Abstract：
Homozygous familial hypercholesterolaemia is a life-threatening genetic condition, which causes extremely elevated LDL-C levels and atherosclerotic cardiovascular disease very early in life. It is vital to start effective lipid-lowering treatment from diagnosis onwards. Even with dietary and current multimodal pharmaceutical lipid-lowering therapies, LDL-C treatment goals cannot be achieved in many children. Lipoprotein apheresis is an extracorporeal lipid-lowering treatment, which is used for decades, lowering serum LDL-C levels by more than 70% directly after the treatment. Data on the use of lipoprotein apheresis in children with homozygous familial hypercholesterolaemia mainly consists of case-reports and case-series, precluding strong evidence-based guidelines. We present a consensus statement on lipoprotein apheresis in children based on the current available evidence and opinions from experts in lipoprotein apheresis from over the world. It comprises practical statements regarding the indication, methods, treatment goals and follow-up of lipoprotein apheresis in children with homozygous familial hypercholesterolaemia and on the role of lipoprotein(a) and liver transplantation.
摘要：
纯合子家族性高胆固醇血症是一种危及生命的遗传病，这导致极高的LDL-C水平和动脉粥样硬化性心血管疾病在生命的早期。从诊断开始开始有效的降脂治疗至关重要。即使使用饮食和当前的多模式药物降脂疗法，许多儿童无法实现LDL-C治疗目标。脂蛋白单采术是一种体外降脂治疗，使用了几十年，治疗后直接降低血清LDL-C水平70％以上。纯合型家族性高胆固醇血症患儿使用脂蛋白单采术的数据主要包括病例报告和病例系列。排除了强有力的循证指南。根据目前现有的证据和来自世界各地的脂蛋白单采专家的意见，我们提出了关于儿童脂蛋白单采的共识声明。它包括关于指示的实际陈述，方法,纯合子家族性高胆固醇血症患儿脂蛋白单采的治疗目标和随访以及脂蛋白（a）和肝移植的作用。
