Primary hyperoxaluria type I (PH I) is a rare genetic disorder that leads to end stage renal disease (ESRD) at an early age due to excessive deposition of calcium oxalate in the kidney. Combined liver-kidney transplantation (LKTx) has been advocated as the treatment of choice for patients with PH I who have progressive renal disease. With combined LKTx the risk of early renal failure secondary to oxalate deposition is anticipated. Here we report a patient with PH I who developed ESRD and underwent a combined LKTx. He lost the kidney graft secondary to early recurrence of oxalosis. Repeat kidney transplantation 13 months after the initial procedure was successful. Elevated plasma oxalate levels persisted for a long time following LKTx and lead to further deposition of oxalate in the second kidney graft. Combined LKTx for patients with PH I requires meticulous preparation and very careful post operative management. Sequential liver transplantation followed by kidney transplantation is to be considered for PH I patients who have ESRD and very high oxalate load.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3509864PMC
http://dx.doi.org/10.1007/8904_2011_67DOI Listing

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