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Management of a Young Patient With High-risk Multiple Myeloma Complicated by Acquired von Willebrand Syndrome: A Diagnostic and Therapeutic Emergency 对一名并发获得性冯-维勒布兰德综合征的高危多发性骨髓瘤年轻患者的管理:诊断和治疗急诊
IF 0.3 Q3 Medicine Pub Date : 2024-01-01 DOI: 10.1177/26348535231222994
Abakarim Ouadii, Condom Pauline, Voisin Sophie, Huart Antoine, Perrot Aurore, Botin Teresa
Multiple myeloma associated with bleeding events secondary to von Willebrand syndrome is underdiagnosed. The management of this entity is highly complex, and aims to control the hemorrhagic syndrome and reduce plasma viscosity with plasmapheresis and multiple myeloma-specific treatment. The authors report the rare case of a young patient with high-risk multiple myeloma complicated by hyperviscosity syndrome and presenting an acquired von Willebrand syndrome with hemorrhagic manifestations, requiring urgent therapeutic management to save the patient's life.
继发于von Willebrand综合征并伴有出血事件的多发性骨髓瘤诊断不足。这种疾病的治疗非常复杂,目的是通过血浆置换术和多发性骨髓瘤特异性治疗控制出血综合征并降低血浆粘度。作者报告了一例罕见的高危多发性骨髓瘤年轻患者的病例,该患者并发高粘度综合征,并出现了伴有出血表现的获得性冯-维勒布兰德综合征,需要紧急治疗以挽救患者的生命。
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引用次数: 0
Biochemical and Functional Characterization of Plasma Derived Human Celestrase 1 血浆衍生的人 Celestrase 1 的生化和功能特性分析
IF 0.3 Q3 Medicine Pub Date : 2024-01-01 DOI: 10.1177/26348535231225317
Sheetal Dolia, Sachin Verma, Suma Ray
C1-esterase inhibitor (C1-INH) is a protein derived from fresh frozen human plasma and is widely used in the treatment of Hereditary Angioedema. C1-INH product must have high purity with preserved functionality. The aim of the study was to perform extensive characterization of human C1-esterase inhibitor (Celestrase 1) with special emphasis on functionality of the protein by different mechanisms along with its purity and structural elucidation. Comparative study. This study describes the purification and characterization of an economically viable, highly pure and efficient human plasma-derived C1-INH prepared from cryopoor plasma by combination of chromatography steps, capable of removing protein contaminants. The purification process includes two orthogonal virus clearance steps -solvent detergent and virus retentive filtration and further, characterized by various biochemical and functional assays along with other commercially available entities. The developed process yields 0.75  ±  0.1 vials of C1-INH /L of cryopoor plasma with 44.4  ±  3.6% overall process recovery. Celestrase 1 shows comparability with an existing market comparator, with respect to its purity by different methods. Celestrase 1 proved its functionality in binding irreversibly to the complement protein by classical pathway of the complement system and in the Kallikrein system. Additionally, the antigen-to-biologic activity ratio an indicative of the functionality for Celestrase 1 (0.94) was found comparable to market comparator (0.79). Identity of the product was confirmed by Western blot analysis. The structural analysis of Celestrase 1 was found to be similar to market comparator and exists predominantly in α-helix secondary structure by Far UV Circular Dichroism (CD). For the treatment of Hereditary Angioedema (HAE), the current study presents a pure, safe, and functionally efficient product that can meet the therapeutic needs of patients deficient in C1-INH.
