Cystic Fibrosis

Patients with cystic fibrosis have an elevated content of sodium and chloride in sweat. During hot weather or strenuous exercise, affected children can suffer severe salt depletion and corresponding electrolyte abnormalities. Intercurrent pulmonary infection may be present. Profound chloride loss is compensated for by a rise in serum bicarbonate, resulting in a metabolic alkalosis. Hyponatremia is present, and serum potassium is decreased. Patients can present with lethargy and signs of hypoperfusion. Treatment is with 0.9 NS, with supplemental potassium. Resuscitation should be aggressive.

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