Name: continuous manual exchange transfusion for patients with sickle cell disease: an efficient method to avoid iron overload
Uploaded: 2017-03-14T14:00:00
Description: Watch this Scientific Journal Video about Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload at JoVE.com

We would like to thank the patients and parents for their continuous support; the caregivers for their dedication; Pr Bierling, director of the French Blood Bank in the Paris area, for his support of the collaborative work between our hospital and his team to develop the continuous manual exchange transfusion method; and the University Paris Diderot and the hospital Robert-Debr&#233; for their support.

该协议遵循医院伦理委员会的指导方针。有在交流会3个步骤：病人准备;最初的等容放血（如适用）;和全血交换，由若干个周期，或连续全血放血，其与稀释PRBCs的输液相关联的。根据患者的血红蛋白水平的，中介等容放血的第四步骤可以中途通过交换阶段加入。在会议开始时，所有必要的材料（袋装RBC和5％血清白蛋白）的必须准备。另外，各步骤必须提前制备。除了精密刻度和双静脉通路，没?...

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	<li>Stuart, M. J., Nagel, R. L. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Sickle-cell+disease.">Sickle-cell disease.</a> Lancet. 364 (9442), 1343-1360 (2004).</li><li>Aygun, B., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Chronic+transfusion+practices+for+prevention+of+primary+stroke+in+children+with+sickle+cell+anemia+and+abnormal+TCD+velocities.">Chronic transfusion practices for prevention of primary stroke in children with sickle cell anemia and abnormal TCD velocities.</a> Am J Hematol. 87 (4), 428-430 (2012).</li><li>Ohene-Frempong, K., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Cerebrovascular+accidents+in+sickle+cell+disease:+rates+and+risk+factors.">Cerebrovascular accidents in sickle cell disease: rates and risk factors.</a> Blood. 91 (1), 288-294 (1998).</li><li>Adams, R. J., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Prevention+of+a+first+stroke+by+transfusions+in+children+with+sickle+cell+anemia+and+abnormal+results+on+transcranial+Doppler+ultrasonography.">Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography.</a> N Engl J Med. 339 (1), 5-11 (1998).</li><li>Scothorn, D. J., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Risk+of+recurrent+stroke+in+children+with+sickle+cell+disease+receiving+blood+transfusion+therapy+for+at+least+five+years+after+initial+stroke.">Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke.</a> J Pediatr. 140 (3), 348-354 (2002).</li><li>Koehl, B., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Comparison+of+automated+erythrocytapheresis+versus+manual+exchange+transfusion+to+treat+cerebral+macrovasculopathy+in+sickle+cell+anemia.">Comparison of automated erythrocytapheresis versus manual exchange transfusion to treat cerebral macrovasculopathy in sickle cell anemia.</a> Transfusion. 56 (5), 1121-1128 (2016).</li><li>Kim, H. C., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Erythrocytapheresis+therapy+to+reduce+iron+overload+in+chronically+transfused+patients+with+sickle+cell+disease.">Erythrocytapheresis therapy to reduce iron overload in chronically transfused patients with sickle cell disease.</a> Blood. 83 (4), 1136-1142 (1994).</li><li>Adamkiewicz, T. V., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Serum+ferritin+level+changes+in+children+with+sickle+cell+disease+on+chronic+blood+transfusion+are+nonlinear+and+are+associated+with+iron+load+and+liver+injury.">Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury.</a> Blood. 114 (21), 4632-4638 (2009).</li><li>Raghupathy, R., Manwani, D., Little, J. A. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Iron+overload+in+sickle+cell+disease.">Iron overload in sickle cell disease.</a> Adv Hematol. , (2010).</li><li>Porter, J. B., Evangeli, M., El-Beshlawy, A. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Challenges+of+adherence+and+persistence+with+iron+chelation+therapy.">Challenges of adherence and persistence with iron chelation therapy.</a> Int J Hematol. 94 (5), 453-460 (2011).</li></ol>

与此过程相关的风险是放血和输血，这可能产生危险的后果之间的意外misbalance。一种快速枯竭会导致血容量减少和急性贫血，而没有出血过量输血会导致血液粘度增加危险。在这两种情况下，SCA患者可从血管闭塞的并发症，以及中风受损。为此，一名护士必须专用于每一个病人，并在整个过程中留在他的床边。此外，还必须密切医疗监督，医生需要在任何问题的情况下，迅速提供。任何意外的血...

