Rev Pneumol Clin
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Bronchopulmonary dysplasia (BPD) is the most common chronic respiratory disease in premature infants. BPD was first described by Northway in 1967 as a chronic respiratory condition that developed in premature infants exposed to mechanical ventilation and high oxygen supplementation. DBP is currently defined by the need for supplemental oxygen at 28 days of life (mild BPD) and at the 36 weeks of post-menstrual age (moderate and severe BPD). ⋯ Regarding its pathophysiological mechanisms, it is now established that BPD is a complex disease combining genetic susceptibility and environmental injuries. The identification of genetic variants involved in BPD is a potential source of innovative development in terms of diagnosis and treatment. Indeed, no curative or effective prophylactic therapeutic exists and BPD treatment is currently symptomatic.
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The improvement over the last two decades in the treatment of cystic fibrosis led to an increase in life expectancy approaching 40 years at birth. Logically, the population of adult patients has been increasing and is currently 50% of patients followed in France. These therapeutic advances have justified the establishment in 2003 of a generalized neonatal screening for cystic fibrosis. ⋯ While treatments available so far goal consequences of cystic fibrosis, a new therapeutic class to correct the functional defect of the mutated protein, called CFTR modulators, is emerging. Ivacaftor, leader of this new class, belonging to the category of "CFTR potentiator" got its access on the market in September 2012 for patients carrying the G551D mutation. New other molecules, named "CFTR correctors" which can have synergistic effect with ivacaftor and concern patients carrying the most common mutation--DF 508--are under development.