Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care

Jain, Kapil; Padley, Simon; Goldstraw, Emma; Kidd, Suzanne; Hogg, Claire; Biggart, Elizabeth; Bush, Andrew

doi:10.1016/j.crad.2007.04.015

Cited by 86 publications

(87 citation statements)

References 34 publications

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“…Studies from Pedersen et al [41] and Mygind et al [42] described different symptoms in the same population. Studies from McManus et al [43] and Jain et al [44] had only a small partial overlap and provided mostly unique information.…”

Section: Searchmentioning

confidence: 99%

Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis

et al. 2016

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Section: Searchmentioning

confidence: 99%

Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis

et al. 2016

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“…The group has published widely on its paediatric patients [13][14][15][16][17] but this is their first report on adults. It presents data from 151 patients who had either been transitioned from the paediatric unit or had been diagnosed as adults.…”

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confidence: 99%

Primary ciliary dyskinesia: the patients grow up

et al. 2016

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“…P rimary ciliary dyskinesia (PCD) is a clinically and genetically heterogeneous group of hereditary disorders characterised by abnormal ciliary beat pattern, often with a low beat frequency and mostly, although not always, detectable ciliary ultra-structural abnormalities [1][2][3][4][5][6][7].…”

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confidence: 99%

Factors influencing age at diagnosis of primary ciliary dyskinesia in European children

Kuehni

Frischer

Strippoli³

et al. 2010

Eur Respir J

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308

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Primary ciliary dyskinesia (PCD) is a hereditary disorder of mucociliary clearance causing chronic upper and lower airways disease. We determined the number of patients with diagnosed PCD across Europe, described age at diagnosis and determined risk factors for late diagnosis.Centres treating children with PCD in Europe answered questionnaires and provided anonymous patient lists.In total, 223 centres from 26 countries reported 1,009 patients aged <20 yrs. Reported cases per million children (for 5–14 yr olds) were highest in Cyprus (111), Switzerland (47) and Denmark (46). Overall, 57% were males and 48% hadsitus inversus. Median age at diagnosis was 5.3 yrs, lower in children withsitus inversus(3.5versus5.8 yrs; p<0.001) and in children treated in large centres (4.1versus4.8 yrs; p = 0.002). Adjusted age at diagnosis was 5.0 yrs in Western Europe, 4.8 yrs in the British Isles, 5.5 yrs in Northern Europe, 6.8 yrs in Eastern Europe and 6.5 yrs in Southern Europe (p<0.001). This strongly correlated with general government expenditures on health (p<0.001).This European survey suggests that PCD in children is under-diagnosed and diagnosed late, particularly in countries with low health expenditures. Prospective studies should assess the impact this delay might have on patient prognosis and on health economic costs across Europe.

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Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care

Cited by 86 publications

References 34 publications

Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis

Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis

Primary ciliary dyskinesia: the patients grow up

Factors influencing age at diagnosis of primary ciliary dyskinesia in European children

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