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Domino liver graft from a patient with homozygous familial hypercholesterolemia
Author(s) -
Liu Chinsu,
Niu DauMing,
Loong CheChuan,
Hsia ChengYuan,
Tsou MeiYung,
Tsai HsinLin,
Wei Choufu
Publication year - 2010
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2009.01133.x
Subject(s) - domino , medicine , liver transplantation , familial hypercholesterolemia , transplantation , steatosis , liver disease , cholesterol , myocardial infarction , gastroenterology , endocrinology , cardiology , biochemistry , biology , catalysis
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.