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Chapitre D'ouvrage Année : 2021

Interstitial lung diseases in the neonatal period

Résumé

Neonatal interstitial lung diseases (ILDs) are rare disorders with a high morbidity and mortality manifesting as severe respiratory insufficiency with frequent PH. The main causes are surfactant disorders, especially disorders of the proteins SP-B, SP-C, ABCA3 and NKX2-1, which can be diagnosed with molecular screening. Other aetiologies are developmental disorders that are more often diagnosed on lung biopsy or autopsy. The most frequent is alveolocapillary dysplasia with misalignment of pulmonary veins, often related to a FOXF1 mutation. The medical management of curable diseases is based mainly on corticosteroids. In some cases, lung transplantation may be discussed. The prognosis relies highly on the rapidity of the management, which should be provided by expert multidisciplinary teams including clinicians, paediatric radiologists, pathologists and geneticists. Genetic counselling should always be provided to the family, even if occurring after a fatal evolution of the disease. Often misdiagnosed and underestimated, neonatal ILD patients should be systematically reported in reference databases for children’s ILD.
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Dates et versions

hal-04557348 , version 1 (26-04-2024)

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Yohan Soreze, Chiara Sileo, Aurore Coulomb l'Hermine, Marie Legendre, Nadia Nathan. Interstitial lung diseases in the neonatal period. Respiratory Diseases of the Newborn Infant, European Respiratory Society, pp.213-230, 2021, ⟨10.1183/2312508X.10014520].⟩. ⟨hal-04557348⟩
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