Pyridoxine-responsive PH1: treatment

Owing to the rarity of PH, the efficacy of pyridoxine therapy has only been tested in very small series of patients. From two recent reports including 18 patients, 50% of patients would be unresponsive to pyridoxine whereas oxaluria would be normalized in 20% of patients and somewhat reduced-but not to normal level-in the remaining 30%. In a few aneodotical cases pyridoxine administration was reported to improve kidney function in patients with renal failure secondary to hyperoxaluria. It is reminded that megadoses of pyridoxine (0.5 to 6 g daily) may induce severe sensory neuropathy.