2008 Volume 21 Issue 2 Pages 182-187
A 22-year-old severely disabled adult patient with valproate (VPA)-induced Fanconi syndrome was described. He developed to hypouricemic acute renal failure (ARF) when he had respiratory failure.
He had been treated with VPA over a period of 20 years: VPA was initiated since 8 months of age when he was diagnosed as having West syndrome. At the age of 22 years and 3 months, the diagnosis of VPA-induced Fanconi syndrome was made based on typical laboratory findings, such as hypophosphatemia, severe hypouricemia (0.5mg/dl), renal glucosuria, tubular proteinuria and generalized aminoaciduria. Three months later, he was hospitalized because of respiratory failure due to pneumonia complicated by asthmatic attack. Although both anti-microbial and anti-asthmatic treatment improved his respiratory failure, he developed to ARF 4 days after admission:his renal function recovered gradually by diuretic therapy. Quantitative measurement of blood reactive oxygen metabolites revealed severe oxidative stress at the time of ARF.
From these findings, we believe that the oxidative stress by respiratory failure caused ARF in our patient because it is speculated that renal reperfusion injury due to vasoconstriction results from an exercise-induced increase in oxygen free radicals and a lack of urate, free radical scavengers in patients with severe hypouricemia due to URAT 1 mutation.
