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Extended half-life of factor IX (FIX) demonstrated clinical benefit and lower treatment burden than standard half-life FIX products in clinical trials. We analysed the impact in efficacy, pharmacokinetics (PKs) and costs of the switch from nonacog alfa (rFIX) to albutrepenonacog alfa (rFIX-FP) in the first patient with haemophilia B (HB) treated in Spain outside clinical trials. A 7-year-old boy presented with HB with poor venous access and repetition infections using rFIX, which was switched to rFIX-FP. Prophylaxis was adjusted by PKs using WAPPS-Hemo tailoring from 100 IU/kg/week of rFIX to 80 IU/kg/3 weeks of rFIX-FP. Comparing 6 months before, rFIX-FP reduced 68.5% FIX consumption/kg and 58.3% infusion frequency, but total costs/weight showed a slight increase. Ratio of half-life between rFIX and rFIX-FP was 3.4–3.7. This case report revealed that switch to rFIX-FP decreased frequency and FIX consumption, without adverse events and bleeds.

IntroductionHospital-acquired thrombosis accounts for a large proportion of all venous thromboembolism (VTE), with significant morbidity and mortality. This subset of VTE can be reduced through accurate risk assessment and tailored pharmacological thromboprophylaxis. This systematic review aimed to determine the comparative accuracy of risk assessment models (RAMs) for predicting VTE in patients admitted to hospital.MethodsA systematic search was performed across five electronic databases (including MEDLINE, EMBASE and the Cochrane Library) from inception to February 2021. All primary validation studies were eligible if they examined the accuracy of a multivariable RAM (or scoring system) for predicting the risk of developing VTE in hospitalised inpatients. Two or more reviewers independently undertook study selection, data extraction and risk of bias assessments using the PROBAST (Prediction model Risk Of Bias ASsessment Tool) tool. We used narrative synthesis to summarise the findings.ResultsAmong 6355 records, we included 51 studies, comprising 24 unique validated RAMs. The majority of studies included hospital inpatients who required medical care (21 studies), were undergoing surgery (15 studies) or receiving care for trauma (4 studies). The most widely evaluated RAMs were the Caprini RAM (22 studies), Padua prediction score (16 studies), IMPROVE models (8 studies), the Geneva risk score (4 studies) and the Kucher score (4 studies). C-statistics varied markedly between studies and between models, with no one RAM performing obviously better than other models. Across all models, C-statistics were often weak (<0.7), sometimes good (0.7–0.8) and a few were excellent (>0.8). Similarly, estimates for sensitivity and specificity were highly variable. Sensitivity estimates ranged from 12.0% to 100% and specificity estimates ranged from 7.2% to 100%.ConclusionAvailable data suggest that RAMs have generally weak predictive accuracy for VTE. There is insufficient evidence and too much heterogeneity to recommend the use of any particular RAM.PROSPERO registration numberSteve Goodacre, Abdullah Pandor, Katie Sworn, Daniel Horner, Mark Clowes. A systematic review of venous thromboembolism RAMs for hospital inpatients. PROSPERO 2020 CRD42020165778. Available from https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=165778https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=165778

ObjectiveTo assess the efficacy and safety of intradialytic exercise for haemodialysis patients.DesignSystematic review and meta-analysis.Data sourcesDatabases, including PubMed, Embase, the Cochrane Library, China Biology Medicine and China National Knowledge Infrastructure, were screened from inception to March 2017.Eligibility criteriaRandomised controlled trials (RCTs) aimed at comparing the efficacy and safety of intradialytic exercise versus no exercise in adult patients on haemodialysis for at least 3 months. A minimum exercise programme period of 8 weeks.Data extractionStudy characteristics and study quality domains were reviewed. Studies were selected, and data extracted by two reviewers.Data analysisThe pooled risk ratios and mean differences (MDs) with 95% CIs for dichotomous data and continuous data were calculated, respectively.ResultsA total of 27 RCTs involving 1215 subjects were analysed. Compared with no exercise, intradialytic exercise increased dialysis adequacy (Kt/V) (MD 0.07, 95% CI 0.01 to 0.12, p=0.02) and maximum volume of oxygen that the body can use during physical exertion peak oxygen consumption (MD 4.11, 95% CI 2.94 to 5.27, p<0.0001), alleviated depression standardised mean difference (−1.16, 95% CI −1.86 to –0.45, p=0.001) and improved physical component summary-short form-36 (SF-36) level (MD 7.72, 95% CI 1.93 to 13.51, p=0.009). Also, intradialytic exercise could significantly reduce systolic blood pressure (MD −4.87, 95% CI −9.20 to –0.55, p=0.03) as well as diastolic blood pressure (MD −4.11, 95% CI −6.50 to –1.72, p=0.0007). However, intradialytic exercise could not improve mental component summary-SF-36 level (MD 3.05, 95% CI −1.47 to 7.57, p=0.19). There was no difference in the incidence of adverse events between the intradialytic exercise and control groups.ConclusionsIntradialytic exercise resulted in benefits in terms of improving haemodialysis adequacy, exercise capacity, depression and quality of life for haemodialysis.

