Brocklebank V, Kumar G, Howie AJ, et al. - Researchers report a cohort of 16 people with diacylglycerol kinase epsilon (DGKE) nephropathy, describing long-term results as well as response to therapy, given recessive mutations in DGKE show genetic pleiotropy, with pathological characteristics documented as either thrombotic microangiopathy or membranoproliferative glomerulonephritis (MPGN), and clinical features of atypical hemolytic uremic syndrome (aHUS), nephrotic syndrome or both. Presentation with isolated nephrotic syndrome was reported in one. On examining pathological features, it was found that DGKE mutations gave an MPGN-like appearance to varying extents, with but more frequently without alterations in arterioles or arteries. The presence of concurrent substantial proteinuria was detected in 10 of 15 patients who presented with aHUS. At least one relapse was reported in 9 with aHUS, although the occurrence of a relapse of aHUS after age five years was noted in only one. Most of the patients had persistent proteinuria. Overall, it was inferred that DGKE-mediated aHUS is eculizumab non-responsive, and safe withdrawal of eculizumab can be achieved in patients who currently receive eculizumab therapy. This has significant patient safety as well as financial implications.