Zebra of the Week: Primary hyperoxaluria type 1

Primary hyperoxaluria type 1 (PH1) is a rare disorder that results from the buildup of oxalate, which normally is filtered through the kidneys and excreted in the urine. In people with PH1, the accumulated oxalate is deposited in the kidneys and urinary tract, where it combines with calcium, forming kidney and bladder stones (calcium oxalate). Signs and symptoms may include recurrent kidney stones; hematuria; and UTIs. PH1 is due to genetic changes in a gene called AGXT; inheritance is autosomal recessive. The FDA recently approved nedosiran (Rivfloza) for the treatment of PH1.