Combined kidney and liver transplantation for familial haemolytic uraemic syndrome.
Recurrent haemolytic uraemic syndrome (HUS) is a genetic form of thrombotic microangiopathy that is mostly associated with low activity of complement factor H. The disorder usually develops in families, leads to end stage renal disease, and invariably recurs after kidney transplantation. We did a simultaneous kidney and liver transplantation in a 2-year-old child with HUS and a mutation in complement factor H to restore the defective factor H, with no recurrence of the disease. The operation was successful, and at discharge, the child had healthy kidney and liver function, with no sign of haemolysis.
Remuzzi
Giuseppe
G
Ospedali Riuniti, Bergamo, Italy. gremuzzi@marionegri.it
Ruggenenti
Piero
P
Codazzi
Daniela
D
Noris
Marina
M
Caprioli
Jessica
J
Locatelli
Giuseppe
G
Gridelli
Bruno
B
D006463:Hemolytic-Uremic Syndrome / Q000235:genetics* / Q000601:surgery*; D006801:Humans; D007223:Infant; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008297:Male; D017354:Point Mutation