C1-esterase 抑制剂(C1-INH)是一种提取自新鲜冷冻人血浆的蛋白质,广泛用于治疗遗传性血管性水肿。C1-INH 产品必须具有高纯度并保留其功能性。这项研究的目的是对人 C1-酯酶抑制剂(Celestrase 1)进行广泛的表征,特别强调该蛋白质通过不同机制发挥的功能以及它的纯度和结构阐明。比较研究。本研究介绍了一种经济可行、高纯度和高效率的人血浆衍生 C1-INH 的纯化和特性分析,该 C1-INH 由低温血浆制备而成,结合了色谱步骤,能够去除蛋白质杂质。纯化过程包括两个正交的病毒清除步骤--溶剂洗涤和病毒保留过滤。所开发的工艺可获得 0.75 ± 0.1 瓶/升低温血浆的 C1-INH,总体工艺回收率为 44.4 ± 3.6%。Celestrase 1 通过不同的方法显示出其纯度与市场上现有的参照物具有可比性。Celestrase 1 通过补体系统的经典途径和 Kallikrein 系统证明了其与补体蛋白不可逆结合的功能。此外,表明 Celestrase 1 功能的抗原生物活性比(0.94)与市场上的同类产品(0.79)相当。通过 Western 印迹分析确认了产品的身份。通过远紫外光圆二色性(CD)分析,发现 Celestrase 1 的结构与市场上的同类产品相似,主要存在于 α-螺旋二级结构中。对于遗传性血管性水肿(HAE)的治疗,目前的研究提出了一种纯净、安全、功能高效的产品,可以满足 C1-INH 缺乏患者的治疗需求。
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引用次数: 0
Effects of Tariff on the Management of Blood Products in Fars Province Hospitals 关税对法尔斯省医院血液制品管理的影响
IF 0.3 Q3 Medicine Pub Date : 2024-01-01 DOI: 10.1177/26348535231225315
Rohollah Baktashi, Fariba Rad, Masoud Torabi Ardekani, Alireza Salah, Hossein Anani, R. M. Khalilabadi
In Iran, blood products were being delivered to healthcare centers without charge. For optimum usage and reducing wastage, the tariff was set on October 23, 2015. Thus, hospitals must pay for blood products to blood transfusion services and then the expenses are paid to healthcare centers by the insurance. This study aimed to explore the effects of the tariff policy on utilization pattern of blood products in healthcare centers of Fars province, Iran. This is a retrospective study which analyzes the recorded data. Throughout this retrospective study, the number of units requested, delivered, returned, and return/delivery ratio of RBC, FFP, cryoprecipitate, and platelet were examined before and after the tariff in different cities of Fars province of Iran. After the tariff, return/delivery ratios of FFP, cryoprecipitate, and platelet increased in comparison with before tariff but were not statistically significant which means the return of unused units was increased after imposing tariff. The results of this investigation showed that tariff may lead to more return of FFP, cryoprecipitate, and platelet units to the transfusion services of Fars province. More research must be performed to further approve it.
在伊朗,血液制品被免费运送到医疗中心。为实现最佳使用并减少浪费,2015 年 10 月 23 日制定了收费标准。因此,医院必须向输血服务机构支付血液制品费用,然后由保险公司向医疗中心支付费用。本研究旨在探讨收费政策对伊朗法尔斯省医疗中心血液制品使用模式的影响。这是一项回顾性研究,对记录的数据进行分析。在这项回顾性研究中,对伊朗法尔斯省不同城市在实行收费政策前后的红细胞、全血细胞生成素、低温沉淀和血小板的申请单位数、交付单位数、退回单位数以及退回/交付比率进行了调查。征收关税后,FFP、低温沉淀和血小板的返还/交付比率与征收关税前相比有所增加,但在统计上并不显著,这意味着征收关税后未使用单位的返还有所增加。调查结果表明,关税可能会导致更多的 FFP、低温沉淀和血小板单位返回法尔斯省的输血服务机构。为进一步证实这一点,必须开展更多的研究。
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引用次数: 0
Characterization of the Components and Functional Properties of a Plasma-Derived Fibrin Sealant 血浆源性纤维蛋白密封胶的组成和功能特性研究
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.1177/26348535231171332
Ilaria Ramaiola, Laura Lopez, Manuel Moron, Pau Maduell, Maite López, Nuria Marzo, Salvador Grancha
Background The activity and purity of Fibrin Sealant Grifols are described in this article. This fibrin sealant contains two components (fibrinogen and thrombin) that mimic the final stages of clotting. Fibrin sealants are primarily used as an adjunct to hemostasis for mild to moderate surgical bleeding in adults when standard surgical techniques are ineffective or impractical. Objective The aim of these studies was to assess the biochemical and functional characteristics of Fibrin Sealant Grifols. Methods Total protein, plasma protein impurities, and molecular forms were analyzed for both components. Clottable fibrinogen and thrombin activity were also measured. Both components were analyzed by SDS-PAGE and Western blot. The fibrin sealant clot was functionally characterized. Results The components had minimal contamination by other plasma proteins and no aggregates or degradation products. The fibrinogen component was almost completely clottable fibrinogen. The thrombin component contains albumin. The fibrinogen component formed a fibrin polymer and clots with tensile strength similar to literature values. The application device applied the components in a 1:1 volume ratio. Clotting time was ∼ 5 s when applied with the drip tip and nearly instantaneous when applied with the airless spray apparatus. Conclusion This fibrin sealant was shown to contain highly purified fibrinogen and thrombin delivered in a 1:1 volume ratio. These properties contribute to the clinical safety and efficacy of this product for many applications.
