Research
A national internet-linked based database for pediatric interstitial lung diseases: the French network
1 AP-HP, Hôpital Trousseau, Pediatric Pulmonary Department, Paris, F-75012, France
2 Université Pierre et Marie Curie-Paris6, Inserm, UMR S-U938, Paris, F-75012, France
3 Pediatric Pulmonary Department, AP-HP, Hôpital Necker Enfants Malades, Paris, F-75015, France
4 Université Paris Descartes-Paris5, Paris, F-75005, France
5 Centre Hospitalier Intercommunal de Créteil, Pediatric Department, Inserm U955, Université Paris Est, Créteil, France
6 Pediatric Department, Centre Hospitalier Universitaire de Lille, Lille, France
7 Pediatric Pulmonary Department, Centre Hospitalier Universitaire de Lyon, Lyon, France
8 Pediatric Pulmonary Department, Centre Hospitalier Universitaire de Montpellier, Montpellier, France
9 Pediatric Pulmonary Department, Centre Hospitalier Universitaire de Nantes, Nantes, France
10 Pediatric Department, Centre Hospitalier Universitaire de Caen, Caen, France
11 Pediatric Department, Centre Hospitalier Universitaire de Bordeaux, Département de Pédiatrie, Centre d'Investigation Clinique (CIC 0005), F-33000, Bordeaux, France
12 Pediatric Pulmonary Department, Centre Hospitalier Universitaire de Marseille, Marseille, France
13 Pediatric Department, Centre Hospitalier Universitaire de Nice, Nice, France
14 Pediatric Pulmonary Department, Centre Hospitalier Universitaire de Toulouse, Toulouse, France
15 Centre Hospitalier Universitaire de Reims, Reims, France
16 Pediatric Department, Centre Hospitalier Universitaire de Nancy, Nancy, France
17 Pediatric Department, Centre Hospitalier Universitaire de Besançon, Besançon, France
18 Pediatric Department, Centre Hospitalier Universitaire de Rouen, Rouen, France
19 Pediatric Pulmonary Department, Hôpital Robert Debré, Paris, F-75019, France
20 Pediatric Department, Centre Hospitalier Universitaire d’Angers, Angers, France
21 Pediatric Department, Centre Hospitalier de Lens, Lens, France
22 Pediatric Department, Centre Hospitalier Universitaire de Grenoble, Grenoble, France
23 Pediatric Department, Centre Hospitalier Universitaire de Tours, Tours, France
24 Inserm UMR S-707, Paris, F-75012, France
Orphanet Journal of Rare Diseases 2012, 7:40 doi:10.1186/1750-1172-7-40
Published: 15 June 2012Abstract
Background
Interstitial lung diseases (ILDs) in children represent a heterogeneous group of rare respiratory disorders that affect the lung parenchyma. After the launch of the French Reference Centre for Rare Lung Diseases (RespiRare®), we created a national network and a web-linked database to collect data on pediatric ILD.
Methods
Since 2008, the database has been set up in all RespiRare® centres. After patient's parents' oral consent is obtained, physicians enter the data of children with ILD: identity, social data and environmental data; specific aetiological diagnosis of the ILD if known, genetics, patient visits to the centre, and all medical examinations and tests done for the diagnosis and/or during follow up. Each participating centre has a free access to his own patients' data only, and cross-centre studies require mutual agreement. Physicians may use the system as a daily aid for patient care through a web-linked medical file, backed on this database.
Results
Data was collected for 205 cases of ILD. The M/F sex ratio was 0.9. Median age at diagnosis was 1.5 years old [0–16.9]. A specific aetiology was identified in 149 (72.7%) patients while 56 (27.3%) cases remain undiagnosed. Surfactant deficiencies and alveolar proteinosis, haemosiderosis, and sarcoidosis represent almost half of the diagnoses. Median length of follow-up is 2.9 years [0–17.2].
Conclusions
We introduce here the French network and the largest national database in pediatric ILDs. The diagnosis spectrum and the estimated incidence are consistent with other European databases. An important challenge will be to reduce the proportion of unclassified ILDs by a standardized diagnosis work-up. This database is a great opportunity to improve patient care and disease pathogenesis knowledge. A European network including physicians and European foundations is now emerging with the initial aim of devising a simplified European database/register as a first step to larger European studies.
