INTRODUCTION Differential diagnosis of thrombotic microangiopathies can be difficult. Atypical hemolytic uremic syndrome is a rare, life-threatening disease caused by uncontrolled chronic activation of alternative complement pathway, resulting in microvascular thrombosis, organ ischemia and damage. Prognosis is poor: up to 65 percent of patients require dialysis or have kidney damage of varying...

To the Editor: Atypical hemolytic–uremic syndrome is a rare microangiopathic hemolytic condition characterized by thrombocytopenia and acute renal failure.1 The prognosis for patients with atypical hemolytic–uremic syndrome with a factor H mutation is poor; 60% have end-stage renal disease or die within a year.2 The expected rate of graft failure due to recurrent atypical hemolytic–uremic syndr...

Hemolytic-uremic syndrome, the main cause of acute renal failure in early childhood, is caused primarily by intestinal infections from some Escherichia coli strains that produce Shiga toxins. The toxins released in the gut are targeted to renal endothelium after binding to polymorphonuclear leukocytes. The presence of Shiga toxins in the feces and the circulating neutrophils of 20 children with...

n engl j med 369;14 nejm.org october 3, 2013 1377 [CI], 0.57 to 0.83) for the first 21 days, 1.00 (95% CI, 0.57 to 1.76) for 22 to 60 days, and 0.38 (95% CI, 0.18 to 0.82) for 61 to 90 days. We also await results of trials such as POINT and TARDIS for confirmation of these results in non-Chinese populations. We agree with Jeong that there may be important differences according to the patients’ ...

J. Ahveninen, S. Kähkönen, H. Tiitinen, E. Pekkonen, J. Huttunen, S. Kaakkola, R. J. Ilmoniemi, and I.P. Jääskeläinen Cognitive Brain Research Unit, Department of Psychology, P.O. Box 13, FIN-00014 University of Helsinki, Finland; BioMag Laboratory, Medical Engineering Centre, Department of Clinical Neurosciences, and Department of Psychiatry, Helsinki University Central Hospital, FIN-00029 HUS...

A 67-year-old man, diagnosed as hepatocellular carcinoma by percutaneous needle aspiration biopsy of liver mass, presented microangiopathic hemolytic anemia, thrombocytopenia and renal failure from the early phase of the illness. We could establish the diagnosis of cancer-associated hemolytic uremic syndrome which was unrelated to chemotherapy clinically. This is a rare case reported in adult h...

Iptacopan (LNP023) is a first-in-class, oral, low molecular weight, Factor B inhibitor being developed for the treatment of diseases associated with activation alternative complement pathway. Current Phase III studies iptacopan include paroxysmal nocturnal hemoglobinuria, C3 glomerulonephritis, IgA nephropathy, and atypical hemolytic uremic syndrome. Preclinical identified CYP2C8, OATP P-gp as ...

1. Cutler C, KimHT, Ayanian S, et al. Prediction of veno-occlusive disease using biomarkers of endothelial injury. Biol Blood Marrow Transplant. 2010;16:1180-1185. 2. Fakhouri F, Jablonski M, Lepercq J, et al. Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome. Blood. 2008;112:4542-4545. 3. Kavanagh D, ...