Successful living-related liver transplantation for familial hypercholesterolemia in the Middle East.

Familial hypercholesterolemia (FH) is an autosomal dominant inherited metabolic disorder resulting in advanced vascular atherosclerosis and premature death, primarily from coronary artery disease. The primary defect is a mutation in the gene encoding for the plasma LDL receptor located on the short arm of chromosome 19. Liver transplantation is currently the most effective method of treating this disorder. Living-related liver transplantation (LRLT) has become an excellent modality for treating children, including those with inherited metabolic diseases. In this paper, we describe the first report of a LRLT for familial hypercholesterolemia and review FH and the role of liver transplantation.