Although such high REAs were also observed in other renal variant patients associated with GLA missense mutations (Met42Leu [5]; Pro210Ser [10]) and the renal involvement was limited to podocyte pathology, the true threshold level of REA below which could induce podocyte injury remains to be elucidated and the identification of other patients carrying IVS4+1326C>T could help further verify the mild α-GalA deficiency phenotype of Fabry podocytopathy caused by IVS4+1326C>T. The gene discussed is GLA; the disease is hyperinsulinemic hypoglycemia, familial, 4.