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LKT is the only effective treatment for PH1 because it replaces both the source (liver) and the target (kidney) of the disease. Most studies report on LKT in patients with PH1 from deceased donors. This study reports on five patients who underwent LKT from a single living  donor between April 2017 and March 2018. Combined LKT was performed for 1 patient and sequential LKT for the remainder. The median age of the patients at the time of diagnosis and transplantation was 5.5 (0.3‐18) and 10 (6‐21) years, respectively. All patients received left lateral liver segment transplantation, except one patient who received right liver lobe transplantation. No liver graft loss was observed, and liver function tests were stable at the final evaluation of all patients. Renal function tests of the patients were also stable at the final assessment, except for the young adult patient. None of the patients suffered from acute rejection. One patient died at the second month following liver transplantation due to severe pneumonia and sepsis. This study concludes that combined or sequential LKT from a single living donor can be safely performed and provides encouraging results for even the youngest and smallest patients with PH1.

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