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Recovery From Dialysis in Patients With Primary Hyperoxaluria Type 1... » Isaúde
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American Journal of Kidney Diseases
2020-09-03 00:00:00

Recovery From Dialysis in Patients With Primary Hyperoxaluria Type 1 Treated With Pyridoxine: A Report of 3 Cases

Description: Primary hyperoxaluria type 1 (PH1) is a genetic disorder characterized by overproduction of oxalate and eventual kidney failure. Kidney failure is usually irreversible in PH1. However, in PH1 patients homozygous for the G170R mutation, pyridoxine is an enzyme co-factor and decreases urine oxalate excretion (UOx) by reducing hepatic oxalate production. We report recovery from dialysis in three PH1 patients homozygous for the G170R mutation in response to pharmacologic dose pyridoxine treatment. Median age at initiation or resumption of pyridoxine treatment was 37 years (range 20-53), and pyridoxine dose was 8.8 mg/kg/d (range 6.8-14.0 mg/kg/d).

Identifier: 10.1053/j.ajkd.2020.07.017
Section: Case Reports
Volume: 0
Author: Elizabeth C. Lorenz, John C. Lieske, Barbara M. Seide, Julie B. Olson, Ramila Mehta, Dawn S. Milliner

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