Successful simultaneous liver-kidney transplantation for renal failure associated with hereditary complement C3 deficiency

Am J Transplant. 2020 Aug;20(8):2260-2263. doi: 10.1111/ajt.15785. Epub 2020 Feb 6.

Abstract

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.

Keywords: clinical research/practice; complement biology; immune deficiency; kidney disease: immune/inflammatory; kidney transplantation/nephrology; liver transplantation/hepatology.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Complement C3 / genetics
  • Glomerulonephritis*
  • Humans
  • Kidney
  • Kidney Failure, Chronic* / surgery
  • Kidney Transplantation*
  • Liver
  • Male

Substances

  • Complement C3