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Ten years of a neonatal screening program for hemoglobinopathies in Friuli-Venezia Giulia: first regional experience in Italy. 弗留利-威尼斯朱利亚地区新生儿血红蛋白病筛查项目十年:意大利首个地区经验。
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2023-11-30 DOI: 10.2450/BloodTransfus.646
Epifania R Testa, Margherita Robazza, Francesca Barbieri, Laura Travan, Maria P Miani, Elisabetta Miorin, Ingrid Toller, Danica Dragovic, Valentina Moretti, Stefano Facchin, Patrizia Valeri, Luciana Geremia, Valeria Brunetta, Roberto Dall'Amico, Andrea Bontadini

Background: Hemoglobinopathies are the commonest genetic defect worldwide (7% of the world's population has at least one hemoglobin mutation). Although prenatal screening for hemoglobinopathies is not obligatory during pregnancy in Italy, it is offered to women by the Italian National Health Service in the pre-conception phase. The screening of newborns is a valid alternative, and has been adopted in various European countries, albeit in a piecemeal fashion. Neonatal screening has the advantage of providing early diagnosis of a hemoglobinopathy. Here we report the findings from the experience with neonatal screening in Friuli-Venezia Giulia since 2010.

Materials and methods: The hemoglobinopathy screening project in Friuli-Venezia Giulia, a Region in north Italy, began in November 2010. High-performance liquid chromatography was performed on dried blood spot samples collected by obstetric nurses from neonates within 5-8 days after birth.

Results: From 2010 to 2019, 11,956 newborns were screened, and abnormal hemoglobin was found in 519 of them (4.34%): the variants identified included HbS, HbC, HbD, HbE and HbX. More specifically, the HbS variant was observed in 347 (2.9%) newborns and the homozygous pattern was identified in 24 (0.2%) cases. The screening also detected two cases of β-thalassemia major.

Discussion: We report our experience of 10 years of screening newborns for hemoglobinopathies in the Region of Friuli-Venezia Giulia, in which 7.7% of people come from malaria-endemic areas. Increased mobility and migratory flows bringing in hemoglobinopathy carriers from endemic areas have led to an increase in mutations in non-malarial countries, with a current incidence of around 4% in the newborns we tested. This means that hemoglobinopathies can be described as a rare condition. Our data show that incidence rates are comparable to those of other inherited disorders such as phenylketonuria, thereby justifying the inclusion of the test for hemoglobinopathies into screening programs for rare diseases.

背景:血红蛋白病是全球最常见的遗传缺陷(全球 7% 的人口至少有一种血红蛋白变异)。虽然在意大利,血红蛋白病的产前筛查并不是怀孕期间的必做项目,但意大利国家医疗服务机构在孕前阶段就为妇女提供了筛查服务。对新生儿进行筛查是一种有效的替代方法,欧洲各国已经采用了这种方法,尽管是以零敲碎打的方式。新生儿筛查的优点是能及早诊断出血红蛋白病。在此,我们报告了弗留利-威尼斯朱利亚省自 2010 年以来开展新生儿筛查的结果:意大利北部弗留利-威尼斯朱利亚大区的血红蛋白病筛查项目始于 2010 年 11 月。对产科护士从新生儿出生后 5-8 天内采集的干血斑样本进行了高效液相色谱分析:从 2010 年到 2019 年,共筛查了 11956 名新生儿,其中 519 名(4.34%)发现血红蛋白异常:发现的变异包括 HbS、HbC、HbD、HbE 和 HbX。更具体地说,在 347 例(2.9%)新生儿中发现了 HbS 变异,在 24 例(0.2%)中发现了同型变异。筛查还发现了两例重型β地中海贫血:我们报告了弗留利-威尼斯朱利亚地区 10 年来对新生儿进行血红蛋白病筛查的经验,该地区 7.7% 的人口来自疟疾流行区。来自疟疾流行地区的血红蛋白病携带者的流动性和移民潮的增加导致非疟疾国家的突变率上升,目前在我们检测的新生儿中,突变率约为 4%。这意味着血红蛋白病可以说是一种罕见病。我们的数据显示,血红蛋白病的发病率与苯丙酮尿症等其他遗传性疾病的发病率相当,因此有理由将血红蛋白病检测纳入罕见病筛查计划。
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引用次数: 0
Assessment of metabolic and hemostatic profile of apheresis platelet concentrates: does the storage medium play a role? 离心血小板浓缩物的代谢和止血情况评估:储存介质是否起作用?
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-07-30 DOI: 10.2450/BloodTransfus.800
Eleni Petrou, Stavros Tsalas, Andreas G Tsantes, Electra Loukopoulou, Sofia Mellou, Sotirios P Fortis, Evdoxia Rapti, Rozeta Sokou, Elias Kyriakou, Panagiota Douramani, Frantzeska Frantzeskaki, George Samonis, Styliani Kokoris, Anastasios Kriebardis, Argirios E Tsantes

