Lee Misan, Barr Janet, Kribs Stewart, Filler Guido
Department of Paediatrics, University of Western Ontario, Ontario, Canada.
Department of Radiology, University of Western Ontario, Ontario, Canada.
BMJ Case Rep. 2017 Sep 1;2017:bcr-2017-219538. doi: 10.1136/bcr-2017-219538.
Patients with homozygous familial hypercholesterolaemia are optimally treated with low-density lipoprotein apheresis. Young patients who do not meet a weight threshold (25 kg) receive regular plasmapheresis. This approach may remove excessive immunoglobulins and vascular access set-up can be challenging. We report the case of a 4 year-old child who exhibited repeated septic infections (5 in 6 months) and had recurrent access issues before two interventions were implemented: (1) the percutaneous central venous line was modified to two implanted paediatric ports, and (2) the patient started receiving two bags of Octaplasma at the end of each plasmapheresis treatment to account for the excessive loss of immunoglobulins. For the paediatric plasmapheresis access port, a 19-gauge Huber needle had to be used for the arterial port to prevent the collapse of the extension. These two simple changes have left the patient infection-free for 9 months.
纯合子家族性高胆固醇血症患者的最佳治疗方法是进行低密度脂蛋白去除术。未达到体重阈值（25千克）的年轻患者接受定期血浆置换。这种方法可能会去除过多的免疫球蛋白，而且建立血管通路可能具有挑战性。我们报告了一名4岁儿童的病例，该儿童在实施两项干预措施之前出现反复的败血症感染（6个月内5次）且反复出现通路问题：（1）将经皮中心静脉导管改为两个植入式儿科端口，（2）患者在每次血浆置换治疗结束时开始接受两袋Octaplasma，以补充免疫球蛋白的过度流失。对于儿科血浆置换通路端口，动脉端口必须使用19号休伯针以防止延长管塌陷。这两个简单的改变使该患者9个月未发生感染。
