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A national internet-linked based database for pediatric interstitial lung diseases: the French network.

Nadia Nathan 1, 2, * Rola Taam 3 Ralph Epaud 4 Christophe Delacourt 3 Antoine Deschildre 5 Philippe Reix 6 Raphaël Chiron 7 Ulrika de Pontbriand 8 Jacques Brouard 9 Michaël Fayon 10 Jean-Christophe Dubus 11 Lisa Giovannini-Chami 12 François Bremont 13 Katia Bessaci 14 Cyril Schweitzer 15 Marie-Laure Dalphin 16 Christophe Marguet 17 Véronique Houdouin 18 Françoise Troussier 19 Anne Sardet 20 Eglantine Hullo 21 Isabelle Gibertini 22 Malika Mahloul 23 Delphine Michon 1, 23 Adrien Priouzeau 23 Laurie Galeron 1, 2 Jean-François Vibert 23 Guillaume Thouvenin 1, 2 Harriet Corvol 1, 2 Jacques Deblic 3 Annick Clément 1, 2
Abstract : ABSTRACT: 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(R)), 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(R) 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]. CONCLUSION: 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.
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Submitted on : Wednesday, September 26, 2012 - 5:01:31 PM
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Nadia Nathan, Rola Taam, Ralph Epaud, Christophe Delacourt, Antoine Deschildre, et al.. A national internet-linked based database for pediatric interstitial lung diseases: the French network.. Orphanet Journal of Rare Diseases, BioMed Central, 2012, 7 (1), pp.40. ⟨10.1186/1750-1172-7-40⟩. ⟨inserm-00735780⟩

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