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儿童治疗性血液成分单采:一家儿科透析中心的经验

Therapeutic apheresis in children: experience in a pediatric dialysis center.

作者信息

De Palo T, Giordano M, Bellantuono R, Colella V, Troise D, Palumbo F, Caringella D A

机构信息

Pediatric Nephrology and Dialysis, Children Hospital Giovanni XXIII, Bari, Italy.

出版信息

Int J Artif Organs. 2000 Dec;23(12):834-9.

Abstract

The use of apheretic procedures in pediatric patients has always been restricted by technical difficulties and the low incidence of diseases requiring this kind of treatment. The aim of the present study was to describe the solutions adopted to solve technical difficulties related to priming, vascular access and monitoring and then to evaluate clinical results. Between 1982 and 2000, 51 consecutive children (28 male, 23 female) with a mean age of 4.9 +/- 4.8 years (3 months-14.8 years) and a mean weight of 19.7 +/- 12.8 kg (5-52 kg), with renal and/or extra-renal diseases requiring apheretic procedures were selected for the study. The overall number of procedures performed were: 226 plasma-exchange (PE), 6 LDL-apheresis (LDL-A) and 8 protein A immunoadsorption (IAPA) sessions. Our therapeutic protocol involves hematic flux of 20-100 ml/min and ultrafiltration of 5-20 ml/min. In each 70-95 minute session we exchanged plasmatic volume with fresh frozen plasma or with a solution of 6% albumin in lactated Ringer's, using heparin (10-20 UI/kg/h). We used Paired Filtration Dialysis Monitor in PE and LDL-A; Citem 10 in IAPA. As plasma separator, we used a filter made of polypropylene, 0.2 m2 surface, 30 ml priming (Hemaplex BT 900). Hemolytic uremic syndrome was the most commonly treated disease (18/51 cases) with good results in 10/18 cases. We recorded, good results in vasculitis as well, in one girl with focal glomerulosclerosis in transplanted kidney and rapid improvement in all children with Guillaine-Barré Syndrome. PE treatment was effective in metabolic disorders such as tirosynemia and familiar hypercholesterolemia. Only 4/12 patients with acute liver failure due to viral hepatitis recovered. We had poor results in the remaining eight cases. Complications were rare and no viral infection was found in any patient. Our data show that it is possible to use these procedures in pediatric patients even though clinical indications and real effectiveness still need to be cleared up.

摘要

儿科患者使用血液分离程序一直受到技术难题以及需要此类治疗的疾病发病率低的限制。本研究的目的是描述为解决与预充、血管通路和监测相关的技术难题而采取的解决方案,然后评估临床结果。1982年至2000年期间,选取了51例连续的儿童患者(男28例,女23例),平均年龄4.9±4.8岁(3个月至14.8岁),平均体重19.7±12.8千克(5至52千克),患有需要血液分离程序的肾脏和/或肾外疾病,纳入本研究。进行的程序总数为:226次血浆置换(PE)、6次低密度脂蛋白吸附(LDL-A)和8次蛋白A免疫吸附(IAPA)治疗。我们的治疗方案包括血液流速20 - 100毫升/分钟和超滤5 - 20毫升/分钟。在每次70 - 95分钟的治疗中,我们使用肝素(10 - 20国际单位/千克/小时),用新鲜冰冻血浆或6%白蛋白乳酸林格氏液置换血浆量。在PE和LDL-A中我们使用配对过滤透析监测仪;在IAPA中使用Citem 10。作为血浆分离器,我们使用了聚丙烯材质的滤器,表面积0.2平方米,预充量30毫升(Hemaplex BT 900)。溶血尿毒综合征是最常治疗的疾病(18/51例),10/18例效果良好。我们也记录到血管炎治疗效果良好,1例移植肾局灶性肾小球硬化的女孩以及所有吉兰 - 巴雷综合征患儿病情迅速改善。PE治疗对酪氨酸血症和家族性高胆固醇血症等代谢紊乱有效。12例病毒性肝炎所致急性肝衰竭患者中仅4例康复。其余8例效果不佳。并发症罕见,未在任何患者中发现病毒感染。我们的数据表明,即使临床适应症和实际疗效仍需明确,但在儿科患者中使用这些程序是可行的。

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