A national internet-linked based database for pediatric interstitial lung diseases: the French network.
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.
AP-HP, Hôpital Trousseau, Pediatric Pulmonary Department, Paris, France. nadia.nathan@trs.aphp.fr
Nadia
N
Nathan
Rola Abou
RA
Taam
Ralph
R
Epaud
Christophe
C
Delacourt
Antoine
A
Deschildre
Philippe
P
Reix
Raphaël
R
Chiron
Ulrika
U
de Pontbriand
Jacques
J
Brouard
Michaël
M
Fayon
Jean-Christophe
JC
Dubus
Lisa
L
Giovannini-Chami
François
F
Bremont
Katia
K
Bessaci
Cyril
C
Schweitzer
Marie-Laure
ML
Dalphin
Christophe
C
Marguet
Véronique
V
Houdouin
Françoise
F
Troussier
Anne
A
Sardet
Eglantine
E
Hullo
Isabelle
I
Gibertini
Malika
M
Mahloul
Delphine
D
Michon
Adrien
A
Priouzeau
Laurie
L
Galeron
Jean-François
JF
Vibert
Guillaume
G
Thouvenin
Harriet
H
Corvol
Jacques
J
Deblic
Annick
A
Clement
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D005260:Female; D005602:France; D006078:Government Programs; D006801:Humans; D007223:Infant; D020407:Internet; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D008297:Male; D005791:Patient Care; D035583:Rare Diseases