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Cystic Fibrosis

  •  Autosomal recessive almost always in caucasians

  •  Dysfunction of the exocrine glands

  •  Elevated concentration of Na and Cl in sweat

 X-ray  

  • Atelectasis

  • Discoid, segmental, lobar with RUL predominance

  • Mucoid impaction

  • Cylindrical or cystic bronchiectasis

  • Peribronchial cuffing

  • Hilar adenopathy

  • Pulmonary arterial hypertension

  • Recurrent pneumonias, particularly staph and Pseudomonas

  • Hypertrophic osteoarthropathy

  • Pneumothorax

 Other findings

  • 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

  • Sinusitis

  • Hypoplastic frontal sinuses

 Clinically

  • Chronic cough, recurrent pulmonary infections

 

 WH/’91

 

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