Brocklebank V, Kumar G, Howie AJ, et al. - Given that genetic pleiotropy is demonstrated by recessive mutations in diacylglycerol kinase epsilon (DGKE), with pathological characteristics documented as either thrombotic microangiopathy or membranoproliferative glomerulonephritis (MPGN), and clinical characteristics of atypical hemolytic uremic syndrome (aHUS), nephrotic syndrome or both, and since pathophysiological mechanisms as well as optimal treatment strategies have not yet been described, so, researchers report a cohort including 16 people with DGKE nephropathy, and one presented with isolated nephrotic syndrome. As per the study of pathological characteristics, it was shown that DGKE mutations afforded an MPGN-like appearance to varied extents, with but more commonly without alterations in arterioles or arteries. Most of the patients suffered persistent proteinuria. Reaching end stage renal disease was evident in only 2 people, 20 years following the initial presentation, and successful renal transplantation with no relapse was reported in one. Eculizumab was administered to 6 individuals. One individual experienced relapses on treatment. Eculizumab was withdrawn in 4 people, with one spontaneously resolving aHUS relapse occurring. Therefore, DGKE-mediated aHUS was identified to be eculizumab non-responsive, and it was also suggested that in people who currently take eculizumab treatment it can be safely withdrawn.