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

Nathan Nadia, Taam Rola, Epaud Ralph, Christophe Delacourt, Deschildre Antoine, Reix Philippe, Chiron Raphaël, De, Brouard Jacques, Fayon Michaël, Dubus Jean-Christophe, Giovannini-Chami Lisa, Bremont François, Bessaci Katia, Schweitzer Cyril, Dalphin Marie-Laure, Marguet Christophe, Houdouin Véronique, Troussier Françoise, Sardet Anne, Hullo Eglantine, Gibertini Isabelle, Mahloul Malika, Michon Delphine, Priouzeau Adrien, Galeron Laurie, Vibert Jean-François, Thouvenin Guillaume, Corvol Harriet, Deblic Jacques, Clement Annick, French

Description

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 .

Sujets

• Interstitial lung disease
• Network
• Epidemiology
• Database

Informations

• Publié par: biomed
• Publié le: 01 janvier 2012
• Nombre de lectures: 104
• Langue: English

Extrait

Nathanet al. Orphanet Journal of Rare Diseases2012,7:40 http://www.ojrd.com/content/7/1/40

R E S E A R C HOpen Access A national internetlinked based database for pediatric interstitial lung diseases: the French network 1,2* 3,45 3,46 7 Nadia Nathan, Rola Abou Taam, Ralph Epaud , Christophe Delacourt, Antoine Deschildre , Philippe Reix , 8 910 1112 Raphaël Chiron , Ulrika de Pontbriand , Jacques Brouard, Michaël Fayon, JeanChristophe Dubus, 13 1415 1617 Lisa GiovanniniChami, François Bremont, Katia Bessaci, Cyril Schweitzer, MarieLaure Dalphin, 18 1920 2122 Christophe Marguet, Véronique Houdouin, Françoise Troussier, Anne Sardet, Eglantine Hullo, 23 241,24 241,2 Isabelle Gibertini, Malika Mahloul, Delphine Michon, Adrien Priouzeau, Laurie Galeron, 24 1,21,2 3,41,2 JeanFrançois Vibert, Guillaume Thouvenin, Harriet Corvol, Jacques deBlic, Annick Clementand French W RespiRare Group

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 W (RespiRare ),we created a national network and a weblinked database to collect data on pediatric ILD. W Methods:Since 2008, the database has been set up in all RespiRarecentres. 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. Keywords:Interstitial lung disease, Network, Epidemiology, Database

* Correspondence: [e-mail] 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 Full list of author information is available at the end of the article

© 2012 Nathan et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.