Abstract: Objective To analyze clinical data of 4 cases of renal disease with reversible posterior encephalopathy syndrome (PRES), and improve the understanding of the disease. Methods Data of clinical manifestations, etiology and treatment of 4 cases of PRES hospitalized in our hospital from May 2014 - April 2018 were summarized and analyzed. Results Four cases were diagnosed with nephrotic syndrome. In 3 cases with headache, other clinical manifestations were found in 48 hours. There were convulsions in 4 children, including 1 case with acute kidney injury, 2 cases with liver function damage in the application of tacrolimus therapy. The tacrolimus blood concentrations increased in varying degrees. In 1 case, CYP3A5 gene was detected as hybrid mutant (AG), tacrolimus was decreased/stop. Clinical symptoms were quickly improved in 4 cases after active treatment. No recurrence of PRES was found in the follow-up period. Conclusion Headache is the first symptom of PRES. When nephrotic syndrome is associated with convulsion, it should be differentiated from PRES. Hyperperfusion caused by high blood pressure and tacrolimus induced endothelial injury can be the cause. The CYP3A5 gene detection should be performed as early as possible, to closely monitor liver function and tacrolimus blood concentrations in children with cytotoxic edema on cranial magnetic resonance imaging.

Key words: nephrotic syndrome, child, posterior reversible encephalopathy syndrome