本文介绍了纤维蛋白密封胶Grifols的活性和纯度。这种纤维蛋白密封剂含有两种成分(纤维蛋白原和凝血酶),模拟凝血的最后阶段。当标准手术技术无效或不切实际时,纤维蛋白密封剂主要用于成人轻度至中度手术出血的辅助止血。目的评价纤维蛋白密封胶Grifols的生化和功能特性。方法分析总蛋白、血浆蛋白杂质及两组分的分子形态。测定可溶纤维蛋白原和凝血酶活性。用SDS-PAGE和Western blot对两种成分进行分析。纤维蛋白密封凝块的功能特征。结果各组分受其他血浆蛋白污染最小,无聚集物和降解产物。纤维蛋白原成分几乎完全为可溶纤维蛋白原。凝血酶成分含有白蛋白。纤维蛋白原成分形成纤维蛋白聚合物和凝块,其抗拉强度与文献值相似。应用装置以1:1的体积比应用组件。使用滴头时,凝血时间为~ 5 s,使用无空气喷雾装置时,凝血时间几乎是瞬间的。结论该纤维蛋白密封胶含有高纯度的纤维蛋白原和凝血酶,其体积比为1:1。这些特性有助于本产品在许多应用中的临床安全性和有效性。
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引用次数: 0
Venous Thromboembolism Prophylaxis with Enoxaparin Versus Unfractionated Heparin in Patients with Low Body Weight 低体重患者应用依诺肝素与未分离肝素预防静脉血栓栓塞
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.1177/26348535231156848
David Michael Kaylor, Regan Merrell Wade, Kathryn Brinkley Chappell, Meredith Huff Niemann, Vanessa Marie VanArsdale
Background Although reduced doses of enoxaparin have been studied in low body weight patients, no study has directly compared enoxaparin to unfractionated heparin for venous thromboembolism (VTE) prophylaxis in this population. Objective To determine the most safe and efficacious parenteral venous thromboembolism prophylaxis regimen for low body weight patients. Design Retrospective cohort. Methods Hospitalized patients weighing < 55 kg receiving either enoxaparin or unfractionated heparin for VTE prophylaxis over a period of two and a half years were evaluated. Primary outcomes included rates of VTE and overall bleeding events. Secondary outcomes included rates of major and clinically relevant minor bleeding. Dosing regimens were analyzed to determine associations between dose, weight, creatinine clearance, VTE, and bleeding events. Primary and secondary outcomes were evaluated using Chi squared and Fischer's Exact tests. Results Three hundred and sixty-one patients contributed 400 episodes of VTE prophylaxis. Most patients were female (88%), had a Padua score ≥ 4 (82%) and were admitted to medical/surgical units (76%). VTE rates were 0.4% in patients receiving enoxaparin and 2.5% in patients receiving unfractionated heparin ( P = 0.334). Bleeding rates were 12% and 11% in the enoxaparin and unfractionated heparin groups, respectively ( P = 1). Patients receiving enoxaparin 30 mg daily had bleeding rates of 7.6% compared to 14.7% in patients receiving 40 mg daily ( P = 0.134). Patients receiving heparin twice daily had no bleeding events compared to 12.9% of patients receiving heparin three times daily ( P = 0.222). Conclusion Overall, no difference in VTE or bleeding rates were found between patients weighing < 55 kg receiving enoxaparin versus unfractionated heparin, and there was no difference in bleeding or VTE events in the patients receiving enoxaparin 30 mg daily. Due to this, empirically reducing the dose of enoxaparin for VTE prophylaxis appears reasonable and safe in patients weighing < 55 kg.
