PT - JOURNAL ARTICLE AU - Alexandra Hardisty AU - Tim Lee TI - The presentation and outcome of chronic pulmonary lobar collapse in children DP - 2014 Sep 01 TA - European Respiratory Journal PG - P3303 VI - 44 IP - Suppl 58 4099 - http://erj.ersjournals.com/content/44/Suppl_58/P3303.short 4100 - http://erj.ersjournals.com/content/44/Suppl_58/P3303.full SO - Eur Respir J2014 Sep 01; 44 AB - Chronic idiopathic pulmonary lobar collapse (CIPLC) is a recognised but under-researched entity in children. Whilst studies acknowledge its existence, there is no data describing the progress and outcome of these patients (Hamad A, Interactive CardioVascular and Thoracic Surgery 15(2012) 618-621.)CIPLC is defined as radiologically confirmed lobar collapse, persisting for 3 months or more, in the absence of identified cause, or other respiratory disease. We studied a cohort of children with the condition, with a view to improving our understanding of the natural history of the disease, and in particular functional outcome.We identified 9 children under current respiratory follow up at Leeds Children's Hospital. Medical notes were reviewed, recording serial lung function and growth, positive sputum microbiology, number of antibiotic courses and symptom progression. Qualitative data was obtained from completion of the Cystic Fibrosis Questionnaire (CFQ-R) and telephone interviews.7 patients had left and 2 patients had right lower lobe collapse. Median age at diagnosis was 5.85 years (range 1.01-9.44). There was no statistical difference between the initial mean FEV1 (64.4) and current mean FEV1 (64.1) (z=-0.67, p<0.05). Antibiotic usage also tended to reduce over time. Interviews identified significant parental anxiety about the diagnosis, but that the overall disease burden moderated with time. Current CFQ-R shows a mean of 80.9 for treatment burden and 78.39 for respiratory symptoms.This is the first study describing progress of chronic idiopathic lobar collapse in children. Whilst acknowledging that it is a major diagnosis for families, it provides evidence for giving an optimistic prognosis.