LET

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LETS PLAY NORMAL OR ABNORMAL!

• Joel Thompson, MSIII
• November 13, 2008

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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?

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CXR 11/11/08
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CXR 11/11/08
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CXR 10/30/07
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CXR 10/30/07
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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.
  aeruginosachronic 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.
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Role of radiography

• Lung disease is the most common cause of
  CF-related morbidity and mortalitydiagnosis,
  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
• CTcan 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.