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Cystic Fibrosis
Mucovicidosis
 

        Abnormal secretions from exocrine glands

        Autosomal recessive almost always in Caucasians

o       Defect in gene which codes for cystic fibrosis transmembrane conductance regulator (CFTR)

        Major clinical manifestations: COPD and pancreatic insufficiency

        Elevated concentration of Na and Cl in sweat

 

X-ray

 

  • Atelectasis

    • Discoid, segmental, lobar with RUL 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 Staph,  Pseudomonas and P. cepacia

  • Clubbing and hypertrophic osteoarthropathy can occur

  • Recurrent pneumothorax is common

 

 

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

 

Other 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

 

Clinically

 

  • Chronic cough, recurrent pulmonary infections

  • Higher incidence of asthma and allergy

 

 

 

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