Background: The impact of pathogen reduction technology (PRT) on metabolic and hemostatic profile of treated platelets remains a subject of debate. Platelets Additive Solutions (PASs) are suggested as more appropriate storage medium compared to plasma. To investigate this in terms of zero heterogeneity PRT-treated and control apheresis platelet concentrates (PCs), collected from the same donors and stored in PAS and plasma respectively, were analyzed.

Materials and methods: In the first arm of the study six double dose-apheresis PCs were produced, split and stored in plasma, while in the second arm six split double dose-apheresis PCs from the same donors, were produced and stored in PAS. Control and PRT-treated PCs resulted in both arms. Metabolic and hemostatic markers were evaluated in all the examined groups on days 1, 3 and 5.

Results: A time dependent increased metabolism both in PAS and plasma-stored PCs was evident in PRT-treated PCs. However, the metabolic profile was better preserved in PCs stored in PAS, as higher pH (6.8 vs 6.5, p=0.007) and lower lactate levels (12.6 vs 17.8 mmol/L, p=0.009) were documented in PRT-treated PAS-PCs compared to plasma-PCs, on day 5. A time dependent decreased hemostatic capacity regardless the storage medium was evident in PRT-treated PCs, (PAS-PCs MCF, p=0.004 and plasma-PCs MCF, p=0.007). Similar results were obtained in control PCs.

Discussion: The use of PAS preserves the metabolic profile of PCs more adequately compared to plasma but has no effect on the hemostatic profile. The clinical relevance of these findings needs further investigation.

背景:减少病原体技术(PRT)对经处理血小板的代谢和止血情况的影响仍是一个争论的话题。与血浆相比,血小板添加剂溶液(PAS)被认为是更合适的储存介质。为了从零异质性的角度研究这一点,我们对从相同捐献者处收集并分别储存在血小板添加剂溶液和血浆中的经血小板添加剂溶液处理过的血小板浓缩物(PC)和对照组血小板浓缩物(PC)进行了分析:在第一组研究中,生产了六份双剂量血凝血小板浓缩液,将其拆分并储存在血浆中;在第二组研究中,生产了六份来自同一供体的双剂量血凝血小板浓缩液,将其拆分并储存在 PAS 中。对照组和经 PRT 处理的 PC 均在两组中产生。在第 1、3 和 5 天,对所有受检组的代谢和止血指标进行了评估:结果:经 PRT 处理的 PC 中,PAS 和血浆储存 PC 中的新陈代谢均明显增加,这与时间有关。然而,经 PRT 处理的 PAS PC 与血浆 PC 相比,在第 5 天,PAS PC 的 pH 值(6.8 vs 6.5,p=0.007)更高,乳酸水平(12.6 vs 17.8 mmol/L,p=0.009)更低,因此 PAS PC 的新陈代谢情况得到了更好的保护。经 PRT 处理的 PCs(PAS-PCs MCF,p=0.004;血浆-PCs MCF,p=0.007)的止血能力明显下降,与储存介质无关(PAS-PCs MCF,p=0.004;血浆-PCs MCF,p=0.007)。对照组 PC 也得到了类似的结果:讨论:与血浆相比,PAS 能更充分地保留 PC 的代谢特征,但对止血特征没有影响。这些发现的临床意义需要进一步研究。
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引用次数: 0
In vitro regenerative effects of a pooled pathogen-reduced lyophilized human cord blood platelet lysate for wound healing applications. 用于伤口愈合的集合病原体还原冻干人脐带血血小板裂解液的体外再生效果。
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-09-16 DOI: 10.2450/BloodTransfus.755
Marianna Buscemi, Aida Cavallo, Marco Fabbri, Sabrina Gabbriellini, Elena Ciabatti, Alessandro Mazzoni, Giorgio Soldani, Paolo Rebulla, Paola Losi

