Barbir M, Khaghani A, Kehely A, Tan K C, Mitchell A, Thompson G R, Yacoub M
Harefield Hospital, Middlesex.
Q J Med. 1992 Nov-Dec;85(307-308):807-12.
A 33-year-old female with severe diffuse coronary artery disease and left ventricular outflow tract obstruction secondary to homozygous familial hypercholesterolaemia underwent liver and heart transplantation. During a post-operative follow-up period of 13 months, apart from one episode of cardiac rejection at 3 weeks, she has remained well and without evidence of accelerated coronary disease on angiography. By 3 months after transplantation serum total cholesterol, low density lipoprotein cholesterol, apolipoprotein B and lipoprotein(a) levels had decreased and apolipoprotein A-I and high density lipoprotein cholesterol levels had increased, all to within the normal range. These changes were accompanied by marked regression of xanthomata and were maintained at 13 months. Liver transplantation provides an effective means of correcting serum lipids in patients with homozygous familial hypercholesterolaemia and is an important adjunct to cardiac transplantation.
一名33岁女性，患有严重的弥漫性冠状动脉疾病以及由纯合子家族性高胆固醇血症继发的左心室流出道梗阻，接受了肝脏和心脏移植手术。在术后13个月的随访期内，除了术后3周出现过一次心脏排斥反应外，她情况良好，血管造影未显示有加速性冠状动脉疾病的迹象。移植后3个月时，血清总胆固醇、低密度脂蛋白胆固醇、载脂蛋白B和脂蛋白(a)水平下降，而载脂蛋白A-I和高密度脂蛋白胆固醇水平升高，均在正常范围内。这些变化伴随着黄瘤明显消退，并持续至13个月。肝脏移植为纠正纯合子家族性高胆固醇血症患者的血脂提供了一种有效手段，并且是心脏移植的一项重要辅助措施。
