Ozer Ali, Aktas Hikmet, Bulum Burcu, Emiroglu Remzi
Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar University Atakent Hospital, Istanbul, Turkey.
Department of Pediatric Nephrology, Acibadem Mehmet Ali Aydinlar University Atakent Hospital, Istanbul, Turkey.
Pediatr Transplant. 2019 Jun;23(4):e13406. doi: 10.1111/petr.13406. Epub 2019 Apr 1.
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.
活体亲属肝-肾联合移植（LKT）是1型原发性高草酸尿症（PH1）唯一有效的治疗方法，因为它同时替换了疾病的源头（肝脏）和靶器官（肾脏）。大多数研究报道的是 deceased 供体来源的PH1患者接受LKT的情况。本研究报道了2017年4月至2018年3月期间，5例接受单一活体供体LKT的患者。其中1例患者接受了联合LKT，其余患者接受了序贯LKT。患者诊断时和移植时的中位年龄分别为5.5（0.3 - 18）岁和10（6 - 21）岁。除1例接受右肝叶移植外，所有患者均接受了左外叶肝移植。未观察到肝移植失败，所有患者最后一次评估时肝功能检查结果稳定。除了最年轻的成年患者外，患者的肾功能检查在最后评估时也稳定。所有患者均未发生急性排斥反应。1例患者在肝移植后第2个月因严重肺炎和败血症死亡。本研究得出结论，单一活体供体的联合或序贯LKT可以安全进行，即使是最年幼和体型最小的PH1患者也能取得令人鼓舞的结果。
