Liu Chinsu, Niu Dau-Ming, Loong Che-Chuan, Hsia Cheng-Yuan, Tsou Mei-Yung, Tsai Hsin-Lin, Wei Choufu
Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan.
Pediatr Transplant. 2010 May;14(3):E30-3. doi: 10.1111/j.1399-3046.2009.01133.x. Epub 2009 Mar 10.
HFH is a metabolic disease caused by a defect in the gene that encodes the synthesis of the cellular receptor for LDL-Rs. A high plasma level of cholesterol is present from birth and leads to severe atherosclerosis in childhood, and death from myocardial infarction usually occurs before the age of 20 yr. The liver contains approximately 50-75% of the total body LDL-Rs; therefore, liver transplantation has been carried out to treat this metabolic disorder effectively. The rationale for using an HFH liver for a domino graft is that the absence of functional LDL-Rs in the liver may be compensated for by the extra-hepatic LDL-Rs. Therefore, an HFH liver can possibly be used as a domino graft for a recipient with a normal plasma cholesterol level before transplantation. We herein report a domino liver transplantation using an HFH liver as a domino graft with successful results. To expand the donor pool, especially in Asian countries, domino grafts from HFH should be encouraged by careful selection of the domino recipient.
家族性高胆固醇血症（HFH）是一种代谢性疾病，由编码低密度脂蛋白受体（LDL-Rs）细胞受体合成的基因缺陷引起。从出生起血浆胆固醇水平就很高，并导致儿童期严重的动脉粥样硬化，通常在20岁之前死于心肌梗死。肝脏含有约占全身LDL-Rs总量的50-75%；因此，已进行肝移植以有效治疗这种代谢紊乱。将HFH肝脏用于多米诺骨牌式移植的基本原理是，肝脏中缺乏功能性LDL-Rs可能由肝外LDL-Rs来代偿。因此，在移植前，HFH肝脏可能可以用作多米诺骨牌式移植物给血浆胆固醇水平正常的受者。我们在此报告使用HFH肝脏作为多米诺骨牌式移植物进行多米诺肝移植，结果成功。为了扩大供体库，特别是在亚洲国家，应通过仔细选择多米诺骨牌式受者来鼓励使用来自HFH的多米诺骨牌式移植物。
