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Cystic Fibrosis

General Considerations

  • Disease of abnormal exocrine gland function
  • Autosomal recessive almost always in Caucasians
    • Defect in gene which codes for cystic fibrosis transmembrane conductance regulator (CFTR)
  • Major clinical manifestations are pulmonary and pancreatic insufficiency
  • Elevated concentration of sodium and chloride in sweat
  • Most patients are diagnosed by age 1year

Clinical Findings

  • Positive sweat chloride test
  • Chronic cough
  • Recurrent pulmonary infections
  • Higher incidence of asthma and allergy
  • Diabetes
  • Undescended testicles

Imaging Findings

  • Atelectasis
    • Discoid, segmental, lobar with right upper lobe predominance
  • Mucoid impaction
    • Nodular and fingerlike densities along bronchovascular bundle
  • Cylindrical or cystic bronchiectasis
    • Peribronchial cuffing
  • Hilar adenopathy
  • Pulmonary arterial hypertension and cor pulmonale
  • Recurrent pneumonias,
    • Particularly Staphylococcus,  Pseudomonas and P. cepacia
  • Clubbing and hypertrophic osteoarthropathy can occur
  • Recurrent pneumothorax is common

Differential Diagnosis

  • Asthma
  • Bronchiectasis
  • Aspergillosis

Associated Findings

  • Bulky, fatty stools from lack of pancreatic enzymes
  • Rectal prolapse
  • Meconium ileus — earliest finding
  • Meconium ileus equivalent — due to obstruction from stool in older children
  • Fatty infiltration of the liver
  • Focal biliary cirrhosis with portal hypertension
  • Gallstones
  • Pancreatic fibrosis due to recurrent Pancreatitis
    • Diabetes mellitus
  • Sinusitis
  • Hypoplastic frontal sinuses


  • Goals are to maintain lung function and nutritional therapy
    • Bronchodilators
    • Chest physical therapy
    • Mucolytic agents
    • Pancreatic enzyme supplements
    • Multivitamins


  • Varies from country to country but highest in the United States
  • 80% should reach adulthood

Cystic Fibrosis. White arrow points to mucous-filled bronchus; white circles enclose areas of peribronchial thickening and nodularity. Yellow arrow points to lingular atelectasis.
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cystic fibrosis

Frontal chest x-ray in cystic fibrosis shows diffuse interstitial disease
with bronchiectasis and nodular densities of mucoid impaction

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