In particular, Dinour et al. [10] and Shima et al. [13] reported patients that were either homozygous or compound heterozygous for mutations in SLC2A9, who showed severe hypouricemia (0.1-0.7 mg/dl), very high FE-UA (>150%) and a high incidence of EIARF, nephrolithiasis and CKD compared with the asymptomatic heterozygous carriers, who showed only moderately low serum UA levels (Table 2). This evidence concerns the gene SLC2A9 and chronic kidney disease.