Department of Nephrology, Kidney Transplantation & Hypertension, The Children's Memorial Health Institute, Warsaw, Poland.
Department of Surgery & Organ Transplantation, The Children's Memorial Health Institute, Warsaw, Poland.
Pediatr Nephrol. 2018 Dec;33(12):2227-2237. doi: 10.1007/s00467-017-3880-4. Epub 2018 Jan 10.
Combined and sequential liver-kidney transplantation (CLKT and SLKT) is a definitive treatment in children with end-stage organ failure. There are two major indications: - terminal insufficiency of both organs, or - need for transplanting new liver as a source of lacking enzyme or specific regulator of the immune system in a patient with renal failure. A third (uncommon) option is secondary end-stage renal failure in liver transplant recipients. These three clinical settings use distinct qualification algorithms. The most common indications include primary hyperoxaluria type 1 (PH1) and autosomal recessive polycystic kidney disease (ARPKD), followed by liver diseases associated with occasional kidney failure. Availability of anti-C5a antibody (eculizumab) has limited the validity of CLKT in genetic atypical hemolytic uremic syndrome (aHUS). The liver coming from the same donor as renal graft (in CLKT) is immunologically protective for the kidney and this provides long-term rejection-free follow-up. No such protection is observed in SLKT, when both organs come from different donors, except uncommon cases of living donation of both organs. Overall long-term outcome in CLKT in terms of graft survival is good and not different from isolated liver or kidney transplantation, however patient survival is inferior due to complexity of this procedure.
联合肝肾移植（CLKT 和 SLKT）是治疗儿童终末期器官衰竭的一种确定性治疗方法。有两个主要适应证：- 两个器官的终末期衰竭，或 - 肾衰竭患者需要移植新的肝脏作为缺乏酶或免疫系统特定调节剂的来源。第三种（罕见）选择是肝移植受者的继发性终末期肾衰竭。这三种临床情况使用不同的资格算法。最常见的适应证包括 1 型原发性高草酸尿症（PH1）和常染色体隐性多囊肾病（ARPKD），其次是与偶尔肾衰竭相关的肝脏疾病。抗 C5a 抗体（依库珠单抗）的可用性限制了 CLKT 在遗传性非典型溶血性尿毒症综合征（aHUS）中的有效性。与肾移植物来自同一供体的肝脏对肾脏具有免疫保护作用，这可提供长期无排斥反应的随访。在 SLKT 中，当两个器官来自不同的供体时，不会观察到这种保护，除非是两个器官均来自活体供体的罕见情况。就移植物存活率而言，CLKT 的总体长期预后良好，与单独的肝或肾移植无差异，但由于该手术的复杂性，患者的存活率较低。
