How does nephronophthisis cause hyperkalemia (high serum potassium levels)?

Updated: Apr 09, 2020
  • Author: Eleanor Lederer, MD, FASN; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Nephronophthisis is characterized by enlargement of the kidneys, inflammatory portal fibrosis of the liver, and variable development of end-stage renal disease (ESRD). Patients with the infantile form of this disease generally reach ESRD before the age of 2 years. Patients with the juvenile form reach ESRD at a median age of 13 years. Patients with other forms of the disease have variable natural history.

Ultimately, this disorder causes a progressive interstitial fibrosis and tubulopathy. Routine laboratory evaluation will show increased creatinine and potassium.

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