Liver transplantation for homozygous XXXX : two case reports.

@entity1 @entity1667 @entity753 @entity617 @entity548 @entity618 @entity74 @entity1140 @entity209 @entity731 @entity65 @entity165
Homozygous @entity753 (HFHC) is a rare @entity74 with an incidence of one in one million. It is associated with severe @entity731 and early death from @entity65 . Mutation in the gene that encodes the synthesis of the cellular receptor for low-density lipoprotein (LDL) is responsible for this @entity209 . Currently, the only effective treatment for this disease is liver transplantation, which alone or in association with medications, normalizes plasma @entity165 level. The authors report the results of liver transplantation for two cases of HFHC. The first case, a 15-year-old @entity1 received a whole liver from a deceased donor, and the second, an 11-year-old @entity1 , received a left liver lobe transplant from his mother's sister. Their preoperative fasting lipid concentrations were grossly raised. The older @entity1 had severe @entity731 and had undergone coronary artery bypass grafting 5 months before transplantation. Both had preoperative plasma @entity165 levels higher than 750 mg/dL with normal thyroid and liver function tests. After the operation, the @entity1 received @entity1667 as pulse therapy followed by oral @entity617 , @entity618 , and @entity1140 for immunosuppression. Their hospital stays were 24 and 13 days, respectively. The first case needed reexploration because of @entity548 on the second day after the operation. The lipid concentrations rapidly returned to the normal range in the first week after the operation, remaining in this range over the first 6 months of follow-up. Liver transplantation offers an highly effective treatment for HFHC. It is better to operate on @entity1 before severe @entity731 changes in the coronary arteries. All @entity1 must undergo a complete cardiac evaluation before surgery.
