《儿科重症监护输血和贫血专家倡议：危重儿童红细胞输注专家共识》介绍

贫血是危重症患儿常见表现， 红细胞（red blood cell， RBC）输注是儿科重症监护病房（pediatric intensive care unit， PICU）常用治疗手段， 在挽救患儿生命的同时， 也带来输血相关性严重危害。为促进危重症患儿合理输注RBC，降低输血相关的严重危害， 儿科重症监护输血和贫血专家倡议（transfusion and anemia expertise initiative， TAXI）专家组制订了危重症儿童RBC输注的专家共识。共识由12篇独立文章组成， 包括共识推荐意见汇总1篇， 涉及一般危重症、 呼吸衰竭、 非失血性休克、 非危及生命的出血或失血性休克、 急性脑损伤、 获得性/先天性心脏病、 血液系统和肿瘤性疾病、 体外膜氧合/心室辅助装置/肾脏替代支持治疗共9类特殊人群的RBC输注的专家共识8篇， RBC的选择和处理、 专家共识制订方法及共识应用的共识各1篇； 包括102项推荐意见， 其中57项为临床推荐意见（20项基于循证医学证据， 37项基于专家共识）， 45项为对相关研究的推荐意见。该文仅介绍临床推荐意见。     

《儿科重症监护输血和贫血专家倡议-控制/防止出血：危重儿童血浆和血小板输注实践的建议和专家共识》解读

危重症患儿出血风险高,血小板及血浆输注是儿科重症监护病房（pediatric intensive care unit,PICU）常用治疗措施之一,在挽救患儿生命的同时,也带来输血相关性不良反应。为促进危重症患儿合理输注血浆和血小板,儿科重症监护输血和贫血专家倡议-控制/防止出血（transfusion and anemia expertise initiative,TAXI）专家组于2022年制订了危重儿童血浆和血小板输注实践的建议和专家共识。共识由7篇独立文章组成,包括共识推荐意见汇总1篇,涉及严重创伤、颅内出血或创伤性脑损伤、体外循环（cardiopulmonary bypass,CBP）手术、体外膜氧合、肿瘤性疾病和造血干细胞移植、急性肝功能衰竭或肝移植、非心脏手术、手术室外侵入性手术、脓毒症和（或）弥漫性血管内凝血共8类特殊危重人群的血浆和血小板输注的专家共识4篇及2篇补充内容分别为实验室检查、血浆和血小板的选择与处理、危重儿童血浆和血小板输注策略的研究优先事项,最终形成53项推荐意见,其中4项基于循证医学证据,5项良好实践声明及44项基于专家共识。该文仅介绍临床推荐意见。     

儿童脓毒性休克诊治专家共识--我们需要关注哪些变化

《儿童脓毒性休克（感染性休克）诊治专家共识（2015版）》已发表,是在国际指南的引领下,并结合国内外大量研究文献,在2006年制定的《儿科感染性休克（脓毒性休克）诊断治疗推荐方案》基础上,主要就儿童脓毒性休克定义、诊断和早期集束化治疗方案进行了部分修订。《儿童脓毒性休克（感染性休克）诊治专家共识（2015版）》的制定旨在指导临床一线医师对儿童脓毒性休克早期识别和早期积极干预,并进一步降低病死率和改善预后。     

对儿科急救医生实行输血治疗的调查

目的　调查儿科急救医生的输血行为。对象和方法　对儿科重症监护病房的医学院儿科急救医生进行自我管理的横断面调查。来自加拿大、法国、比利时、瑞典三级儿科重症监护病房 ,说英、法语医生的提纲式调查表。要求参加者回答在以下 4种疾病 :支气管炎、感染性休克、外伤和法洛式四联症 (现教科书及字典均为此称 )实施输血时的有关问题。结果　反馈率 71% (163 /2 3 0 )。促使急救医生考虑对此类患儿输血时 ,他们的血红蛋白为 7～ 13g/dl (70～ 13 0g/L)。各种疾病输血时平均血红蛋白值差异有显著意义 (P<0 .0 0 0 1)。低氧分压、高乳酸血症、高儿科死亡风险评分、消化道活动性出血、急诊手术和年龄 (<2周 )是决定输注红细胞的重要因素。参加问卷调查者的个人因素并无意义。 4种疾病输注悬浮红细胞的数量无显著差异。结论　该调查显示促使儿科急救医生在考虑输注红细胞时 ,血红蛋白标准明显不同 ,且实践方式差异有显著意义。输注悬浮红细胞多少也并非以调节血红蛋白的水平为目的。现标准输血成份中无压积红细胞之称 ,代以悬浮红细胞     

