Cystic Fibrosis

Cystic Fibrosis

Cystic FibrosisGeneral

Autosomal recessive

Almost always in Caucasians

Dysfunction of the exocrine glands

Elevated concentration of Na and Cl insweat

Cystic FibrosisX-ray-1

Atelectasis

Discoid, segmental, lobar with RULpredominance

Mucoid impaction

Cylindrical or cystic bronchiectasis

Peribronchial cuffing

Hilar adenopathy

Cystic FibrosisX-ray-2

Recurrent pneumonias

Particularly staph and pseudomonas

Pneumothorax

Hypertrophic osteoarthropathy

Pulmonary arterial hypertension

Cystic FibrosisOther findings

Meconium ileus

Earliest finding

Meconium ileus equivalent

Due to obstruction from stool in olderchildren

Rectal prolapse

Fatty infiltration of the liver

Cystic FibrosisOther findings

Focal biliary cirrhosis

Leads to portal hypertension

Gallstones

Pancreatic fibrosis

Due to recurrent pancreatitis

Sinusitis

Hypoplastic frontal sinuses

Cystic FibrosisClinical

Chronic cough

Recurrent pulmonary infections