LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Neumología Pediátrica. Anexos ❚ 125 7. Recognize that the incidence of cystic fibrosis-related diabetes increases with increasing age in patients with cystic fibrosis 8. Recognize that cor pulmonale is a complication of advanced cystic fibrosis lung disease 9. Recognize the complications associated with advanced pulmonary disease in patients with cystic fibrosis 10. Know that massive hemoptysis in a patient with cystic fibrosis usually originates from the bronchial circulation 5. Prevention and therapeutic approach a. Prevention 1. Know that prenatal diagnosis of cystic fibrosis is possible 2. Be able to interpret CFTR genetic testing results from prospective parents who are screened prior to conception b. Therapeutic approach 1. Know the treatment of nasal polyps in patients with cystic fibrosis 2. Know the treatments for distal intestinal obstruction syndrome in patients with cystic fibrosis 3. Know the treatments for massive pulmonary hemorrhage in patients with cystic fibrosis 4. Know the treatment options for pneumothorax in patients with cystic fibrosis 5. Know the positive and negative effects of bronchodilators on pulmonary function in patients with cystic fibrosis 6. Know that altered pharmacokinetics occur in patients with cystic fibrosis 7. Know the therapeutic approach for the patient with cystic fibrosis who is exposed to varicella 8. Know the association between pancreatic enzyme replacement therapy and colonic strictures 9. Know the treatment of acute pulmonary exacerbation in cystic fibrosis 10. Know the indications for the use of DNase in cystic fibrosis 11. Know the approach to treatment of initial colonization with Pseudomonas aeruginosa in an infant with cystic fibrosis 12. Know the indications for the use of inhaled tobramycin in cystic fibrosis 13. Know the indications for the use of inhaled hypertonic saline in cystic fibrosis 6. Prognosis a. Natural history b. Prognosis with therapy 1. Know the prognosis of cystic fibrosis with current therapy F. Interstitial lung disease 1. Epidemiology 2. Etiology/genetics a. Know that bronchiolitis obliterans can follow a variety of insults, including pulmonary infections, inhalation of toxic gases, and graft-versus-host disease b. Know which disease entities are associated with lymphoid interstitial pneumonia c. Know which medications cause interstitial lung disease d. Know that radiation exposure can cause interstitial fibrosis e. Know which collagen vascular diseases produce interstitial lung disease