重度脱水体液复苏时应注意的一个问题

2009年第8期《中华儿科杂志》刊载了由中华医学会儿科学分会消化学组、中华医学会儿科学分会感染学组和《中华儿科杂志》编辑委员会联合组织有关专家制订的“儿童腹泻病诊断治疗原则的专家共识”（以下简称“共识”）。“共识”中对儿童腹泻病重度脱水的治疗介绍说：“静脉输液：采用静脉用的糖盐混合溶液（须在医院进行）：首先以2：1等张液20 ml/kg,于30～60 min内静脉推注或快速滴注以迅速增加血容量,改善循环和肾功能;在扩容后根据脱水性质……”。对此,金英姬等提出疑问：“中国腹泻病诊断治疗方案”中对腹泻病重度脱水患儿第一阶段静脉补液推荐选用的等张液有2:1液、生理盐水和平衡盐液[1];目前,对于小儿休克（包括感染性休克和低血容量性休克等）的体液复苏,主张用生理盐水、林格液等等张液体[2],儿童腹泻病重度脱水多有低血容量性休克,不知为何“共识”中只推荐使用2:1等张液,不用生理盐水？2010年第4期《中华儿科杂志》专家释疑栏目,对“儿童腹泻病诊断治疗原则的专家共识”的一点疑问及答复中解释说：“腹泻期间,水分和电解质（钠、氯化物、钾和碳酸盐）通过稀便大量丢失。     

第九届全国儿科危重症研讨会纪要

中华医学会儿科学分会急诊学组和中华医学会急诊医学分会儿科学组,于2006年9月7至10日在贵州省贵阳市召开了第九届全国儿科危重症研讨会。200余名来自全国22个省、市、自治区（含台湾）的代表参加会议。会议共收到论文186篇,12位专家作了专题讲座,37篇大会发言。论文涉题范围广,质量较往年有所提高,某些研究直指国际医学发展前沿。分组讨论半天（脓毒症、新生儿）,重点对近两年儿科危重症诊治的新进展、合理应用抗生素、儿科感染性休克（脓毒性休克）诊断治疗、以及当前普遍关心的医疗体制改革、医患关系等问题进行了深入讨论。[第一段]     

提升对儿童侵袭性肺部真菌感染的认识与实践

侵袭性肺部真菌感染是小儿呼吸系统疾病中不容忽视的重要一员,其基础疾病、诊断和防治涉及呼吸、血液、重症监护、感染、免疫和新生儿等多个专业以及影像、检验和临床药学等多个学科。中华医学会儿科学分会和中华儿科杂志编辑委员会组织多学科专家,共同探讨制订了“儿童侵袭性肺部真菌感染临床实践专家共识（2022版）”,对相关的18个临床...     

输血相关的循环超负荷及急性肺损伤

输血相关的循环超负荷（transfusion-associated circulatory overload,TACO）和输血相关的急性肺损伤（transfusion-related acute lung injury,TRALI）是大型外科手术中和重症监护病房危重症患儿常见的致死性输血并发症。TACO的特征是心源性肺水肿,而TRALI则主要为非心源性ARDS,二者在临床可能重叠出现,要将其清楚地区分比较困难。目前还没有特异性治疗方法。该文简述TACO和TRALI的概念、流行病学、病理生理、临床表现、诊断及可能的防治措施。     

