Liver-Kidney Transplantation to Cure Atypical Hemolytic Uremic Syndrome

J. M. Saland, P. Ruggenenti, G. Remuzzi
2008 Journal of the American Society of Nephrology  
Hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Typical HUS follows a diarrheal illness caused by a verocytotoxin-producing bacterium, commonly Escherichia coli O157:H7, and resolves in most cases without sequelae. 1 Atypical HUS (aHUS) is much less common and has a considerably worse prognosis in terms of both mortality and development of ESRD. 2 Mutations in genes encoding complement regulatory proteins and
more » ... ry disorders of complement regulation seem to play a central role in the pathogenesis of most cases of aHUS. Triggering of an unregulated complement cascade is thought to damage endothelium, producing microangiopathic hemolytic anemia and thrombosis. Gene mutations
doi:10.1681/asn.2008080906 pmid:19092117 fatcat:z4euwxpgpfbbdihksjeudwpo4m