将β珠蛋白基因的单点突变是负责生产异常血红蛋白（血红蛋白S，HBS）。这会导致镰状细胞性贫血（SCA），全球1的最常见的疾病之一。 SCA患者的急性症状和一些慢性并发症可以通过堆积的红血细胞（PRBCs）的输血治疗。事实上，正常红细胞的输血纠正贫血，同时稀释镰刀红细胞。其结果是，它可以增加氧的运输能力，同时减少溶血和血管闭塞事件。为了避免慢性并发症或用于治疗急性并发症，输血与镰状红细胞的耗竭相结合，或者通过放血或erythrapheresis，是限制在血红蛋白和血粘度危险增加的有效方法，同时减少循环的SS红细胞的数量2。 一个精神的主要原因障碍和神经认知缺陷与SCA 3个孩子是脑血管病变，这种疾病严重的并发症。在SCA儿童与经颅多普勒异常高的速度，长期输血能有效地防止第一冲程4的发生。为了减少复发的那些已经从缺血性卒中患者遭受的风险，输血治疗是最适当的方法5。在慢性治疗的情况下，红细胞的交换输血是比简单的RBC输注更好，因为它消除镰刀细胞，并增加了正常细胞的同时降低血液粘度和限制性铁超负荷。然而，简单的红细胞输注仍然被广泛用作脑宏观血管病治疗。虽然它迅速导致铁超负荷4，这种选择经常发生，因为它在技术上是简单，最大限度地提高输血CA的患者人数回覆。事实上，即使erythrapheresis已报道为SCA患者的慢性输血的最有效的方法，它不能被到处执行;它不适合所有患者，尤其是年幼的孩子;和它势必需要特定和昂贵的设备。 在超过20年了，我们一直在治疗儿童SCA展示脑血管病和谁是与连续手动换血（MET）方法erythrapheresis暂时不合格。在2016年，我们的团队公布了患者随访已经经历连续手动输了好几年，表明我们的方法与令人满意的哈佛商学院下降，有效预防中风和铁过载相媲美的erythrapheresis 6的限制相关。 MET的会话可在没有特定装置任何医院环境，并通过使用所需要的erythrapheresis相同体积下进行。一个这种技术的显着优点是，它可以帮助防止或至少减少，副作用（特别是铁过载）连接到重复输血的患者谁不能够经受erythrapheresis。这篇文章的目的是一步，如何才能让没有任何单采机，或有病人谁没有资格erythrapheresis，使用该医疗中心进行连续MET的会话来形容，一步方法为他们的SCD患者，特别是儿童。

<table border="0" cellpadding="0" cellspacing="0" width="569">
	<tbody>
		<tr height="20">
			<td height="20" style="height:20px;width:195px;">Precision scale</td>
			<td style="width:100px;"></td>
			<td style="width:117px;"></td>
			<td style="width:159px;"></td>
		</tr>
		<tr height="20">
			<td height="20" style="height:20px;">Cannula (x2)</td>
			<td>Macopharma</td>
			<td></td>
			<td></td>
		</tr>
		<tr height="20">
			<td height="20" style="height:20px;">Transfusion tubing (x2)</td>
			<td>Macopharma</td>
			<td></td>
			<td></td>
		</tr>
		<tr height="20">
			<td height="20" style="height:20px;">Bleeding bag (x4)</td>
			<td>Macopharma</td>
			<td></td>
			<td></td>
		</tr>
		<tr height="20">
			<td height="20" style="height:20px;">3 Way tap</td>
			<td></td>
			<td></td>
			<td></td>
		</tr>
		<tr height="20">
			<td height="20" style="height:20px;">Syringe (x4)</td>
			<td></td>
			<td></td>
			<td></td>
		</tr>
		<tr height="23">
			<td height="23" style="height:23px;">Hemoglobin test</td>
			<td>HemoCue</td>
			<td style="width:117px;">Hb 201+&#160;System</td>
			<td></td>
		</tr>
	</tbody>
</table>

continuous manual exchange transfusion for patients with sickle cell disease: an efficient method to avoid iron overload

镰状细胞性贫血（SCA）的儿童可能在脑血管病和中风，这可以通过慢性输注方案可以防止的危险。堆积的红血细胞（PRBCs）反复输血是目前用于治疗慢性输血方案的最简单和最常用的技术。然而，铁超负荷是这种治疗的主要副作用之一。较发达的方式存在，RBC（erythrapheresis），这是目前最安全和最有效的方法，尤其是单采。然而，这是昂贵的，复杂的，并且不能到处执行，它也不是适合于所有的患者。手册换血结合一个或多个手动静脉切开一个PRBC输血。 在镰刀的参考中心细胞病，我们设置了手动换血的连续方法，它是所有医院的设置是可行的，要求没有具体的设备，并广泛适用。在HBS减少方面，中风上一页ention和铁过载防止，这种方法显示出可比较的效率erythrapheresis。在erythrapheresis不可用的情况下，这个方法可以是为患者和护理中心一个很好的选择。

在这里，我们将比较安全，成本和MET的方法与erythrapheresis 6，这是降低的HbS的SCD的患者百分比的最有效的方法的效率。要做到这一点，我们记录在SCD的基准中心1,353输血交流会，包括AET 333会议并会见了1,020会议，都在SCD儿童脑血管病和/或中风的痛苦。对于患者，我们选择了下25千克MET患儿体重和/或那些缺乏高血流静脉通路，这是需要erythrapheresis（外周静?...

Watch this Scientific Journal Video about Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload at JoVE.com

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

我们已经概述了在患者的治疗镰状细胞病的连续手动换血的方法。这个安全协议的目的是要有效地限制在患者需要长期输血的铁过载，并且可以没有任何特殊的设备得到广泛使用。

与镰状细胞疾病患者连续手动换血：的有效方法避免铁超负荷

Children with sickle cell anemia (SCA) may be at risk of cerebral vasculopathy and strokes, which can be prevented by chronic transfusion programs. ...

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