滤除白细胞输血降低珠蛋白生成障碍性贫血患儿非溶血性发热性输血反应

目的了解滤白细胞红细胞（滤白）输注对降低重型β珠蛋白生成障碍性贫血（重型β地贫）患儿非溶血性发热性输血反应（FNHTR）的意义。方法2003年8月-2007年6月在本科输血治疗的重型β地贫患儿265例。男149例,女116例;患儿输血0.5-2.0单位/次。输血次数1-54次/人。悬浮红细胞（悬红）输注1992例次,共3114.5单位,滤白输注2416例次,共4354.5单位。滤白采用血站型滤白细胞红细胞器制作。悬红输注前根据体质量静泳注射2.5-5.0 mg地塞米松磷酸钠预防输血反应,滤白输注前不使用预防输血反应药物。应用SPSS 11.0软件进行统计学分析。结果悬红输注1 992例次中,发生FNHTR 49例次（2.460%）。滤白输注2 416例次中发生FNHTR 2例次（0.083%）。滤白输注FNHTR发生率显著低于悬红输注,差异有统计学意义（χ^2=53.95 P〈0.001）。结论输注滤白为重型β地贫患儿未来接受骨髓移植建立了安全的输血平台;重型β地贫患儿应输注滤白,以最大程度降低FNHTR发生率。     

体外膜氧合在儿童危重症应用的专家共识

10多年来,国内儿童重症领域的体外膜氧合（ECMO）技术发展迅速,已经成为危重症儿童挽救性治疗的关键策略,是体外生命支持系统的重要组成部分。该技术在我国发展尚不平衡,其技术规范和质量控制仍需进一步提高。为指导和规范我国儿童危重症 ECMO技术的临床实践形成本共识。     

Transfusion Related Acute Lung Injury in a Neonate

Transfusion related acute lung injury (TRALI) is a life threatening and potentially fatal complication of blood component transfusion, which largely remains under- diagnosed and under-reported, especially in neonates. The present case aims to emphasize that TRALI should be kept as a differential diagnosis in all groups of patients, including neonates, who develop acute respiratory distress and fresh lung infiltrations in the chest radiograph within 6?h of blood component transfusion in the absence of evidence of volume overload or cardiac dysfunction. Its recognition is important in view of the associated illness and death, for instituting correct management, and for eliminating implicated donors from donor panels.     

先天性红细胞生成异常性贫血Ⅰ型——1例报告暨文献复习

目的报告一例先天性红细胞生成异常性贫血并进行文献复习,了解该病的临床特点和诊治方法。方法报告病历和诊断思路并进行相关文献复习。结果先天性红细胞生成异常性贫血（CDAⅠ型）为遗传性疾病,临床表现为贫血、黄疸以及脾肿大,实验室检查可见正细胞或大细胞性贫血、骨髓幼红细胞核畸形及染色质异常、铁蛋白升高。特异性的诊断标准为红细胞电镜可见红细胞胞核呈海绵样或瑞士奶酪样改变。本病易与溶血性贫血等病相混淆。目前针对CDA的治疗包括：①输血;②脾脏切除;③骨髓移植;④干扰素。结论先天性红细胞生成异常性贫血是一组罕见的遗传性疾病,以骨髓红细胞成熟异常和原位溶血为特征,临床易误诊。特异性的诊断标准为红细胞电镜可见红细胞胞核呈海绵样或瑞士奶酪样改变,骨髓移植治疗是主要的治疗方法。     

