - Consensus statement; diagnosis; primary ciliary dyskinesia; treatment; linked retinitis-pigmentosa; congenital heart-disease; recombinant human dnase; situs-inversus totalis; outer dynein arm; kartageners-syndrome; cystic-fibrosis; mucociliary clearance; immotile-cilia; lung transplantation (en)
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http://linked.open.../riv/klicoveSlovo
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http://linked.open...odStatuVydavatele
| - GB - Spojené království Velké Británie a Severního Irska
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http://linked.open...ontrolniKodProRIV
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http://linked.open...i/riv/nazevZdroje
| - European Respiratory Journal
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http://linked.open...in/vavai/riv/obor
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http://linked.open...ichTvurcuVysledku
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http://linked.open...cetTvurcuVysledku
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http://linked.open...UplatneniVysledku
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http://linked.open...v/svazekPeriodika
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http://linked.open...iv/tvurceVysledku
| - Barbato, A.
- Bush, A.
- Pohunek, Petr
- Frischer, T.
- Eber, E.
- Escribano, A.
- Kuehni, CE
- Snijders, D.
- Strippoli, MPF
- Azevedo, I.
- Baktai, G.
- Haarman, E.
- Hesselmar, B.
- Hogg, C.
- Jorissen, M.
- Lucas, J.
- Nielsen, KG
- Omran, H.
- Bartoloni, L.
- OCallaghan, C.
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http://linked.open...ain/vavai/riv/wos
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http://linked.open...n/vavai/riv/zamer
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issn
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number of pages
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http://localhost/t...ganizacniJednotka
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