Atypical HUS

概要

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Hemolytic uremic syndrome (HUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. HUS is classified into two groups: diarrheal-associated (D+HUS) and non-diarrheal [atypical (a)-HUS]. D+HUS is the common form, accounting for 90% of cases of HUS. D+HUS is caused by the infection of Shigatoxin produced by bacteria, such as E. coli O157 : H7. aHUS is rare and has a poor prognosis, with mortality rates of up to 25% and 50% requiring renal transplantation. Recent studies have revealed that aHUS is a disease of complement dysregulation with 50-60% of cases involving the complement regulatory genes, factor H (CFH), membrane cofactor protein (MCP;CD46) and factor I (FI) and so on. However, to date there are a few reports of aHUS in Japan. This may be caused by paucity of diagnostic strategy for aHUS. Therefore, it is necessary to establish an appropriate diagnostic pathway focusing on the complement system for choosing the most favorable therapeutic approach in Japan.
2012-02-29