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Atypical haemolytic uraemic syndrome

Lookup NU author(s): Professor David KavanaghORCiD, Professor Tim Goodship, Dr Anna Richards


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The haemolytic uraemic syndrome (HUS) is characterized by the triad of thrombocytopenia, microangiopathic haemolytic anaemia and acute renal failure. HUS may be classified as either diarrhoeal-associated or non-diarrhoeal/atypical (aHUS). aHUS has recently been shown to be a disease of complement dysregulation, with 50% of cases involving the complement regulatory genes, factor H (CFH), membrane cofactor protein (MCP; CD46), and factor I (IF). However, incomplete penetrance of mutations in each of these genes is reported. This suggests that a precipitating event or trigger is required to unmask the complement regulatory deficiency. The reported precipitating events predominantly cause endothelial injury. Discovery of these mutations has revealed important genotype-phenotype correlations. MCP-HUS has a better prognosis and a better outcome after transplantation than either CFH-HUS or IF-HUS. © The Author 2006.

Publication metadata

Author(s): Kavanagh D, Goodship THJ, Richards A

Publication type: Review

Publication status: Published

Journal: British Medical Bulletin

Year: 2006

Volume: 77-78

Issue: 1

Pages: 5-22

ISSN (print): 0007-1420

ISSN (electronic): 1471-8391


DOI: 10.1093/bmb/ldl004

PubMed id: 16968692