LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Neumología Pediátrica. Anexos ❚ 123 3. Know that ion transport defect does not recur in lungs transplanted into patients with cystic fibrosis 4. Know the relationship between ion transport defects in cystic fibrosis and abnormalities in transepithelial voltage 5. Know the sequence of the functional changes in the lungs of patients with cystic fibrosis 6. Know the mechanism of hepatobiliary disease in patients with cystic fibrosis 7. Know the pathogenic features of cystic fibrosis-related diabetes mellitus and that it is distinct from either type 1 or type 2 diabetes 8. Know the mechanism of steatorrhea in patients with cystic fibrosis 9. Know the mechanism of fat-soluble vitamin deficiencies in patients with cystic fibrosis 10. Know the mechanism of hypochloremic alkalosis in infants with cystic fibrosis 11. Know the association between pancreatitis and cystic fibrosis 12. Know the signs and symptoms associated with distal intestinal obstruction syndrome in patients with cystic fibrosis 13. Know the primary bacterial pathogens associated with endobronchial infection in cystic fibrosis and understand the significance of each organism's presence in respiratory tract cultures 14. Understand the potential sources of acquisition of pathogenic bacteria in cystic fibrosis patients, including contact with other cystic fibrosis patients 15. Recognize the role of intercurrent viral respiratory tract infections in the clinical course of cystic fibrosis patients 16. Identify the primary features of inflammation in cystic fibrosis lung disease and the role of infectious pathogens in exacerbating airway inflammation in cystic fibrosis 4. Diagnosis and clinical manifestations a. History 1. Recognize the clinical manifestations of meconium ileus in a newborn infant 2. Recognize the clinical manifestations of distal intestinal obstruction syndrome 3. Recognize the clinical manifestations of cystic fibrosis in a patient with untreated steatorrhea 4. Recognize the clinical manifestations of a typical exacerbation of cystic fibrosis lung disease 5. Recognize the clinical manifestations of deficiencies of the following vitamins: A, D, E, and K 6. Recognize the clinical manifestations of cystic fibrosis-related diabetes 7. Recognize the clinical manifestations of hepatobiliary disease in a patient with cystic fibrosis 8. Recognize the clinical manifestation of pancreatitis in a patient with cystic fibrosis 9. Know that extracellular DNA and F-actin contribute to the viscoelastic properties of airway secretions in cystic fibrosis b. Physical examination 1. Recognize the physical findings typically associated with diffuse bronchiectasis 2. Be able to recognize nasal polyps