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[Cystic fibrosis in infants revealed by severe respiratory distress. Mid-term course]
A Sardet1, J Couvreur, J Costil
1Services de Pédiatrie et Pneumologie de l'enfant de Réanimation, Hôpital Trousseau, Paris.
Insights
Early intensive care for infants with cystic fibrosis (CF) presenting with respiratory distress is crucial. This intensive management, not mechanical ventilation, impacts long-term prognosis in children with CF.
Area of Science:
- Pediatric Pulmonology
- Neonatal Care
- Genetic Diseases
Abstract:
We report the follow-up of 13 infants aged 2.5 to 24 months in whom cystic fibrosis disease presented as respiratory distress. All infants received an intensive treatment. Mean clinical, radiological and microbiological follow-up was 3.1 years. Ten children aged 1.5 to 8.5 years are still alive. One of them presents with serious respiratory failure, with a Shwachman score of 50. The Shwachman score is between 85 and 70 for 8 of 13 and 65 for one. The prognosis of this group did not differ from the one usually observed in the disease. It depends partially on early intensive care management and is not altered by mechanical ventilation.