ObjectivesBlood transfusion is life-saving for patients experiencing acute blood loss and severe anaemia. In low-income and middle-income countries (LMICs), low blood donation rates and unavailability of whole blood and blood components (blood products) impairs timely blood transfusion. To fulfil patient-specific blood orders, a hospital blood transfusion service (HBTS) receives orders from a prescriber for blood transfusion, tests and prepares blood products for the patient. This study sought to describe the current state of LMIC HBTS.DesignA cross-sectional survey explored LMIC HBTS access to blood products, testing methods, policies and structure. Surveys were administered in English, Spanish, French and Russian, followed by a mixed-methods analysis.SettingHBTS within LMICs.ParticipantsFrom among 124 public and private facilities invited to participate, we received 71 (57%) responses. Of these responses, 50 HBTS from 27 LMICs performed on-site blood transfusions.ResultsMost LMIC HBTS perform blood collection to generate blood products for their patients (36/47, 77%); few relied exclusively on an external supply of blood products (11/47, 23%). The primary reason for blood transfusion was adult anaemia for non-malignant conditions (17/112, 15%). Testing methods varied by gross national income per capita. Blood transfusion delays to patients were common (17/30, 57%) attributed to inadequate blood inventories (13/29, 45%). Other barriers included lack of regular clinician education about transfusion (8/29, 28%) and sustainable financial models for the HBTS (4/29, 14%).ConclusionThis survey describes the status of HBTS in diverse LMICs, illustrating that the availability of blood products remains a principal problem, requiring HBTS to generate its own facility’s blood supply. Currently, blood shortages are not reported as a patient-specific adverse event making systematic tracking of delays in transfusion difficult. These findings highlight areas for further exploration related to the lack of available blood inventories for transfusions at HBTS in LMICs.

Objective To assess the effect of tranexamic acid on blood transfusion, thromboembolic events, and mortality in surgical patients.Design Systematic review and meta-analysis. Data sources Cochrane central register of controlled trials, Medline, and Embase, from inception to September 2011, the World Health Organization International Clinical Trials Registry Platform, and the reference lists of relevant articles.Study selection Randomised controlled trials comparing tranexamic acid with no tranexamic acid or placebo in surgical patients. Outcome measures of interest were the number of patients receiving a blood transfusion; the number of patients with a thromboembolic event (myocardial infarction, stroke, deep vein thrombosis, and pulmonary embolism); and the number of deaths. Trials were included irrespective of language or publication status.Results 129 trials, totalling 10 488 patients, carried out between 1972 and 2011 were included. Tranexamic acid reduced the probability of receiving a blood transfusion by a third (risk ratio 0.62, 95% confidence interval 0.58 to 0.65; P<0.001). This effect remained when the analysis was restricted to trials using adequate allocation concealment (0.68, 0.62 to 0.74; P<0.001). The effect of tranexamic acid on myocardial infarction (0.68, 0.43 to 1.09; P=0.11), stroke (1.14, 0.65 to 2.00; P=0.65), deep vein thrombosis (0.86, 0.53 to 1.39; P=0.54), and pulmonary embolism (0.61, 0.25 to 1.47; P=0.27) was uncertain. Fewer deaths occurred in the tranexamic acid group (0.61, 0.38 to 0.98; P=0.04), although when the analysis was restricted to trials using adequate concealment there was considerable uncertainty (0.67, 0.33 to 1.34; P=0.25). Cumulative meta-analysis showed that reliable evidence that tranexamic acid reduces the need for transfusion has been available for over 10 years.Conclusions Strong evidence that tranexamic acid reduces blood transfusion in surgery has been available for many years. Further trials on the effect of tranexamic acid on blood transfusion are unlikely to add useful new information. However, the effect of tranexamic acid on thromboembolic events and mortality remains uncertain. Surgical patients should be made aware of this evidence so that they can make an informed choice.