背景:虽然在低体重患者中研究了减少剂量的依诺肝素,但没有研究直接比较依诺肝素和未分离肝素在这一人群中预防静脉血栓栓塞(VTE)的效果。目的探讨低体重患者最安全有效的静脉外血栓栓塞预防方案。设计回顾性队列。方法住院患者称重<在两年半的时间里,对55公斤接受依诺肝素或未分割肝素预防静脉血栓栓塞的患者进行了评估。主要结局包括静脉血栓栓塞率和总出血事件。次要结局包括大出血和临床相关小出血的发生率。对给药方案进行分析,以确定剂量、体重、肌酐清除率、静脉血栓栓塞和出血事件之间的关系。使用卡方检验和费舍尔精确检验评估主要和次要结局。结果361例患者提供了400次静脉血栓栓塞预防。大多数患者为女性(88%),Padua评分≥4(82%),住院/外科(76%)。静脉血栓栓塞率在接受依诺肝素治疗的患者中为0.4%,在接受未分离肝素治疗的患者中为2.5% (P = 0.334)。依诺肝素组和未分割肝素组的出血率分别为12%和11% (P = 1)。每日服用依诺肝素30 mg的患者出血率为7.6%,而每日服用40 mg的患者出血率为14.7% (P = 0.134)。每日接受两次肝素治疗的患者无出血事件,而每日接受三次肝素治疗的患者为12.9% (P = 0.222)。结论总体而言,体重和体重的患者静脉血栓栓塞和出血发生率无显著差异;服用依诺肝素与未分离肝素的患者相比,服用每日30毫克依诺肝素的患者在出血或静脉血栓栓塞事件方面没有差异。因此,从经验上看,减少依诺肝素预防静脉血栓栓塞的剂量对体重为100公斤的患者似乎是合理和安全的。55公斤。
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引用次数: 0
A Pilot Study Evaluating the Ex Vivo Effects of Varying Factor VIII Concentration on Clot Kinetics and Architecture in Patients With Haemophilia A 一项评估不同因子VIII浓度对A型血友病患者凝块动力学和结构的体外影响的初步研究
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.1177/26348535231191440
Chanel Morris, Johan Potgieter, Janette Bester
Background Haemophilia A (HA) is a bleeding disorder, due to a deficiency in factor VIII (FVIII). These patients are unable to produce a stable fibrin clot in the propagation phase of coagulation as they do not generate sufficient thrombin. The primary treatment for HA in South Africa remains replacement therapy with standard half-life FVIII clotting factor concentrate, aimed at reducing bleeding episodes. Objectives To evaluate the effect of varying concentrations of FVIII on whole blood (WB) clot architecture and kinetics during clot formation in patients with severe HA. Design A cross-sectional study where blood from 20 patients with HA was exposed to FVIII ex vivo and compared to a control group of 20 healthy individuals. Methods Scanning electron microscopy (SEM) was used to study WB clot architecture and thromboelastography ® (TEG ® ) was used to quantify WB clot kinetics. Results Scanning electron microscopy studies revealed that patients with HA have sparse, disorganized fibrin networks with limited crosslinking and red blood cells (RBCs) stacked in rouleaux formation. Haemophilia A blood spiked to a 10 to 15 IU/dL FVIII concentration showed improvements in the organisation of the fibrin network with some altered RBCs. In addition, blood spiked to a 30 to 35 IU/dL FVIII concentration showed an increase in fibrin formation with normal RBCs. Thromboelastography ® showed that patients with HA had an increased clot initiation time and decreased clot strength. When spiked to a 10 to 15 IU/dL FVIII concentration the clot kinetic profile showed normalization. However, an increase in FVIII concentration higher than 30 IU/dL showed altered clot architecture and kinetics. Conclusion Based on the current study, FVIII levels at 10 to 15 IU/dL improved clot kinetics but did not normalize the architecture. It may be sufficient for prevention of haemorrhages. Factor VIII levels at 30 to 35 IU/dL resulted in rapid but weaker clot formation. However, at this concentration the clot architecture was normalised which is important for haemostasis. Here it was also evident that the findings of these two modalities (TEG ® and SEM) should not be separated but interpreted in conjunction with each other.
