Clinical features

 

  1. The clinical features and clinical course of cystic fibrosis (CF) are variable, even within families who carry the same mutations.
  2. Clinical features and their prevalence in CF patients include:
  3. Chronic suppurative lung disease, 95%
  4. Pancreatic exocrine insufficiency, leading to malabsorption, 85%
  5. Sweat gland salt loss, 100%
  6. Male infertility (absent or altered vas deferens), 99%
  7. Meconium Ileus, 20%
  8. Distal intestinal obstruction syndrome, 20%
  9. CF-related diabetes, 20%
  10. CF liver disease, 20%
  11. Nasal polyps, 10%
  12. Some clinical features of CF seem to be mediated by the specific mutations in the CFTR gene that an individual inherits.
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