BackgroundInterstitial 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.MethodsSince 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.ResultsData 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].ConclusionsWe 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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Our data do not support biological screening at the time of a TIVAD insertion. We emphasise the contribution of a medical history of venous thromboembolism and prospective Doppler-US for identifying asymptomatic catheter venous thrombosis to select patients who may benefit from biological screening and possible anticoagulant therapy.

Objective: To delineate the perinatal risk factors of neurodevelopmental disabilities in very preterm birth applying logistic regression analysis. Design: This prospective, geographically defined collaborative study was carried out in the Franche-Comté region of France. Subjects: From October 1, 1990 to September 30, 1992, perinatal and follow-up data were collected on 203 consecutive live-born singleton or twin non-malformed infants with strictly ascertained gestational ages of less than 33 weeks. Main Outcome Measure: The rate of cerebral palsy and/or severe mental retardation as diagnosed by a family physician or pediatrician with a screening-skill test performed at 2 years of age. Results: 167/171 (98%) survivors were evaluated. Twenty-two of the 167 examined infants (13%) showed signs of cerebral palsy, and 10 of these had severe cerebral palsy or mental retardation. Risk factors for disabilities were selected by a multivariate approach: premature rupture of membranes ≥48 h (OR 4.3, 95% CI 1.6–11.8); monochorionic twin placentation (OR 6.0, 95% CI 1.7–21.3), and respiratory distress syndrome (OR 2.8, 95% CI 1.1–7.1). Conclusion: This geographically defined prospective study gives epidemiological data and highly suggests that there is a link between prenatal events (premature rupture of membranes, monochorionic twin placentation), postnatal events (respiratory distress syndrome), and neurological disabilities in former preterm infants.

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