血友病A (HA)是一种出血性疾病,由于缺乏因子VIII (FVIII)。这些患者不能在凝血增殖阶段产生稳定的纤维蛋白凝块,因为他们不能产生足够的凝血酶。南非HA的主要治疗方法仍然是使用标准半衰期FVIII凝血因子浓缩物的替代疗法,旨在减少出血发作。目的评价不同浓度FVIII对严重HA患者血栓形成过程中全血(WB)凝块结构和动力学的影响。设计一项横断面研究,将20名HA患者的血液在体外暴露于FVIII,并与20名健康个体的对照组进行比较。方法采用扫描电子显微镜(SEM)研究WB血块结构,采用血栓弹性成像(TEG®)定量分析WB血块动力学。结果扫描电镜研究显示,HA患者的纤维蛋白网络稀疏,无组织,交联有限,红细胞呈rouleaux状堆积。血友病A血液中FVIII浓度达到10至15 IU/dL时,纤维蛋白网络的组织得到改善,红细胞发生改变。此外,血液中FVIII浓度达到30至35 IU/dL时,与正常红细胞相比,纤维蛋白形成增加。血栓弹性成像显示,HA患者凝块起始时间增加,凝块强度降低。当FVIII浓度达到10 ~ 15 IU/dL时,血凝块动力学曲线恢复正常。然而,当FVIII浓度高于30 IU/dL时,凝块结构和动力学发生改变。根据目前的研究,10至15 IU/dL的FVIII水平改善了凝块动力学,但并没有使结构正常化。它可能足以预防出血。因子VIII水平在30至35 IU/dL导致快速但较弱的凝块形成。然而,在这个浓度下,凝块结构正常化,这对止血很重要。这里也很明显,这两种模式(TEG®和SEM)的发现不应该分开,而应该相互结合来解释。
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引用次数: 0
A Multifaceted Educational Intervention to Reduce Plasma Utilization 减少血浆利用的多层面教育干预
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.1177/26348535231177667
Maha A. Badawi, Ali Algiraigri, Afnan J. Al-Sulami, Anas A. Abunar, Rami Alharbi, Yoon Soo Park, Ara Tekian
Blood transfusion services maintain quality practice through adherence to guidelines but are faced with blood component wastage due to multiple factors. One factor, in particular, is poor ordering and utilization practices followed by physicians who may not be familiar with the indications and the optimal conditions for the handling of blood products. This study evaluates whether an educational intervention would help reduce ordering of plasma and improve wastage rates after thawing. The study followed a quasi-experimental pre-test post-test design. A multifaceted educational intervention was introduced at a tertiary hospital-based combining predisposing, enabling, and reinforcing activities, based on the literature of effective continuous professional development. The activities included a brief survey to evaluate participant knowledge about indications of plasma and its storage condition, a lecture, and periodic reports and reminders sent to all department heads in the hospital. Respondents to the survey and lecture attendees included physicians in adult critical care and anesthesia departments. Monthly plasma utilization and wastage rates were compared before and after introducing the intervention. Sixty physicians participated in the survey. The questions were answered correctly by less than half. After introducing the intervention, monthly plasma transfusion rates decreased; however, wastage rates did not improve. There is a knowledge gap among physicians who order plasma. The educational intervention resulted in lower plasma transfusion rates but did not affect wastage rates. Future interventions would benefit from including physicians in other departments as well as nurses.
