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J Nephrol. 1998 Mar-Apr;11 Suppl 1:49-50.

Pyridoxine-responsive PH1: treatment.

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  • 1Département médico-chirurgical de Néphrologie, Cliniques Universitaires de Bruxelles, Hôpital Erasme, Belgium.


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.

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