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Lookup NU author(s): Professor Kevin Marchbank
This is the authors' accepted manuscript of an article that has been published in its final definitive form by Wiley-Blackwell Publishing, Inc., 2020.
For re-use rights please refer to the publisher's terms and conditions.
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 synthesised 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 24 months of follow up. Simultaneous liver-kidney transplant is a viable option to be considered in this rare setting.
Author(s): Nayagam JS, McGrath S, Montasser M, Delaney M, Cairns TD, Marchbank KJ, Yang Y, Denton H, Sacks SH, Cook HT, Shah S, Heaton N, Pickering MC, Suddle A
Publication type: Article
Publication status: Published
Journal: American Journal of Transplantation
Print publication date: 01/08/2020
Online publication date: 23/01/2020
Acceptance date: 14/01/2020
Date deposited: 18/01/2020
ISSN (print): 1600-6135
ISSN (electronic): 1600-6143
Publisher: Wiley-Blackwell Publishing, Inc.
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