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Combined liver-kidney transplantation for rare diseases. | LitMetric

Combined liver-kidney transplantation for rare diseases.

World J Hepatol

Department of Medicine, Merkur University Hospital, Zagreb 10000, Croatia.

Published: October 2020

AI Article Synopsis

  • Combined liver and kidney transplantation (CLKT) is used for patients with both liver and kidney failure, particularly in cases like end-stage chronic kidney disease from liver genetic defects, especially in children but also in some adults.
  • The review highlights various rare conditions, such as primary hyperoxaluria and nephronophthisis, that can lead to ESKD and may necessitate CLKT as an effective treatment for renal function while preventing disease recurrence.
  • In addition, while CLKT has shown promise in certain genetic disorders and immune protection against donor-specific antibodies, alternatives like eculizumab are becoming more common for specific conditions like atypical hemolytic uremic syndrome.

Article Abstract

Combined liver and kidney transplantation (CLKT) is indicated in patients with failure of both organs, or for the treatment of end-stage chronic kidney disease (ESKD) caused by a genetic defect in the liver. The aim of the present review is to provide the most up-to-date overview of the rare conditions as indications for CLKT. They are major indications for CLKT in children. However, in some of them ( atypical hemolytic uremic syndrome or primary hyperoxaluria), CLKT may be required in adults as well. Primary hyperoxaluria is divided into three types, of which type 1 and 2 lead to ESKD. CLKT has been proven effective in renal function replacement, at the same time preventing recurrence of the disease. Nephronophthisis is associated with liver fibrosis in 5% of cases and these patients are candidates for CLKT. In alpha 1-antitrypsin deficiency, hereditary C3 deficiency, lecithin cholesterol acyltransferase deficiency and glycogen storage diseases, glomerular or tubulointerstitial disease can lead to chronic kidney disease. Liver transplantation as a part of CLKT corrects underlying genetic and consequent metabolic abnormality. In atypical hemolytic uremic syndrome caused by mutations in the genes for factor H, successful CLKT has been reported in a small number of patients. However, for this indication, CLKT has been largely replaced by eculizumab, an anti-C5 antibody. CLKT has been well established to provide immune protection of the transplanted kidney against donor-specific antibodies against class I HLA, facilitating transplantation in a highly sensitized recipient.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7643210PMC
http://dx.doi.org/10.4254/wjh.v12.i10.722DOI Listing

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