Fast Five Quiz: Primary Hyperoxaluria Type 1 Management

Bradley Schwartz, DO

Disclosures

March 04, 2022

Figure 1. Polarizing microscopy showing oxalate crystals in a child's kidney with primary oxalosis, a rare congenital metabolic disorder.

Patients with PH1 typically present with urinary oxalate excretion > 100 mg/d. Early medical management to reduce the oxalate level and to prevent deterioration of renal function is essential. Unfortunately, dietary oxalate restrictions are of no substantial benefit in this type of hyperoxaluric disease.

After diagnosis of PH1, management with high fluid intake (approximately 3 L/m2/d) should be initiated, with the goal of diluting urine both during the day and in the evening. Patient adherence can be challenging.

Pyridoxine deficiency increases urinary oxalate excretion; thus, high-dose pyridoxine (vitamin B6) may reduce the production of oxalate by improving the conversion of glyoxylate to glycine and reducing the substrate available for metabolism to oxalate. In some patients, high-dose pyridoxine therapy may enable a normal urinary oxalate level (< 40 mg/d) to be achieved. In patients with disease that is resistant to pyridoxine, combination treatment with available therapies may be necessary.

Learn more about the management of PH1.

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