Signs

• Obstructive airway disease: tachypnea, dyspnea, cyanosis, wheezes, crackles, sternal retractions, digital clubbing, and barrel chest

• Failure to thrive: despite apparent adequate caloric intake, children may be below age-based normal in both height and weight, and adults may be near/below ideal body weight or have a low BMI

• Salty taste to the skin

• Hepatobiliary disease: asymptomatic or evidenced by hepatomegaly, splenomegaly, or prolonged bleeding

• Recurrent pancreatitis (usually in pancreatic-sufficient patients): episodic epigastric abdominal pain, persistent vomiting, and fever

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