In PH1, when glomerular filtration rate drops to 30-45 mL/min/1.73 m2 of body surface area, the kidney is no longer able to proficiently excrete the oxalate load it receives. As a result, plasma oxalate levels increase, resulting in systemic oxalosis. Systemic oxalosis leads to oxalate deposition in the bone, kidneys, skin, retina, myocardium, vessel walls, and central nervous system.
As kidney function declines, the systemic storage of plasma oxalate increases. Improper management prior to kidney transplantation may precipitate oxalate storage in the renal graft, which can lead to premature allograft dysfunction or loss.
Learn more about the pathophysiology of PH1.
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Cite this: Bradley Schwartz. Fast Five Quiz: Primary Hyperoxaluria Type 1 - Medscape - Mar 04, 2022.