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Interactive Case: PH1

CE / CME

Diagnosis and Management of Primary Hyperoxaluria Type 1: An Interactive Case Challenge

Physician Assistants/Physician Associates: 1.00 AAPA Category 1 CME credit

Nurses: 1.00 Nursing contact hour

Physicians: maximum of 1.00 AMA PRA Category 1 Credit™

Released: December 12, 2023

Expiration: December 11, 2024

David S. Goldfarb
David S. Goldfarb, MD, FASN, FNKF
CME/CE Information

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The kidney stone is composed of calcium oxalate monohydrate. Serum chemistries demonstrate sodium 139 mEq/L, potassium 4.1 mEq/L, Cl 99 mEq/L, HCO3 24 mEq/L, BUN 20 mg/dL, and creatinine 1.1 mg/dL. A helical stone protocol CT exam of the abdomen/pelvis revealed bilateral radiopaque stones with suggestion of mild, diffuse nephrocalcinosis. A 24-hour urine collection is now done, with the key finding being an oxalate excretion of 108 mg/day.

The patient is diagnosed with PH and prescribed high fluid intake and potassium citrate. Genetic testing is now done and reveals he is homozygous for mutations in the AGXT gene, consistent with PH1. The genotype he has is known not to be pyridoxine responsive. The patient’s GFR is estimated (by Schwartz formula) to be 51 mL/min/1.73 m2. The patient is referred to a nephrologist, who recommends that he be started on RNAi therapy.

Which of the following medications would you write a prescription for to help this patient with PH as an approved RNAi treatment option that works in the liver to inhibit oxalate production by reducing the production of the enzyme glycolate oxidase (GO)?