Management of Cystic fibrosis
Cystic fibrosis is the commonest of all inherited disorders (1 per 2500 live
births). Clinical features include:
• family history
• presents in infancy
• meconium ileus in the neonate
• recurrent chest infections (cough and wheeze)
• failure to thrive
• malabsorption
Diagnosis: can be diagnosed antenatally (in utero) neonatal screening--
CFTR protein
Treatment: oral pancreatic enzyme replacement for malabsorption
attention to respiratory problems
Cystic fibrosis is the commonest of all inherited disorders (1 per 2500 live
births). Clinical features include:
• family history
• presents in infancy
• meconium ileus in the neonate
• recurrent chest infections (cough and wheeze)
• failure to thrive
• malabsorption
Diagnosis: can be diagnosed antenatally (in utero) neonatal screening--
CFTR protein
Treatment: oral pancreatic enzyme replacement for malabsorption
attention to respiratory problems