Transcript THIS IS WHY WE ARE IN THE TOP 20!!

Joel Thompson, MSIII
November 13, 2008
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?
CXR
11/11/08
CXR
11/11/08
CXR
10/30/07
CXR
10/30/07
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.
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.