1. Joel Thompson, MSIII November 13, 2008

2. And Now, Your History… 18 yo F with “Cough increased known *pulmonary disease* patient” What primary chronic pulmonary diseases commonly affect the pediatric population? What chronic pulmonary diseases with clinically significant extra-pulmonary manifestations commonly affect the pediatric population?

3. CXR 11/11/08

4. CXR 11/11/08

5. CXR 10/30/07

6. CXR 10/30/07

7. And The Diagnosis… Cystic Fibrosis Mutation to CFTR on chromosome 17 that results in malfunction to varying degrees Results in thick mucus secretions that tend to obstruct hollow secretory structures (bile and pancreatic ducts, airways, bowel, sinuses, vas deferens/fallopian tubes, etc.) Can lead to chronic bronchitis-type pulmonary disease, chronic rhinosinusitis, chronic pancreatitis/pancreatic failure, meconium ileus/SBO, biliary cirrhosis/cholelithiasis, infertility, poor bone mineralization, HPOA Airways become colonized by bacteria, most commonly Staph aureus, H. influenzae, and P. aeruginosa—chronic lung infections that are frequently resistant to treatment Due to both genetic resistance and difficulty of delivering sufficient concentrations of antibiotics through thick mucus Katkin, Julie P. “Clinical manifestations and diagnosis of cystic fibrosis.” Uptodate.com. Accessed November 12, 2008.

8. Role of radiography Lung disease is the most common cause of CF-related morbidity and mortality—diagnosis, recognition, and follow-up for progression are paramount! Findings on CXR include hyperinflation (earliest sign), prominent bronchovascular markings (upper lobes first), peribronchial cuffing (bronchiectasis), cyst formation (late), kyphosis (late), flattened diaphragm (late), PTX (late) In advanced stages, CXR may not correlate with clinical changes CT—can define extent of bronchiectasis (if focal, may be resectable) Katkin, Julie P. “Clinical manifestations of pulmonary disease in cystic fibrosis.” Uptodate.com. Accessed November 12, 2008.