Genetic hypokalemia

INTRODUCTION: Hypokalemia is the most frequent electrolytic disturbance in hospitalized patients. It is sometimes familial. Careful clinical and biological evaluation may guide further genetic analysis. CURRENT KNOWLEDGE AND KEY POINTS: Genetic hypokalemia is linked to disorders of mineralocorticoid hormone synthesis or action (glucocorticoid-remediable hyperaldosteronism, congenital adrenal hyperplasia, apparent excess of mineralocorticoids), to renal tubular disorders (Liddle's syndrome, Bartter's and Gitelmann's syndrome, tubular acidosis) or to disorders of cellular transfer of potassium (hypokalemic periodic paralysis). FUTURE PROSPECTS AND PROJECTS: Molecular mechanisms of adult Bartter's syndrome are probably different from pediatric syndromes. A better clinical and biological evaluation with longitudinal follow-up could allow significant progress in the knowledge of the natural history and prognosis of these syndromes.