Cystic Fibrosis
Cystic Fibrosis

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Cystic FibrosisGeneral
Autosomal recessive
Almost always in Caucasians
Dysfunction of the exocrine glands
Elevated concentration of Na and Cl insweat

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Cystic FibrosisX-ray-1
Atelectasis
Discoid, segmental, lobar with RULpredominance
Mucoid impaction
Cylindrical or cystic bronchiectasis
Peribronchial cuffing
Hilar adenopathy

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Cystic FibrosisX-ray-2
Recurrent pneumonias
Particularly staph and pseudomonas
Pneumothorax
Hypertrophic osteoarthropathy
Pulmonary arterial hypertension

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Cystic FibrosisOther findings
Meconium ileus
Earliest finding
Meconium ileus equivalent
Due to obstruction from stool in olderchildren
Rectal prolapse
Fatty infiltration of the liver

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Cystic FibrosisOther findings
Focal biliary cirrhosis
Leads to portal hypertension
Gallstones
Pancreatic fibrosis
Due to recurrent pancreatitis
Sinusitis
Hypoplastic frontal sinuses

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Cystic FibrosisClinical
Chronic cough
Recurrent pulmonary infections

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