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1. Cystic Fibrosis
2. Cystic Fibrosis General
3. Cystic Fibrosis X-ray-1
4. Cystic Fibrosis X-ray-2
5. Cystic Fibrosis Other findings
6. Cystic Fibrosis Other findings
7. Cystic Fibrosis Clinical
Cystic Fibrosis
Cystic Fibrosis
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Cystic Fibrosis
General
Autosomal recessive
Almost always in Caucasians
Dysfunction of the exocrine glands
Elevated concentration of Na and Cl in
sweat
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Cystic Fibrosis
X-ray-1
Atelectasis
Discoid, segmental, lobar with RUL
predominance
Mucoid impaction
Cylindrical or cystic bronchiectasis
Peribronchial cuffing
Hilar adenopathy
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Cystic Fibrosis
X-ray-2
Recurrent pneumonias
Particularly staph and pseudomonas
Pneumothorax
Hypertrophic osteoarthropathy
Pulmonary arterial hypertension
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Cystic Fibrosis
Other findings
Meconium ileus
Earliest finding
Meconium ileus equivalent
Due to obstruction from stool in older
children
Rectal prolapse
Fatty infiltration of the liver
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Cystic Fibrosis
Other findings
Focal biliary cirrhosis
Leads to portal hypertension
Gallstones
Pancreatic fibrosis
Due to recurrent pancreatitis
Sinusitis
Hypoplastic frontal sinuses
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Cystic Fibrosis
Clinical
Chronic cough
Recurrent pulmonary infections
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