دورية أكاديمية
Factors influencing age at diagnosis of primary ciliary dyskinesia in European children.
| العنوان: | Factors influencing age at diagnosis of primary ciliary dyskinesia in European children. |
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| المؤلفون: | Kuehni CE; Institute of Social and Preventive Medicine (ISPM), University of Berne, Berne, Switzerland., Frischer T, Strippoli MP, Maurer E, Bush A, Nielsen KG, Escribano A, Lucas JS, Yiallouros P, Omran H, Eber E, O'Callaghan C, Snijders D, Barbato A |
| مؤلفون مشاركون: | ERS Task Force on Primary Ciliary Dyskinesia in Children |
| المصدر: | The European respiratory journal [Eur Respir J] 2010 Dec; Vol. 36 (6), pp. 1248-58. Date of Electronic Publication: 2010 Jun 07. |
| نوع المنشور: | Journal Article; Research Support, Non-U.S. Gov't |
| اللغة: | English |
| بيانات الدورية: | Publisher: European Respiratory Society Country of Publication: England NLM ID: 8803460 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1399-3003 (Electronic) Linking ISSN: 09031936 NLM ISO Abbreviation: Eur Respir J Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: Sheffield, United Kingdom : European Respiratory Society Original Publication: Copenhagen : Published jointly by the Society and Munksgaard, 1988- |
| مواضيع طبية MeSH: | Kartagener Syndrome/*diagnosis , Situs Inversus/*diagnosis, Adolescent ; Advisory Committees ; Child ; Child, Preschool ; Cross-Sectional Studies ; Europe ; Female ; Health Care Costs ; Humans ; Kartagener Syndrome/economics ; Kartagener Syndrome/epidemiology ; Male ; Mucociliary Clearance ; Situs Inversus/economics ; Situs Inversus/epidemiology |
| مستخلص: | 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% had situs inversus. Median age at diagnosis was 5.3 yrs, lower in children with situs inversus (3.5 versus 5.8 yrs; p < 0.001) and in children treated in large centres (4.1 versus 4.8 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. |
| فهرسة مساهمة: | Investigator: I Azevedo; G Baktai; A Barbato; J de Blic; A Bush; C O'Callaghan; KH Carlsen; L Larisa Dracea; E Eber; S Elborn; A Escribano; T Frischer; E Haarman; B Hesselmar; C Hogg; M Jorissen; B Karadag; CE Kuehni; M Korppi; J Lucas; E Maurer; P Minic; H Mussaffi; KG Nielsen; H Omran; P Pohunek; K Priftis; MA Riikjärv; G Petrova Stoyanova; V Pohanka; D Snijders; MP Strippoli; T Svobodovà; P Yiallouros; Z Zivkovic |
| تواريخ الأحداث: | Date Created: 20100610 Date Completed: 20110330 Latest Revision: 20220410 |
| رمز التحديث: | 20231215 |
| DOI: | 10.1183/09031936.00001010 |
| PMID: | 20530032 |
| قاعدة البيانات: | MEDLINE |
| تدمد: | 1399-3003 |
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| DOI: | 10.1183/09031936.00001010 |