输血服务机构通过遵守指南来保持高质量的做法,但由于多种因素而面临血液成分的浪费。其中一个特别的因素是,医生可能不熟悉血液制品的适应症和处理血液制品的最佳条件,他们所遵循的订购和使用做法不佳。本研究评估教育干预是否有助于减少血浆订购和改善解冻后的流失率。本研究采用准实验前测后测设计。基于有效的持续专业发展的文献,在一家三级医院引入了多方面的教育干预,结合了诱发、促进和强化活动。这些活动包括一个简短的调查,以评估参与者对血浆指征及其储存条件的了解,一个讲座,以及定期向医院所有部门主管发送报告和提醒。调查对象和讲座参与者包括成人重症监护和麻醉部门的医生。干预前后每月血浆利用率和浪费率的比较。60名医生参与了调查。这些问题答对的不到一半。引入干预措施后,每月血浆输注率下降;然而,流失率并没有改善。订购血浆的医生之间存在知识差距。教育干预导致血浆输注率降低,但对浪费率没有影响。未来的干预将受益于包括其他部门的医生和护士。
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引用次数: 0
Frequency and Clinical Significance of Antinuclear Antibody Positivity in Immune Thrombocytopenia in Adults 成人免疫性血小板减少症患者抗核抗体阳性的频率及临床意义
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.1177/26348535231165594
Rabeea Munawar Ali, Sidra Zafar, Madiha Abid, Aqsa Javed Butt, Muhammad Shujat Ali, Munira Borhany
Background Immune thrombocytopenia is an immune-mediated disorder with heterogenous presentation. It shows association with other autoimmune disorders with varying frequency. Objective The objective of this study was to establish the frequency of antinuclear antibody (ANA) in our population and to evaluate its association with severity of clinical presentation, disease remission and association with other autoimmune diseases. Design Cross-sectional study. Methods This was a descriptive cross-sectional study that included 160 patients with immune thrombocytopenia purpura (ITP) presented at National Institute of Blood Disease and Bone Marrow Transplantation from February 2021 to July 2022. Patients were screened for ANA and other causes of secondary ITP. ANA-positive patients were compared with ANA-negative patients in terms of bleeding manifestations and platelet counts at initial presentation, association with other autoimmune diseases and response to first-line treatment. Results In our study 43 (26.9%) patients were ANA positive and 117 (73.1%) were negative for ANA. The initial platelet counts, and bleeding symptoms were not found to be significantly different among the 2 groups. And 79.1% of the ANA-positive patients had other autoimmune markers as well compared to 20.9% ANA-negative patients, showing a significant association ( P<.001). Response to treatment with steroid ± azathioprine assessed at 3 and 6 months were similar in both the groups. Conclusion Our results demonstrate that ANA positivity is not associated with severity of presenting symptoms, initial platelet counts, or response to treatment. However, the presence of ANA is associated with increased risk of other autoimmune diseases in patients with ITP.
