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

Jeremy S. Nayagam; Samuel McGrath; Mahmoud Montasser; Michael Delaney; Tom D. Cairns; Kevin J. Marchbank; Harriet Denton; Yi Yang; Steven H. Sacks; H. Terry Cook; Sapna Shah; Nigel Heaton; Matthew C. Pickering; Abid Suddle

doi:10.1111/ajt.15785

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

Jeremy S. Nayagam^*, Samuel McGrath, Mahmoud Montasser, Michael Delaney, Tom D. Cairns, Kevin J. Marchbank, Harriet Denton, Yi Yang, Steven H. Sacks, H. Terry Cook, Sapna Shah, Nigel Heaton, Matthew C. Pickering, Abid Suddle

^*Corresponding author for this work

Peter Gorer Department of Immunobiology

Research output: Contribution to journal › Article › peer-review

2 Citations (Scopus)

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.

Original language	English
Pages (from-to)	2260-2263
Number of pages	4
Journal	American Journal of Transplantation
Volume	20
Issue number	8
Early online date	6 Feb 2020
DOIs	https://doi.org/10.1111/ajt.15785
Publication status	Published - 1 Aug 2020

Keywords

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

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1111/ajt.15785

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Nayagam, J. S., McGrath, S., Montasser, M., Delaney, M., Cairns, T. D., Marchbank, K. J., Denton, H., Yang, Y., Sacks, S. H., Cook, H. T., Shah, S., Heaton, N., Pickering, M. C., & Suddle, A. (2020). Successful simultaneous liver-kidney transplantation for renal failure associated with hereditary complement C3 deficiency. American Journal of Transplantation, 20(8), 2260-2263. https://doi.org/10.1111/ajt.15785

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title = "Successful simultaneous liver-kidney transplantation for renal failure associated with hereditary complement C3 deficiency",

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",

author = "Nayagam, {Jeremy S.} and Samuel McGrath and Mahmoud Montasser and Michael Delaney and Cairns, {Tom D.} and Marchbank, {Kevin J.} and Harriet Denton and Yi Yang and Sacks, {Steven H.} and Cook, {H. Terry} and Sapna Shah and Nigel Heaton and Pickering, {Matthew C.} and Abid Suddle",

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Nayagam, JS, McGrath, S, Montasser, M, Delaney, M, Cairns, TD, Marchbank, KJ, Denton, H, Yang, Y, Sacks, SH, Cook, HT, Shah, S , Heaton, N, Pickering, MC & Suddle, A 2020, 'Successful simultaneous liver-kidney transplantation for renal failure associated with hereditary complement C3 deficiency', American Journal of Transplantation, vol. 20, no. 8, pp. 2260-2263. https://doi.org/10.1111/ajt.15785

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AU - Nayagam, Jeremy S.

AU - McGrath, Samuel

AU - Montasser, Mahmoud

AU - Delaney, Michael

AU - Cairns, Tom D.

AU - Marchbank, Kevin J.

AU - Denton, Harriet

AU - Yang, Yi

AU - Sacks, Steven H.

AU - Cook, H. Terry

AU - Shah, Sapna

AU - Heaton, Nigel

AU - Pickering, Matthew C.

AU - Suddle, Abid

PY - 2020/8/1

Y1 - 2020/8/1

N2 - 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.

AB - 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.

KW - clinical research/practice

KW - complement biology

KW - immune deficiency

KW - kidney disease: immune/inflammatory

KW - kidney transplantation/nephrology

KW - liver transplantation/hepatology

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JO - American Journal of Transplantation

JF - American Journal of Transplantation

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

Abstract

Keywords

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