Cystic fibrosis

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Background

  • Autosomal recessive genetic disorder that occurs due to a mutation in cystic fibrosis transmembrane conductance regulator protein or CTFR, which is a chloride channel that is essential to the function of mucus and sweat. Patient's with cystic fibrosis do not have CTFR, therefore are unable to produce the essential components of digestive fluids, mucus, and sweat. Therefore the most common problem that these patients have are diseases related to the lung (pneumonia, bronchiectasis) and GI tract (pancreatitis).

Clinical Features

Differential Diagnosis

Evaluation

Management

Disposition

See Also

External Links

References