Domino liver graft from a patient with homozygous familial hypercholesterolemia
Article first published online: 11 MAR 2009
© 2009 John Wiley & Sons A/S
Volume 14, Issue 3, pages E30–E33, May 2010
How to Cite
Liu, C., Niu, D.-M., Loong, C.-C., Hsia, C.-Y., Tsou, M.-Y., Tsai, H.-L. and Wei, C. (2010), Domino liver graft from a patient with homozygous familial hypercholesterolemia. Pediatric Transplantation, 14: E30–E33. doi: 10.1111/j.1399-3046.2009.01133.x
- Issue published online: 9 APR 2010
- Article first published online: 11 MAR 2009
- Accepted for publication 5 January 2009
- domino liver transplantation;
- homozygous familial hypercholesterolemia;
- sequential liver transplantation
Liu C, Niu D-M, Loong C-C, Hsia C-Y, Tsou M-Y, Tsai H-L, Wei C. Domino liver graft from a patient with homozygous familial hypercholesterolemia. Pediatr Transplantation 2010: 14:E30–E33. © 2009 John Wiley & Sons A/S.
Abstract: 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.