Background: Cord blood platelets, easily obtained from blood units not suitable for hematopoietic stem cell transplantation, represent an abundant source of growth factors for use in wound healing. Although several protocols have been described for platelet lysate production, no standard manufacturing protocol is available. The use of pooled cord blood platelets could thus facilitate standardization. In this study, the effect of varying concentrations (up to 20%) of a pooled pathogen-reduced lyophilized cord blood platelet lysate (PRL-CBPL) was investigated in different cell types involved in the wound healing process. The effect of heparin addition was also evaluated. In parallel, a comparison was performed with a single donor cord blood platelet lysate (SD-CBPL).

Materials and methods: The effect of PRL-CBPL on the viability and proliferation of different cell lines (L929 mouse fibroblasts and HaCaT keratinocytes) and human primary cells (fibroblasts-NHDF, coronary artery smooth muscle cells-HCASMC and coronary artery endothelial cells-HCAEC), on HaCaT migration and the chemotactic effect on human monocytes (THP-1) was evaluated.

Results: PRL-CBPL showed a lower PDGF-AB amount compared to SD-CBPL. Differing concentrations of both CBPL were necessary to influence cell viability and proliferation. 3% was the optimal concentration for L929 and HaCaT as well as for NHDF and HCASMC, while HCAEC required 10%. The effect of added heparin was more evident on SD-CBPL and in particular on NHDF and HCASMC proliferation. Keratinocyte scratch closure was obtained with 3 and 5% PRL-CBPL and SD-CBPL respectively. Both CBPLs caused an increase in the number of migrated THP-1 monocytes in a concentration-dependent manner up to 20% with a higher monocyte migration for SD-CBPL with respect to PRL-CBPL and in cells treated with heparin.

Discussion: The data obtained suggest that PRL-CBPL is an effective standardized alternative to SD-CBPL.

背景:脐带血血小板易于从不适合造血干细胞移植的血液单位中获得,是伤口愈合中使用的生长因子的丰富来源。虽然已有几种血小板裂解液生产方案,但还没有标准的生产方案。因此,使用汇集的脐带血血小板有助于实现标准化。本研究调查了不同浓度(最多 20%)的集合病原体还原冻干脐带血血小板裂解液(PRL-CBPL)对伤口愈合过程中不同类型细胞的影响。此外,还评估了添加肝素的效果。同时,还与单一供体脐带血血小板裂解液(SD-CBPL)进行了比较:评估了 PRL-CBPL 对不同细胞系(L929 小鼠成纤维细胞和 HaCaT 角质细胞)和人原代细胞(成纤维细胞-NHDF、冠状动脉平滑肌细胞-HCASMC 和冠状动脉内皮细胞-HCAEC)的活力和增殖、HaCaT 迁移和对人单核细胞(THP-1)的趋化作用的影响:结果:与 SD-CBPL 相比,PRL-CBPL 的 PDGF-AB 量更低。影响细胞活力和增殖所需的两种 CBPL 浓度不同。3% 是 L929 和 HaCaT 以及 NHDF 和 HCASMC 的最佳浓度,而 HCAEC 则需要 10%。添加肝素对 SD-CBPL,特别是对 NHDF 和 HCASMC 增殖的影响更为明显。3%和 5%的 PRL-CBPL 和 SD-CBPL 可分别获得角质细胞划痕闭合。两种 CBPL 都能以浓度依赖性的方式增加 THP-1 单核细胞的迁移数量,最高可达 20%,其中 SD-CBPL 的单核细胞迁移率高于 PRL-CBPL 和肝素处理过的细胞:讨论:获得的数据表明,PRL-CBPL 是 SD-CBPL 的有效标准化替代品。
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引用次数: 0
Blood transfusion-associated anaphylaxis in perioperative- and non-perioperative patients in Western Norway 2002-2021. 2002-2021 年挪威西部围手术期和非围手术期患者输血相关过敏性休克。
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-05-15 DOI: 10.2450/BloodTransfus.738
Bjarte Skoe Erikstein, Marie Bjørbak Alnæs, Torunn Oveland Apelseth

