Potassium Tolerance in Sickle-Cell Hemoglobinopathy

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To the editor: De Fronzo and associates in the March 1979 issue (1) have described a K+ secretory defect in six patients with sickle-cell hemoglobinopathy but hyperkalemia (even during acute and chronic potassium loading) was notably absent. However, these authors made note of an abstract (2) describing hyperkalemia in a patient with sickle-cell hemoglobinopathy and commented that they have seen a chronically hyperkalemic patient with HbSS disease. We have recently studied a patient with documented HbSS disease who developed life-threatening hyperkalemia while receiving only modest amounts of potassium.

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