IntroductionCardiac surgery remains a high-risk procedure for bleeding despite advances in patient blood management. Conventional centrifugation-based autotransfusion devices primarily recover red blood cells, losing platelets and coagulation factors. The SAME autotransfusion device (i-SEP, Nantes, France) introduces an innovative filtration-based approach, recovering erythrocytes, leucocytes and platelets to enhance perioperative haemostasis. The main objective is to determine whether the filtration-based SAME device reduces significant perioperative bleeding compared with the centrifugation-based system in high-risk cardiac surgery patients.Methods and analysisThe Centrifugation-based vs filtration-based intraOperative cell saLvage on qualiTy of peRioperAtive haemostasis iN cardiac surgEry (COLTRANE) trial is a multicentre, parallel-group, single-blinded, superiority-randomised clinical trial. Conducted over 19 months in 10 French hospitals, the study will target patients at high risk of bleeding undergoing on-pump cardiac surgery via sternotomy. A total of 570 patients (285 per group) are required to achieve 80% statistical power for detecting clinically significant differences. Eligible patients will be randomised to either a centrifugation-based or filtration-based autotransfusion group. Both groups will follow standardised perioperative and cardiopulmonary bypass management, with the devices used only intraoperatively. The primary outcome is the proportion of patients with clinically significant perioperative bleeding defined as classes 2 to 4 of the Universal Definition of Perioperative Bleeding. The secondary outcomes include device efficiency and safety, perioperative haemostasis, lengths of intensive care unit and hospital stays, early postoperative morbidity and 30-day all-cause mortality. Ancillary studies will be performed to evaluate cell recovery and washing performance, the viscoelastic properties of retransfused blood (Quantra Qplus; Stago, Asnières-sur-Seine, France), and the effect of salvaged leucocytes on postoperative inflammation and immune function.Ethics and disseminationThis trial has received a favourable opinion from the Committee for the Protection of Persons and authorisation from the French authorities (Comité de protection des personnes Nord Ouest, IDRCB: 2023-A02566-39). Protocol V.1.1 was approved on 22 January 2024. The trial is registered on ClinicalTrials.gov (NCT06425614). The findings will be disseminated through oral communications at national and international scientific meetings and peer-reviewed journal publications. Individual participant data will be made available on reasonable request to qualified researchers, following review and approval by the study sponsor and ethics committee.Trial registration numberClinicalTrials.gov, NCT06425614.

ObjectivesSickle cell disease (SCD) leads to chronic and acute complications that require specialised care to manage symptoms and optimise clinical results. The National Heart Lung and Blood Institute (NHLBI) evidence-based guidelines assist providers in caring for individuals with SCD, but adoption of these guidelines by providers has not been optimal. The objective of this study was to identify barriers to treating individuals with SCD.MethodsThe SCD Implementation Consortium aimed to investigate the perception and level of comfort of providers regarding evidence-based care by surveying providers in the regions of six clinical centres across the USA, focusing on non-emergency care from the providers’ perspective.ResultsRespondents included 105 providers delivering clinical care for individuals with SCD. Areas of practice were most frequently paediatrics (24%) or haematology/SCD specialist (24%). The majority (77%) reported that they were comfortable managing acute pain episodes while 63% expressed comfort with managing chronic pain. Haematologists and SCD specialists showed higher comfort levels prescribing opioids (100% vs 67%, p=0.004) and managing care with hydroxyurea (90% vs 51%, p=0.005) compared with non-haematology providers. Approximately 33% of providers were unaware of the 2014 NHLBI guidelines. Nearly 63% of providers felt patients’ medical needs were addressed while only 22% felt their mental health needs were met.ConclusionsA substantial number of providers did not know about NHLBI’s SCD care guidelines. Barriers to providing care for patients with SCD were influenced by providers’ specialty, training and practice setting. Increasing provider knowledge could improve hydroxyurea utilisation, pain management and mental health support.