Background: Anaphylaxis after blood transfusion is a feared complication accounting for severe morbidity. A retrospective study was performed at Haukeland University Hospital, Bergen, Norway, to investigate the rate and features of transfusion-associated anaphylaxis (TAA) occurring between 2002-2021.

Materials and methods: Identified cases of TAA were studied by an immunologist and an allergist to extract information about general characteristics, amplifying factors, co-morbidity, treatment, and treatment responses. TAA was reported as perioperative or non-perioperative.

Results: We identified 29 cases of TAA: 13 perioperative and 16 non-perioperative. Allergic transfusion reaction had an incidence rate of 34/100,000 transfusions and TAA a rate of 7/100,000 transfusions. The incidence of allergic reactions and TAA increased 2.6- and 6.4-fold during the study period. The first perioperative TAA was discovered 12 years into the study period but was equally frequent as non-perioperative transfusion-associated anaphylaxis in the last five years of the study period. 52% of the TAA cases had relevant co-morbidity and 100% of them had amplifying factors. Although only 38% of the non-perioperative patients received epinephrine as treatment, 94% of them had a good treatment response to their total treatment regimen. Poorer treatment response was observed with higher age, more cardiovascular- and respiratory disease, higher use of amplifying and sedating medications and a higher severity score.

Discussion: Our findings indicate that TAA, especially in the perioperative setting, is underdiagnosed. The increased incidence of TAA in our study is temporally related to the introduction of a national hemovigilance program, introduction of standardized laboratory testing for anaphylaxis and increased multidisciplinary focus on the condition. In conclusion, increased awareness of TAA, and especially in the perioperative setting, is needed. A multidisciplinary approach is necessary to improve identification and reporting of TAA.

