To decrease the oxalate level and prevent deterioration of renal function, early medical treatment of PH1 is essential. In some patients, this may include early liver-kidney transplantation. However, both survival rates and organ survival rates in patients with PH1 are inferior to those seen in patients who undergo general transplant.
A number of medications have been shown to be useful in the management of PH1. For example, high-dose pyridoxine may reduce the production of oxalate by improving the conversion of glyoxylate to glycine, thus diminishing the substrate available for metabolism to oxalate. A combination of orthophosphate with pyridoxine has been shown to confer long-term treatment benefits. However, phosphate therapy is contraindicated in patients with renal failure. The RNA inhibitor lumasiran was approved in 2020 for the treatment of PH1.
In some patients, urinary alkalinization can be achieved with potassium citrate (0.1-0.15 g/kg body weight) or sodium citrate in cases of renal failure. This inhibits the crystallization of calcium oxalate stones. Concurrent administration with thiazide diuretics to decrease the calciuria is common.
The excess oxalate in PH1 is endogenous; as such, dietary measures do not play a major role in the management of PH1. However, dietary modifications in secondary hyperoxaluria have been useful.
Learn more details about the treatment of PH1.
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Cite this: Bradley Schwartz. Fast Five Quiz: Primary Hyperoxaluria Type 1 - Medscape - Mar 04, 2022.