IntroductionPatient involvement in healthcare has become increasingly important, not only as an expression of respect for autonomy but also as a means of empowering individuals to safeguard their care. In blood transfusion, informed consent is a critical tool for shared decision-making. This protocol outlines a scoping review to map how transfusion consent processes and forms are used globally and to characterise their key features. We aim to identify technologies used to obtain consent, evaluate compliance with international recommendations, describe practices in special populations and examine patients’ and families’ understanding of the consent process.Methods and analysisFollowing the Joanna Briggs Institute methodology, we will search PubMed, Embase, LILACS, Web of Science and Scopus; grey literature will be explored via Google Scholar and the Open Access Theses and Dissertations platform. No language or date restrictions will be applied. Reference lists of included studies will also be screened. Two reviewers will independently screen studies and chart data; disagreements will be resolved by a third reviewer. Synthesis will be diagrammatic/tabular with structured narrative, reported according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses: extension for Scoping Reviews. Where data allow, findings will be stratified by clinical setting. A stakeholder consultation with two transfusion medicine experts and two patient representatives will be undertaken to validate and refine interpretations.Ethics and disseminationOnly publicly available sources will be used; research ethics approval is not required. Findings will be submitted to a peer-reviewed journal and presented at scientific meetings.Trial registration number10.17605/OSF.IO/NU69J.

ObjectivePlatelet and fresh frozen plasma (FFP) transfusions are routinely employed in the management of severe dengue. Previous research has indicated a potential link between ABO blood groups and susceptibility to dengue, with evidence suggesting that mosquito vector feeding preferences may be influenced by host blood type. These factors could potentially impact transfusion demands during outbreaks. This retrospective study aimed to investigate the relationship between ABO blood groups and transfusion requirements in patients with dengue.DesignRetrospective study.SettingThe study was conducted at a tertiary care hospital in Kerala.MethodsClinical and laboratory data were reviewed for 199 patients confirmed with dengue who received blood component transfusions and compared with two control groups: 200 randomly selected patients with dengue who did not require transfusions and 200 patients without dengue who required transfusions, over a period spanning January 2015 to March 2023.ResultsAmong transfused dengue cases, blood groups O (41.71%), A (28.14%) and B (23.12%) were most prevalent; however, no statistically significant association was observed between ABO blood group and transfusion requirement. Furthermore, the total volumes of FFP and platelet transfusions did not differ significantly across ABO groups among patients with dengue. Notably, platelet transfusions were significantly more frequent in dengue cases (92.0%) compared with transfused patients without dengue (35.5%), whereas FFP transfusions were more common in non-dengue transfused cases (84.5%) than in patients with dengue (44.7%). Patients with dengue also received significantly higher mean volumes of both FFP and platelets.ConclusionDespite earlier reports linking ABO blood types to dengue susceptibility, this study found no significant association with transfusion requirements, warranting confirmation through larger multicentre studies.

IntroductionThalassaemia is an inherited blood disorder, for which definitive treatments remain largely inaccessible. The recommended approach to reduce the disease burden is by prevention through screening. Currently, the implementation of thalassaemia preventive measures is poorly regulated in Indonesia. Thalassaemia prevention and education are best targeted to the youth, but information on their awareness towards thalassaemia is limited. This study aims to investigate the knowledge, attitude and practice (KAP) towards thalassaemia among Indonesian youth.MethodsThis observational study took place between January and May 2021. An online questionnaire was disseminated to Indonesian youth aged 15–24. Eligible respondents included carriers, unaffected individuals and individuals with unknown carrier status. The questionnaire comprised 28 questions to assess KAP. A cut-off of 75% was used to categorise participant’s KAP into poor or negative and good or positive. Descriptive statistics, χ2 test, logistic regression and Pearson correlation were performed for data analysis.ResultsA total of 906 responses were gathered, and 878 were analysed. Most respondents had poor knowledge (62.1%), positive attitude (83.3%) and poor practice (54.4%) towards thalassaemia. The results implied that respondents had limited understanding regarding the types of thalassaemia and the difference between asymptomatic carriers and individuals without the thalassaemia trait. Many (82.6%) believed they were not carrying thalassaemia trait despite the fact that most (95.7%) never got tested. Age, education, gender, residence and family income were key factors that correlated with or predicted the youth’s KAP towards thalassaemia. Older respondents and women were more likely to have good KAP.ConclusionThalassaemia screening targeted to the youth is urgently needed, and future interventions must consider sociodemographic factors that may affect how they perceive the disease. Social media appeals to the youth as an important source of information, but school, parents and health professionals should also be involved in delivering education about thalassaemia.