背景:输血后过敏性休克是一种可怕的并发症,会导致严重的发病率。挪威卑尔根的豪克兰大学医院开展了一项回顾性研究,调查2002-2021年间发生的输血相关过敏性休克(TAA)的发病率和特征:一名免疫学家和一名过敏学家对确定的 TAA 病例进行了研究,以提取有关一般特征、放大因素、并发症、治疗和治疗反应的信息。TAA 按围手术期或非围手术期报告:结果:我们发现了 29 例 TAA:13 例围手术期,16 例非围手术期。过敏性输血反应的发生率为 34/100,000,TAA 的发生率为 7/100,000。在研究期间,过敏反应和TAA的发生率分别增加了2.6倍和6.4倍。首例围手术期 TAA 是在研究期间的 12 年后发现的,但在研究期间的最后 5 年,其发生率与非围手术期输血相关过敏性休克相同。52% 的 TAA 病例有相关并发症,100% 的病例有扩大因素。虽然只有 38% 的非手术患者接受了肾上腺素治疗,但其中 94% 的患者对其总体治疗方案反应良好。年龄越大、心血管和呼吸系统疾病越多、使用扩容和镇静药物越多以及严重程度评分越高,治疗反应越差:讨论:我们的研究结果表明,TAA,尤其是围手术期的 TAA 诊断不足。在我们的研究中,TAA 发生率的增加与国家血液监测计划的引入、过敏性休克标准化实验室检测的引入以及多学科对该病症关注度的提高有关。总之,需要提高对 TAA 的认识,尤其是在围手术期。有必要采用多学科方法来改进 TAA 的识别和报告。
{"title":"Blood transfusion-associated anaphylaxis in perioperative- and non-perioperative patients in Western Norway 2002-2021.","authors":"Bjarte Skoe Erikstein, Marie Bjørbak Alnæs, Torunn Oveland Apelseth","doi":"10.2450/BloodTransfus.738","DOIUrl":"10.2450/BloodTransfus.738","url":null,"abstract":"<p><strong>Background: </strong>Anaphylaxis after blood transfusion is a feared complication accounting for severe morbidity. A retrospective study was performed at Haukeland University Hospital, Bergen, Norway, to investigate the rate and features of transfusion-associated anaphylaxis (TAA) occurring between 2002-2021.</p><p><strong>Materials and methods: </strong>Identified cases of TAA were studied by an immunologist and an allergist to extract information about general characteristics, amplifying factors, co-morbidity, treatment, and treatment responses. TAA was reported as perioperative or non-perioperative.</p><p><strong>Results: </strong>We identified 29 cases of TAA: 13 perioperative and 16 non-perioperative. Allergic transfusion reaction had an incidence rate of 34/100,000 transfusions and TAA a rate of 7/100,000 transfusions. The incidence of allergic reactions and TAA increased 2.6- and 6.4-fold during the study period. The first perioperative TAA was discovered 12 years into the study period but was equally frequent as non-perioperative transfusion-associated anaphylaxis in the last five years of the study period. 52% of the TAA cases had relevant co-morbidity and 100% of them had amplifying factors. Although only 38% of the non-perioperative patients received epinephrine as treatment, 94% of them had a good treatment response to their total treatment regimen. Poorer treatment response was observed with higher age, more cardiovascular- and respiratory disease, higher use of amplifying and sedating medications and a higher severity score.</p><p><strong>Discussion: </strong>Our findings indicate that TAA, especially in the perioperative setting, is underdiagnosed. The increased incidence of TAA in our study is temporally related to the introduction of a national hemovigilance program, introduction of standardized laboratory testing for anaphylaxis and increased multidisciplinary focus on the condition. In conclusion, increased awareness of TAA, and especially in the perioperative setting, is needed. A multidisciplinary approach is necessary to improve identification and reporting of TAA.</p>","PeriodicalId":49260,"journal":{"name":"Blood Transfusion","volume":" ","pages":"502-513"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11576146/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141181428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Identification of the novel c.300C>G variation on the ABO*A1.02 allele associated with an AweakB phenotype. 鉴定与 A 弱 B 表型相关的 ABO*A1.02 等位基因 c.300C>G 变异。
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-02-28 DOI: 10.2450/BloodTransfus.642
Yuqing Shen, Junshun Gong, Yuyu Zhang, Naizhu Su, Lou Can, Jiaming Li, Dong Xiang, Xiaohong Cai, Hang Lei
{"title":"Identification of the novel c.300C>G variation on the ABO*A1.02 allele associated with an A<sub>weak</sub>B phenotype.","authors":"Yuqing Shen, Junshun Gong, Yuyu Zhang, Naizhu Su, Lou Can, Jiaming Li, Dong Xiang, Xiaohong Cai, Hang Lei","doi":"10.2450/BloodTransfus.642","DOIUrl":"10.2450/BloodTransfus.642","url":null,"abstract":"","PeriodicalId":49260,"journal":{"name":"Blood Transfusion","volume":" ","pages":"475-480"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11576153/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140337449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Inclusion of cryoprecipitate, pathogen-reduced, in the WHO model lists of essential medicines for adults and children: a call for action. 将减少病原体的低温沉淀纳入世卫组织成人和儿童基本药物示范清单:呼吁采取行动。
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-01-31 DOI: 10.2450/BloodTransfus.687
Jay S Epstein, Yuyun Maryuningsih, Jean-Claude Faber, W Martin Smid, Thierry Burnouf
{"title":"Inclusion of cryoprecipitate, pathogen-reduced, in the WHO model lists of essential medicines for adults and children: a call for action.","authors":"Jay S Epstein, Yuyun Maryuningsih, Jean-Claude Faber, W Martin Smid, Thierry Burnouf","doi":"10.2450/BloodTransfus.687","DOIUrl":"10.2450/BloodTransfus.687","url":null,"abstract":"","PeriodicalId":49260,"journal":{"name":"Blood Transfusion","volume":" ","pages":"481-483"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11576152/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139693364","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Development and evaluation of trigger tools to identify pediatric blood management errors. 开发和评估用于识别儿科血液管理错误的触发工具。
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-27 DOI: 10.2450/BloodTransfus.606
Swaminathan Kandaswamy, Cassandra D Josephson, Margo R Rollins, Jennifer Jones, Patricia Zerra, Ruchika Goel, Jennifer Andrews, Jeanne E Hendrickson, Lani Lieberman, Evan W Orenstein

