Early-onset lipoprotein glomerulopathy in a 23-month-old boy with homozygous APOE Kyoto variant

The authors investigate the clinical presentation and management of a 23-month-old boy with nephrotic syndrome caused by homozygous APOE Kyoto variant, leading to lipoprotein glomerulopathy. They emphasize the importance of early renal biopsy and genetic testing in atypical pediatric nephrotic syndrome, which enabled targeted lipid-lowering therapy that resulted in significant clinical improvement. This case underscores the distinct phenotype associated with the APOE Kyoto variant and the need for alternative treatment strategies beyond immunosuppressive therapy.