Domino liver graft from a patient with homozygous familial hypercholesterolemia.
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.
Liu
Chinsu
C
Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan. csliu@vghtpe.gov.tw
Niu
Dau-Ming
DM
Loong
Che-Chuan
CC
Hsia
Cheng-Yuan
CY
Tsou
Mei-Yung
MY
Tsai
Hsin-Lin
HL
Wei
Choufu
C
D000293:Adolescent; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II; D008113:Liver Neoplasms / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D008875:Middle Aged