How does type II pseudohypoaldosteronism (PHAII) (Gordon syndrome) affect hyperkalemia (high serum potassium level)?

Updated: Apr 09, 2020
  • Author: Eleanor Lederer, MD, FASN; Chief Editor: Vecihi Batuman, MD, FASN  more...
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The genes causing this disorder code for protein kinases that are localized to the distal tubule and that regulate ion transport in this nephron segment. WNK4 appears to have several roles in regulating sodium, potassium, and chloride transport through transcellular and paracellular pathways. [42] Interestingly, PHAII from mutations in WNK1 is significantly less severe than PHAII from mutations in WNK4 or KLHL3, whereas PHAII from mutations in CUL3 is more severe. [43] All forms of PHII generally respond to treatment with thiazide diuretics.

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