A national internet-linked based database for pediatric interstitial lung diseases: the French network. - Archive ouverte HAL Access content directly
Journal Articles Orphanet Journal of Rare Diseases Year : 2012

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

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Ralph Epaud
  • Function : Author
  • PersonId : 930573
Jacques Brouard
  • Function : Author
  • PersonId : 906137
Jean-Christophe Dubus
  • Function : Author
  • PersonId : 930579
Lisa Giovannini-Chami
  • Function : Author
  • PersonId : 930580
François Bremont
  • Function : Author
  • PersonId : 930581
Katia Bessaci
  • Function : Author
  • PersonId : 930582
Marie-Laure Dalphin
  • Function : Author
  • PersonId : 930584
Véronique Houdouin
  • Function : Author
  • PersonId : 930586
Anne Sardet
  • Function : Author
  • PersonId : 930588
Isabelle Gibertini
  • Function : Author
  • PersonId : 930590

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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Dates and versions

inserm-00735780 , version 1 (26-09-2012)

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Nadia Nathan, Rola Abou 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, 2012, 7 (1), pp.40. ⟨10.1186/1750-1172-7-40⟩. ⟨inserm-00735780⟩
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