Hemolytic uremic syndrome complicated by acute necrotizing encephalopathy of childhood

Abstract

 Hemolytic uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, acute renal failure, and is usually induced by enterohemorrhagic Escherichia coli (EHEC). Central nervous system (CNS) involvement, including encephalopathy, is one of severe complications in HUS and EHEC infection. But little has been reported on acute necrotizing encephalopathy of childhood (ANE) as a complication in HUS. We had two cases of EHEC infection, HUS and encephalopathy with symmetrical thalamic lesions typical of ANE. We summarized previous case reports of HUS with CNS involvement, and those of rare association of HUS and ANE. It is suggested that the pathogenesis of severe CNS involvement in HUS, including ANE, is under the influence of cytokine release, which may be the therapeutic target against severe CNS involvement in HUS.