Transfusion therapy in critically ill children

Critically ill children in pediatric intensive care units are commonly indicated for blood transfusion due to many reasons. Children are quite different from adults during growth and development, and that should be taken into consideration. It is very difficult to establish a universal transfusion guideline for critically ill children, especially preterm neonates. Treating underlying disease and targeted replacement therapy are the most effective approaches. Red blood cells are the first choice for replacement therapy in decompensated anemic patients. The critical hemoglobin concentration may be higher in critically ill children for many reasons. Whole blood is used only in the following conditions or diseases: (1) exchange transfusion; (2) after cardiopulmonary bypass; (3) extracorporeal membrane oxygenation; (4) massive transfusion, especially in multiple component deficiency. The characteristics of hemorrhagic diseases are so varied that their therapy should depend on the specific needs associated with the underlying disease. In general, platelet transfusion is not needed when a patient has platelet count greater than 10,000/mm3 and is without active bleeding, platelet functional deficiency or other risk factors such as sepsis. Patients with risk factors or age less than 4 months should be taken into special consideration, and the critical thrombocyte level will be raised. Platelet transfusion is not recommended in patients with immune-mediated thrombocytopenia or thrombocytopenia due to acceleration of platelet destruction without active bleeding or life-threatening hemorrhage. There are many kinds of plasma-derived products, and recombinant factors are commonly used for hemorrhagic patients due to coagulation factor deficiency depending on the characteristics of the diseases. The most effective way to correct disseminated intravascular coagulation (DIC) is to treat the underlying disease. Anticoagulant therapy is very important; heparin is the most common agent used for DIC but the results are usually not satisfactory. Antithrombin III, protein C, or recombinant thrombomodulin has been used successfully to treat this condition. For reducing the risk of organism transmission and adverse reactions resulting from blood transfusion, the following measures have been suggested: (1) replacement therapy using products other than blood (e.g., erythropoietin, iron preparation, granulocyte colony-stimulating factor); (2) special component replacement therapy for specific diseases; (3) autotransfusion; (4) subdividing whole packed blood products into smaller volumes to reduce donor exposure; (5) advances in virus-inactivating procedures. To avoid viral transmission, vapor-heated or pasteurized products and genetic recombinant products are recommended. Cytomegalovirus (CMV)-seronegative blood, leukoreduced and/or irradiated blood are recommended for prevention of CMV infection, graft-versus-host-disease and alloimmunization in neonate and immunocompromised patient transfusion. There is no reason to prescribe a plasma product for nutritional supplementation because of the risk of complications. The principle: complications of transfusion must be avoided, the rate of blood exposure should be reduced and the safety of the transfused agents or components should be maintained must always be kept in mind.     

PICU婴儿脓毒症及严重脓毒症临床特征及预后危险因素分析

目的 分析PICU婴儿脓毒症、严重脓毒症的临床特征,探讨影响其预后的危险因素.方法 对2006年1月至2011年12月我院PICU收治的141例脓毒症患儿运用回顾性病例对照（存活组与死亡组）的研究策略,以患儿入院日为研究起点,死亡或出院为终点,进行临床特点分析.并选择性别、年龄、是否有基础疾病、脓毒症严重程度、脏器受累数、入PICU当天的危重病评分、血生化指标（乳酸、白蛋白、血糖）、血气指标（碱剩余、碳酸氢根、pH值）、是否休克、培养阳性等14个变量,建立Logistic回归模型,分析预后的危险因素.结果 141例患儿的年龄1～12个月,其中脓毒症72例,严重脓毒症69例,死亡29例,病死率为20.6％.29例死亡患儿中,13例有基础疾病.脓毒症和严重脓毒症患儿的感染部位均以肺部为主,分别占47.2％（34/72）、47.8％（33/69）;血培养均有34例阳性,分别占47.2％ （34/72）、49.3％ （34/69）.单因素分析显示,性别、基础疾病、年龄、脓毒症严重程度、休克、脏器受累数、危重病例评分、血乳酸、血气pH值、碱剩余、碳酸氢根与脓毒症死亡有关.经逐步引入剔除法,建立Logistic回归模型,仍然与死亡相关的因素包括脓毒症严重程度（OR=22.5,95％CI=5.089,99.475）和脏器受累数（OR=3.305,95％CI=2.152,5.075）.结论 婴儿脓毒症严重程度越重和器官受累数越多,死亡风险越大.     

Transfusion of Blood and Components in Critically Ill Children

The physicians prescribing transfusions must have a thorough understanding of the various blood products, their indications and contraindications, and requirements for modification of the blood products to prevent probable adverse effects. Decision to give an RBC transfusion should not be based solely on Hb concentration, it should take in account high severity of illness; active bleeding; emergency surgery; etc. Using restrictive transfusion strategy of transfusion RBCs can decrease transfusion requirements without increasing adverse outcomes. In most circumstances, platelets should be maintained greater than 10 × 10⁹/L. Platelet counts greater than 20 × 10⁹/L are indicated for invasive procedures and greater than 50 × 10⁹/L for major surgeries or invasive procedures with risk of bleeding. Whenever possible, ABO-compatible platelets should be administered. Fresh frozen plasma should be transfused in multiple coagulation factor deficiencies, DIC with bleeding, replacement of rare single congenital factor deficiencies when specific concentrates are not available (e.g., protein C or factor II, V, X, XI, or XIII deficiency). During transfusion child should be monitored carefully.     

