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Cystic
Fibrosis
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Autosomal recessive almost always in caucasians
-
Dysfunction of the exocrine glands
-
Elevated concentration of Na and Cl in sweat
X-ray
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Atelectasis
-
Discoid,
segmental, lobar with RUL predominance
-
Mucoid
impaction
-
Cylindrical
or cystic bronchiectasis
-
Peribronchial
cuffing
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Hilar
adenopathy
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Pulmonary
arterial hypertension
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Recurrent
pneumonias,
particularly staph and Pseudomonas
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Hypertrophic
osteoarthropathy
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Pneumothorax
Other
findings
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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
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Gallstones
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Pancreatic
fibrosis due to recurrent pancreatitis
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Sinusitis
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Hypoplastic
frontal sinuses
Clinically
WH/’91
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