RT Journal Article
SR Electronic
T1 Interstitial lung diseases in children in a tertiary hospital in North Italy
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P1246
VO 42
IS Suppl 57
A1 Deborah Snijders
A1 Serena Calgaro
A1 Ilaria Bertozzi
A1 Silvia Quartesan
A1 Ivana Kozuh
A1 Francesca Lunardi
A1 Angelo Barbato
YR 2013
UL http://erj.ersjournals.com/content/42/Suppl_57/P1246.abstract
AB Interstitial lung diseases (ILDs) in children are an heterogeneous group of lung disease with very different pathophysiology. In most European countries rare orphan diseases such as pediatric interstitial lung diseases (ILDs) are still underdiagnosed.Objective: To investigate/ describe pediatric ILDs in a tertiary university hospital in North Italy.Methods: we included 18 children admitted to the Pediatric Department of the Padova University Hospital from 2003 until 2012 with a diagnosis of ILD. Children were identified retrospectively through clinical charts and clinical, radiological, functional, pathological, biological and genetic data were collected.Results: Data were available for x children presenting with ILD.Median age at diagnosis was 5.5 years [1-16 years], and the sex ratio was 0.5 male/female.Radiologic, laboratory histology and genetics led to the following diagnosis: Bronchiolitis obliterans organizing pneumonia (BOOP), surfactant protein B mutation and viral induced interstitial lung disease were the most common diagnoses. At high resolution CT-scan, 14/18 children showed in 16 % a Honey comb pattern, in 16 %, a reticular picture, in 16% a nodular picture, and in 52% ground glass opacity.Chronic cough and wheezing episode were the most common symptoms reported.Conclusion: In Italy, pediatric ILD is still under estimated. A national database, such as the German, UK or France registry, and national guidelines for diagnosis and possible therapies are necessary to allow physicians and patients to have a more adequate diagnosis and follow-up.