Background: Pediatric Patient Blood Management (PBM) programs require continuous surveillance of errors and near misses. However, most PBM programs rely on passive surveillance methods. Our objective was to develop and evaluate a set of automated trigger tools for active surveillance of pediatric PBM errors.

Materials and methods: We used the Rand-UCLA method with an expert panel of pediatric transfusion medicine specialists to identify and prioritize candidate trigger tools for all transfused blood products. We then iteratively developed automated queries of electronic health record (EHR) data for the highest priority triggers. Two physicians manually reviewed a subset of cases meeting trigger tool criteria and estimated each trigger tool's positive predictive value (PPV). We then estimated the rate of PBM errors, whether they reached the patient, and adverse events for each trigger tool across four years in a single pediatric health system.

Results: We identified 28 potential triggers for pediatric PBM errors and developed 5 automated trigger tools (positive patient identification, missing irradiation, unwashed products despite prior anaphylaxis, transfusion lasting >4 hours, over-transfusion by volume). The PPV for ordering errors ranged from 38-100%. The most frequently detected near miss event reaching patients was first transfusions without positive patient identification (estimate 303, 95% CI: 288-318 per year). The only adverse events detected were from over-transfusions by volume, including 4 adverse events detected on manual review that had not been reported in passive surveillance systems.

Discussion: It is feasible to automatically detect pediatric PBM errors using existing data captured in the EHR that enable active surveillance systems. Over-transfusions may be one of the most frequent causes of harm in the pediatric environment.

背景:儿科患者血液管理 (PBM) 计划需要持续监控错误和险情。然而,大多数 PBM 项目都依赖于被动监控方法。我们的目标是开发和评估一套自动触发工具,用于主动监控儿科 PBM 错误:我们使用兰德-加州大学洛杉矶分校的方法,由儿科输血医学专家组成专家小组,对所有输血产品的候选触发工具进行识别和优先排序。然后,我们对电子健康记录(EHR)数据进行迭代式自动查询,找出优先级最高的触发工具。两名医生人工审核符合触发工具标准的病例子集,并估算每种触发工具的阳性预测值 (PPV)。然后,我们估算了在一个儿科医疗系统中,每种触发工具在四年内的 PBM 错误率、是否到达患者以及不良事件的发生率:我们确定了 28 种潜在的儿科 PBM 错误触发因素,并开发了 5 种自动触发工具(阳性患者识别、缺失照射、之前发生过敏性休克但未清洗产品、输血持续时间超过 4 小时、按输血量计算输血过量)。排序错误的 PPV 在 38-100% 之间。患者最常发生的近乎失误事件是首次输血时患者身份未得到确认(估计为 303 例,95% CI:288-318 例/年)。检测到的唯一不良事件是按输血量计算的过度输血,其中包括人工审核发现的 4 起被动监测系统未报告的不良事件:讨论:利用电子病历中捕获的现有数据自动检测儿科 PBM 错误是可行的,这使得主动监测系统成为可能。过度输血可能是儿科环境中最常见的伤害原因之一。
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引用次数: 0
Genomic characterization of clinically significant blood group variants in Aboriginal Australians. 澳大利亚原住民中具有临床意义的血型变异的基因组特征。
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-11 DOI: 10.2450/BloodTransfus.664
Sudhir Jadhao, Candice Davison, Eileen Roulis, Simon Lee, Tamika Campbell, Reece Griffin, Maree Toombs, Alex Brown, Maree Perry, Bushra Nasir, David O Irving, Catherine A Hyland, Robert L Flower, Shivashankar H Nagaraj

