Onaca Nicholas, Sanchez Edmund Q, Melton Larry B, Netto George J, Glastad Karl A, Martin Patriciu A, Ueno Takehisa, Levy Marlon F, Goldstein Robert M, Klintmalm Goran B
Baylor Regional Transplant Institute, Dallas, TX, USA.
Transplantation. 2005 Aug 15;80(3):421-4. doi: 10.1097/01.tp.0000168147.88707.80.
Liver transplantation (LTX) corrects the enzymatic defect responsible for type 1 primary hyperoxaluria (PH1). It has been advocated in combination with kidney transplantation (KTX) in patients with renal failure from PH1 because KTX alone can result in early graft loss. A 58-year-old male patient with PH1 on hemodialysis underwent resection of the left lateral segment of the liver followed by orthotopic auxiliary left lateral segment liver transplantation and kidney transplantation from a deceased donor. The serum oxalate dropped from 34.8 micromol/L before transplant to 3.6-8.3 in the first months posttransplant to <1 micromol/L (normal range 0.4-3.0). One year after posttransplant, the patient has an iothalamate glomerular filtration rate of 58 ml/min. Orthotopic auxiliary LTX is an alternative to whole LTX in PH1. By using a split deceased donor liver, it does not deprive the donor pool and protects the recipient from liver failure in case of graft loss.
肝移植（LTX）可纠正导致1型原发性高草酸尿症（PH1）的酶缺陷。对于因PH1导致肾衰竭的患者，一直主张将肝移植与肾移植（KTX）联合进行，因为单独进行肾移植可能导致早期移植物丢失。一名58岁接受血液透析的PH1男性患者接受了左外叶肝切除术，随后接受了原位辅助左外叶肝移植以及来自已故供体的肾移植。血清草酸盐从移植前的34.8微摩尔/升降至移植后最初几个月的3.6 - 8.3微摩尔/升，之后降至<1微摩尔/升（正常范围0.4 - 3.0）。移植后一年，患者的碘他拉酸盐肾小球滤过率为58毫升/分钟。原位辅助肝移植是PH1患者全肝移植的一种替代方法。通过使用劈开的已故供体肝脏，既不会减少供体库，又能在移植物丢失时保护受者免于肝衰竭。
