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首页> 外文期刊>Therapeutic apheresis and dialysis: official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy >Low-density lipoprotein apheresis in children with familial hypercholesterolemia: follow-up to 21 years.
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Low-density lipoprotein apheresis in children with familial hypercholesterolemia: follow-up to 21 years.

机译：家族性高胆固醇血症儿童的低密度脂蛋白血液分离术：随访至21岁。

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Twenty-seven patients (14 girls, 13 boys) affected by familial hypercholesterolemia who had begun low-density lipoprotein (LDL) apheresis treatment before the age of 15 were studied. The median age at diagnosis was 4 years and the blood LDL cholesterol level was 704 +/- 163 mg/dL. Screening was performed for homozygous or double heterozygous mutations of the LDL cholesterol receptor gene and mutations were found in 24 of the patients. The mean age at the beginning of treatment was 8.5 years and the mean length of follow up was 12.6 years. The two main procedures used were direct adsorption of lipoproteins and dextran sulfate cellulose adsorption. Nine patients experienced anaphylactic reactions due to bradykinin and six had to have their treatment changed. The LDL cholesterol level before the session was lowered by 45 +/- 11% of the value at diagnosis. The LDL cholesterol reduction in a session was 72 +/- 10%. Tendinous xanthomas disappeared or diminished dramatically in 62% of the children. In 22 patients no cardiovascular event occurred during LDL apheresis treatment. Three had angina pectoris; two others had surgical management of aortic stenosis, but no clinical manifestations. Seven children had normal cardiovascular pictures while on treatment. Eleven had abnormalities of the aortic root or coronary arteries, which in six cases had appeared before treatment; the other five children did not undergo prior cardiac evaluation. In five children the abnormalities appeared during treatment. Based on these data, LDL-apheresis can be recommended for the treatment of homozygous familial hypercholesterolemia, even in young children, with good efficiency on biological parameters, cutaneous lesions and cardiovascular events.
机译：研究了27例家族性高胆固醇血症患者（14例女孩，13例男孩），这些患者在15岁之前就开始了低密度脂蛋白（LDL）血液分离术。诊断时的中位年龄为4岁，血液中的LDL胆固醇水平为704 +/- 163 mg / dL。对LDL胆固醇受体基因的纯合或双重杂合突变进行了筛选，在24名患者中发现了突变。开始治疗的平均年龄为8.5岁，平均随访时间为12.6年。所使用的两个主要程序是脂蛋白的直接吸附和硫酸葡聚糖的纤维素吸附。 9名患者因缓激肽而出现过敏反应，其中6名患者必须改变治疗方法。会议前的低密度脂蛋白胆固醇水平降低了诊断值的45 +/- 11％。一个疗程中的LDL胆固醇降低为72 +/- 10％。在62％的儿童中，巨大的黄瘤消失或明显减少。在22名患者中，LDL血液分离术治疗期间未发生心血管事件。 3例患有心绞痛。另外两个有主动脉瓣狭窄的手术治疗，但无临床表现。七名儿童在治疗期间的心血管影像正常。有11例主动脉根部或冠状动脉异常，有6例在治疗前出现。其他五个孩子没有接受过心脏评估。在五名儿童中，治疗期间出现异常。基于这些数据，即使在幼儿中，LDL置换也可推荐用于治疗纯合子家族性高胆固醇血症，对生物学参数，皮肤病变和心血管事件具有良好的疗效。

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