背景:免疫性血小板减少症是一种具有异质性表现的免疫介导的疾病。它与其他不同频率的自身免疫性疾病有关。目的本研究的目的是建立抗核抗体(ANA)在我们人群中的频率,并评估其与临床表现的严重程度、疾病缓解和与其他自身免疫性疾病的关联。设计横断面研究。方法:这是一项描述性横断面研究,纳入了2021年2月至2022年7月在美国国家血液病和骨髓移植研究所提交的160例免疫性血小板减少性紫癜(ITP)患者。对患者进行ANA和其他继发性ITP原因的筛查。ana阳性患者与ana阴性患者在初次就诊时的出血表现、血小板计数、与其他自身免疫性疾病的相关性以及对一线治疗的反应方面进行比较。结果ANA阳性43例(26.9%),阴性117例(73.1%)。两组患者初始血小板计数、出血症状无明显差异。与20.9%的ana阴性患者相比,79.1%的ana阳性患者也有其他自身免疫标志物,显示出显著的相关性(P<.001)。两组在3个月和6个月时对类固醇±硫唑嘌呤治疗的反应相似。结论:我们的研究结果表明,ANA阳性与出现症状的严重程度、初始血小板计数或对治疗的反应无关。然而,ANA的存在与ITP患者发生其他自身免疫性疾病的风险增加有关。
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引用次数: 0
Retracted: “Factors Affecting Time to Engraftment During Autologous Stem Cell Transplantation in Patients With Multiple Myeloma” 撤稿:《多发性骨髓瘤患者自体干细胞移植中影响移植时间的因素》
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.1177/26348535231199808
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引用次数: 0
Lipid Profile, Nutritional Status and Severity Biomarkers in Adults With Sickle Cell Anemia 成人镰状细胞性贫血患者的血脂、营养状况和严重程度生物标志物
Q3 Medicine Pub Date : 2023-01-01 DOI: 10.1177/26348535231193889
Ingrid Cristiane Pereira Gomes, João Pedro Costa Machado Teles, Anny Catarina Sousa Coelho, Maria Carollyne Passos Cruz, Lindemberg Costa de Albuquerque, Mariana Amaral Carvalho, Paulo Herlan Castro dos Santos, Sérgio Nolasco dos Santos, Rosana Cipolotti
Lipid alterations have been described in sickle cell anemia (SCA), but their association with the disease severity is not fully understood and their correlation with the nutritional status of this population has not been widely studied. This study aimed to assess the lipid profile and its relation with the nutritional status, including anthropometric characteristics and daily macronutrient intake, and with the severity biomarkers in adults with SCA in a steady state. Cross-sectional study This was an analytical cross-sectional study with 55 adults with SCA and 60 members of the control group. Sociodemographic, anthropometric, nutritional, and laboratory data were collected between March 2019 and June 2020. Mann–Whitney test was used to compare groups and Spearman's correlation coefficient was used to test the relation between variables. The SCA group presented higher calorie, protein, and carbohydrate intake, lower levels of high-density lipoprotein-cholesterol (HDL-c), and a higher triglyceride (TG)/HDL-c ratio than the control. HDL-c showed a positive correlation with hemoglobin and hematocrit (HcT) and a negative correlation with indirect bilirubin and lactate dehydrogenase (LDH). The TG/HDL-c ratio was positively linked with reticulocyte count, LDH, and leukocyte count and negatively linked with Hb. The study findings showed hypocholesterolemia in SCA and its independent correlation with macronutrient intake. The links of HDL-c and TG/HDL-c ratio with hemolysis indices indicate that they are easily accessible, low-cost markers capable of predicting SCA severity.
镰状细胞性贫血(SCA)中脂质改变已被描述,但其与疾病严重程度的关系尚不完全清楚,且其与该人群营养状况的相关性尚未得到广泛研究。目的:本研究旨在评估成人SCA稳定状态下的血脂及其与营养状况的关系,包括人体测量特征和每日常量营养素摄入量,以及与严重程度生物标志物的关系。设计横断面研究方法:这是一项分析性横断面研究,有55名成年SCA患者和60名对照组成员。2019年3月至2020年6月期间收集了社会人口学、人体测量学、营养和实验室数据。组间比较采用Mann-Whitney检验,变量间关系采用Spearman相关系数检验。结果SCA组的卡路里、蛋白质和碳水化合物摄入量高于对照组,高密度脂蛋白-胆固醇(HDL-c)水平较低,甘油三酯(TG)/HDL-c比值较高。HDL-c与血红蛋白和红细胞压积(HcT)呈正相关,与间接胆红素和乳酸脱氢酶(LDH)负相关。TG/HDL-c比值与网织红细胞计数、LDH和白细胞计数呈正相关,与Hb呈负相关。结论:研究结果显示SCA患者的低胆固醇血症及其与宏量营养素摄入的独立相关性。HDL-c和TG/HDL-c比值与溶血指标的联系表明,它们是容易获得的、低成本的、能够预测SCA严重程度的标志物。
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引用次数: 0
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Plasmatology
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