Background: Hematological disorders are often treated with blood transfusions. Many blood group antigens and variants are population-specific, and for patients with rare blood types, extensive donor screening is required to find suitable matches for transfusion. There is a scarcity of knowledge regarding blood group variants in Aboriginal Australian populations, despite a higher need for transfusion due to the higher prevalence of renal diseases and anemia.

Materials and methods: In this study, we applied next-generation sequencing and analysis to 245 samples obtained from Aboriginal Australians from South-East Queensland, to predict antigen phenotypes for 36 blood group systems.

Results: We report potential weak antigens in blood group systems RH, FY and JR that have potential clinical implications in transfusion and pregnancy settings. These include partial DIII type 4, weak D type 33, and Del RHD (IVS2-2delA). The rare Rh phenotypes D+ C+ E+ c- e+ and D+ C+ E+ c+ e- were also detected.

Discussion: The comprehensive analyses of blood group genetic variant profiles identified in this study will provide insight and an opportunity to improve Aboriginal health by aiding in the identification of appropriate blood products for population-specific transfusion needs.

背景:血液病通常通过输血来治疗。许多血型抗原和变异体都具有人群特异性,对于稀有血型患者,需要对捐献者进行广泛筛查,以找到合适的配型进行输血。尽管澳大利亚土著居民因肾病和贫血发病率较高而需要输血,但他们对血型变异的了解却很少:在这项研究中,我们对从昆士兰东南部澳大利亚原住民那里获得的 245 份样本进行了新一代测序和分析,以预测 36 种血型系统的抗原表型:我们报告了血型系统 RH、FY 和 JR 中潜在的弱抗原,这些抗原对输血和妊娠有潜在的临床影响。这些抗原包括部分 DIII 型 4、弱 D 型 33 和 Del RHD(IVS2-2delA)。此外,还发现了罕见的 Rh 表型 D+ C+ E+ c- e+ 和 D+ C+ E+ c+ e-:本研究中发现的血型基因变异图谱的综合分析将提供洞察力和机会,通过帮助识别适合特定人群输血需求的血液制品来改善原住民的健康状况。
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引用次数: 0
Implementing Patient Blood Management in major digestive surgery: should we do more? 在大型消化外科手术中实施患者血液管理:我们是否应该做得更多?
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-07-22 DOI: 10.2450/BloodTransfus.828
Marco Catarci
{"title":"Implementing Patient Blood Management in major digestive surgery: should we do more?","authors":"Marco Catarci","doi":"10.2450/BloodTransfus.828","DOIUrl":"10.2450/BloodTransfus.828","url":null,"abstract":"","PeriodicalId":49260,"journal":{"name":"Blood Transfusion","volume":" ","pages":"551-552"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11576143/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141972196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The new SoHO Regulation. What should the blood system expect? 新的《SOHO条例》。血液系统应该做些什么?
IF 2.4 3区 医学 Q2 HEMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-09-30 DOI: 10.2450/BloodTransfus.889
Simonetta Pupella
{"title":"The new SoHO Regulation. What should the blood system expect?","authors":"Simonetta Pupella","doi":"10.2450/BloodTransfus.889","DOIUrl":"10.2450/BloodTransfus.889","url":null,"abstract":"","PeriodicalId":49260,"journal":{"name":"Blood Transfusion","volume":" ","pages":"461-463"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11576147/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142511375","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Blood Transfusion
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