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Management and prognosis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Mortality has been high in reported series. However, it is now recognised that with adequate oral potassium chloride supplementation, usually greater than 4g/day, and careful attention to fluid balance, the child may remain well and thrive normally.

Stools remain abnormally loose, but this causes little inconvenience after the first months of life.

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