DGKE and hemolytic-uremic syndrome: The 2016 International Hemolytic Uremic Syndrome Group classification is organized considering HUS etiology as: 1) infection-induced HUS (Shiga toxin producing Escherichia coli, Streptococcus pneumoniae, Influenza A, human immunodeficiency virus); 2) HUS with coexisting diseases or conditions (bone marrow or solid organ transplantation, systemic malignancies, autoimmune conditions, drugs, malignant hypertension); 3) HUS due to cobalamin C disorder; and 4) HUS due to alternative complement pathway dysregulation and mutation in diacylglycerol kinase ε (DGKE) gene1-4.