American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons · 2020
Successful simultaneous liver-kidney transplantation for renal failure associated with hereditary complement C3 deficiency.
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Nayagam Jeremy S, McGrath Samuel, Montasser Mahmoud, Delaney Michael, Cairns Tom D, Marchbank Kevin J, Denton Harriet, Yang Yi, Sacks Steven H, Cook H Terry, Shah Sapna, Heaton Nigel, Pickering Matthew C, Suddle Abid
Аннотация
Hereditary complement C3 deficiency is associated with recurrent bacterial infections and proliferative glomerulonephritis. We describe a case of an adult with complete deficiency of complement C3 due to homozygous mutations in C3 gene: c.1811delT (Val604Glyfs*2), recurrent bacterial infections, crescentic glomerulonephritis, and end-stage renal failure. Following isolated kidney transplantation he would remain C3 deficient with a similar, or increased, risk of infections and glomerulonephritis. As C3 is predominantly synthesized in the liver, with a small proportion of C3 monocyte derived and kidney derived, he proceeded to simultaneous liver-kidney transplantation. The procedure has been successful with restoration of his circulating C3 levels, normal liver and kidney function at 26 months of follow-up. Simultaneous liver-kidney transplant is a viable option to be considered in this rare setting.