Transfusion transmitted diseases

Transfusion transmitted disease (TTD) is a major challenge to the transfusion services all over the world. The problem of TTD is directly proportionate to the prevalence of the infection in the blood donor community. In India, hepatitis B/C, HIV, malaria, syphilis, cytomegalo virus, parvo-virus B-19 and bacterial infections are important causes of concern. Hepatitis B and C infections are prevalent in India and carrier rate is about 1–5% and 1%, respectively. Post transfusion hepatitis B/C is a major problem in India (about 10%) because of low viraemia and mutant strain undetectable by routine ELISA. HIV prevalence among blood donors is different in various parts of the country. It may not be so alarming as projected by some agencies. In one study from north India, confirmed HIV positivity was found in 0.2/1000 blood donor. Post transfusion CMV is difficult to prevent but use of leukocyte filters may help to reduce it significantly. Parvo virus B-19 infection in blood donors is 39.9% which may increase morbidity in multitransfused or immunocompromised patients. Current symphilis tests may not be sensitive but it should be continued to exclude high-risk donors. Malaria is a real problem for India due to the lack of a simple and sensitive screening test. Incidence of bacterial contamination is greatly reduced due to improved collection/ preservation techniques and use of antibiotics in patients. However, proper vigilance and quality control is needed to prevent this problem. Total dependence of altruistic repeat voluntary donors and use of sensitive laboratory tests may help Indian blood transfusion services to reduce incidences of TTDs.     

Blood component therapy

The blood component support in pediatric patients is more challenging as compared to adult patients, as such, a thorough understanding of various blood components and indications for each is critical when making the decision for transfusion. Transfusion needs in pediatric group parallel the changes that accompany the transitions from fetus to neonate, neonate to infant, and throughout childhood. Modified or unmodified blood components viz. red blood cells, platelets, granulocytes, fresh frozen plasma and cryoprecipitate are required for transfusion support in pediatric population. In general, fetuses and infants younger than 4 months of age have specialized transfusion requirements whereas transfusion of infants older than 4 months and children parallels those for adults. Transfusion practices differ widely among pediatric care units depending upon individual preferences, hospital transfusion policy and resource availability. There is a need to implement best transfusion practices and despite the lack of firm evidences, existing pediatric transfusion guidelines can help pediatric care providers in their decisions related to component transfusion.     

Transfusion in Sickle Cell Disease: Experience from a Gujarat Centre

Objectives

Following impressions that the use of blood transfusion in sickle cell disease may be inappropriately high, transfusion practice at a major blood bank in an area of high prevalence of sickle cell disease was assessed.

Methods

Retrospective review of blood usage in sickle cell disease at a major blood bank in south Gujarat in 2010 was conducted with prospective more detailed data collection over 18 wk period (April 7 through August 15) in 2011. The results were compared with transfusion usage in the Jamaican Sickle Cell Clinic.

Results

In 2010, this blood bank processed a total of 19,037 units of which 384 (5.2 %) units were for patients with sickle cell disease. Median transfusion use was 1 unit but 16 patients (4.2 %) of those transfused received 10 units or more and five patients received over 20 units. More detailed prospective analysis revealed that most transfusions occurred between ages 5–15 y, 40 % of subjects had pretransfusion hemoglobin levels below 6 g/dL, symptoms were generally vague such as fever, bone pain, weakness and that 26 % denied any specific symptoms.

Conclusions

Transfusion usage greatly exceeds that in the Jamaican Sickle Cell Clinic. Transfusion therapy carries risks and cost and more detailed investigation and diagnosis of anemic episodes is necessary to define the role of transfusion among other potential therapies. Eventually, guidelines evolved by Indian specialists should determine the indications for transfusion in sickle cell disease.