RT Journal Article
SR Electronic
T1 Age at diagnosis in children with primary ciliary dyskinesia (PCD) in Russian Federation (RF)
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p1183
VO 38
IS Suppl 55
A1 Liliya Selimzyanova
A1 Elena Sereda
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p1183.abstract
AB Background: PCD is often diagnosed late.Aim: To identify age at PCD diagnosis in children in RF.Methods: A retrospective study of medical histories in 43 patients with PCD.Results: We followed up19 girls (44%), 24 boys (56%). Situs inversus was revealed in 30 (70%), situs solitus – in 13 (30%) patients. Median age at onset of the first symptoms was 0 (IQR 0-0, range 0 to 4). 20 patients were diagnosed at age 0-4 years, 11 at age 5-9, 10 at age 10-14, 2 at age 15-18. Median age at diagnosis in all patients was 5 yrs (IQR 1-10,2, range 0-16). Median age at diagnosis was lower in children with situs inversus compared to those without (3,5 (IQR 0,7-7, range 0-14) vs 9 yrs (IQR 6-13, range 0,6-16), p<0,005). Before PCD was diagnosed, all the patients had had histories of infectious diseases of upper respiratory tract, due to this the patients had been treated by pediatricians and otolaryngologists. Children usually were directed to pulmonologists late. Lung diseases, which were causes to direct them to pulmonologists were: recurrent bronchitis -in 19 patients (44%), bronchiectasis - in 6 (14%) (in 2 of them- congenital lung malformations, in 2- unknown bronchiectasis, 2 patients were directed to our Centre with suggested cystic fibrosis), chronic bronchitis without bronchiectasis - in 2 children (5%), recurrent or prolonged pneumonias - in 5 (11%), asthma – in 4 (10%), interstitial lung diseases – in 2 patients (5%). Kartagener's syndrome was diagnosed in 5 (11%) patients.Conclusion: PCD in Russian Federation is diagnosed at an average of 5 years. Diagnosis of PCD without situs inversus presents particular difficulties. Children with PCD usually are directed to pulmonologists late.