Volume 70, Issue 3, Pages 423-431 (August 2006) A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders  N. Besbas, D. Karpman, D. Landau, C. Loirat, W. Proesmans, G. Remuzzi, G. Rizzoni, C.M. Taylor, N. Van de Kar, L.B. Zimmerhackl  Kidney International  Volume 70, Issue 3, Pages 423-431 (August 2006) DOI: 10.1038/sj.ki.5001581 Copyright © 2006 International Society of Nephrology Terms and Conditions

Figure 1 Sub-group diagnosis in 167 children with non-diarrheal, VTEC negative HUS entered into the registry of the European Pediatric Research Group. Part 1 subgroups are colored sections, part 2 are gray. Of the 34 with mutations in complement regulators, 22 involved FH, six FI, and six MCP. Two further cases had acquired anti-FH antibodies. Abnormality in ADAMTS13 protease and or gene was found in 12 cases, two of whom also had a complement FH mutation. HUS attributable to pneumococcal sepsis occurred in 12 children. There were eight cases, four sibling pairs, in whom the etiology was unknown but the disorder was clearly familial. In one case HUS complicated existing mesangioproliferative glomerulonephritis. In 22 of the unclassified cases complement disregulation was suspected because of relapsing disease and low C3 concentration, or abnormal Western blot profile of complement FH. Kidney International 2006 70, 423-431DOI: (10.1038/sj.ki.5001581) Copyright © 2006 International Society of Nephrology Terms and Conditions