How does congenital hypoaldosteronism cause hyperkalemia?

Updated: Apr 09, 2020
  • Author: Eleanor Lederer, MD, FASN; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Congenital hypoaldosteronism

Congenital hypoaldosteronism is caused by mutations in the CYP11B2 gene, which encodes the type II corticosterone methyloxidase enzyme. It is inherited in an autosomal recessive manner. Patients with this disorder have decreased aldosterone and salt wasting. They will have an increased serum ratio of 18-hydroxycorticosterone to aldosterone.

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