Concanavalin A as a promising lectin-based anti-cancer agent: the molecular mechanisms and therapeutic potential.

Concanavalin A (ConA), the most studied plant lectin, has been known as a potent anti-neoplastic agent for a long time. Since initial reports on its capacity to kill cancer cells, much attention has been devoted to unveiling the lectin's exact molecular mechanism. It has been revealed that ConA can bind to several receptors on cancerous and normal cells and modulate the related signaling cascades. The most studied host receptor for ConA is MT1-MMP, responsible for most of the lectin's modulations, ranging from activating immune cells to killing tumor cells. In this study, in addition to studying the effect of ConA on signaling and immune cell function, we will focus on the most up-to-date advancements that unraveled the molecular mechanisms by which ConA can induce autophagy and apoptosis in various cancer cell types, where it has been found that P73 and JAK/STAT3 are the leading players. Moreover, we further discuss the main signaling molecules causing liver injury as the most significant side effect of the lectin injection. Altogether, these findings may shed light on the complex signaling pathways controlling the diverse responses created via ConA treatment, thereby modulating these complex networks to create more potent lectin-based cancer therapy. Video Abstract.

Effectiveness of Autologous Fat Transfer in the Treatment of Scar-Related Conditions: A Systematic Review and Meta-analysis.

BACKGROUND AND AIM: Autologous fat transfer (AFT) has been introduced as a potential treatment option for scar-tissue and its related symptoms. However, the scientific evidence for its effectiveness remains unclear. This meta-analysis aims to evaluate the available evidence regarding the effectiveness of autologous fat transfer for the treatment of scar-tissue and its related conditions. METHODS: PubMed/Medline database was queried from its inception till the end of November 2021. All the relevant studies assessing the effect of autologous fat transfer in the treatment of scar-related conditions were pooled in using a random-effects model. RESULTS: 9 studies (n=179) were included in the meta-analysis. Pooled analysis demonstrates significant improvement in all subscales of the POSAS patient score with most prominent in color 2.4 points (95% CI 1.78-3.041), stiffness 2.9 points (95% CI 2.33-3.45), irregularity 2.2 points (95% CI 1.093-3.297) and thickness 1.8 points (95% CI 0.804-2.719), respectively. Pain and itch improved relatively lesser, 1.3 points (95% CI 0.958-1.674) and 0.6 points (95% CI 0.169-1.215), respectively. The POSAS observer scale showed a relatively lower improvement with the least in vascularity 0.5 points (95% CI 0.098-0.96), pigmentation 0.8 points (95% CI 0.391-1.276) and surface area 0.8 points (95% CI 0.34-1.25). Thickness improved by 1.4 points (95% CI 0.582-2.3), relief 1.0 points (95% CI 0.461-1.545) and pliability 1.5 points (95% CI 1.039-2.036). CONCLUSION: Our findings demonstrate that autologous fat transfer (AFT) is a promising treatment for scar-related conditions as it provides beneficial results in the scar quality. Future research should focus on the long-term effects of AFT and high-level evidence studies such as, randomized controlled trials (RCTs) and cohort studies are required. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Ultrasound-Guided Out-of-Plane Versus In-Plane Radial Artery Cannulation in Adult Cardiac Surgical Patients.

OBJECTIVES: The primary objective was to compare the rate of first-pass radial arterial cannulation using out-of-plane ultrasound guidance with in-plane imaging. The secondary endpoints were a comparison of the number of times the cannula was redirected, the number of attempts, the number of skin punctures, the incidence of hematoma, the time to completion of the cannulation procedure, and the number of failed attempts between the 2 ultrasound imaging techniques. DESIGN: A prospective, randomized, observational study. SETTING: A tertiary cardiac care center. PARTICIPANTS: Adult patients undergoing elective cardiac surgery. INTERVENTIONS: Radial artery cannulation with ultrasound guidance. MEASUREMENTS AND MAIN RESULTS: Eighty-four adult patients scheduled for elective cardiac surgery were randomly assigned to the out-of-plane ultrasound group (group I, n = 42) or the in-plane ultrasound group (group II, n = 42) for left radial artery cannulation. A linear ultrasound probe was used to identify the radial artery. In each approach, the number of times first-pass success was achieved, the number of times the cannula was redirected, the number of skin punctures, the incidence of hematomas, and the number of failed attempts were recorded. The first-pass success rate was greater in the in-plane ultrasound group and was statistically significant (p = 0.007). In the out-of-plane ultrasound group, a larger number of patients needed redirection of the cannula (p = 0.002). The number of patients in whom the skin needed to be punctured more than once was greater in the out-of-plane ultrasound group compared with the in-plane ultrasound group (p = 0.002). The incidence of hematoma formation and time to completion of the technique were similar in both groups (p = 0.241 and p = 0.792, respectively). CONCLUSIONS: In-plane ultrasound guidance appeared to be superior for achieving a higher first-pass success rate more often with minimal redirections and skin punctures compared with out-of-plane ultrasound guidance.

Changing professional behaviours: mixed methods study utilising psychological theories to evaluate an educational programme for UK medical doctors.

BACKGROUND: The Theory of Planned Behaviour (TPB) has been proposed as a useful framework to investigate professional behaviour, however, was not yet applied to the evaluation of an educational intervention. This study will address this gap by utilising the TPB to evaluate the effectiveness of an education programme delivered by the professional regulator for UK doctors in enhancing three professional behaviours: raising concerns, engaging in reflective practice, and use of regulator confidentiality guidance. METHODS: This is a comprehensive mixed methods study combining qualitative (interviews) and quantitative (quasi-experiment) data. Intervention participants were asked to complete a survey measuring the variables in the TPB (attitudes, subjective norms, perceived behavioural control, and intention) for the three professional behaviours before, immediately post, and 3-months later following the education programme. Ninety-four doctors completed the survey pre/post intervention and 38 at all three times. One hundred and eleven doctors from the same hospital trust who did not take part in the intervention completed the survey at two time points and formed the control group. Forty-two interviews were conducted with intervention participants. RESULTS: The quantitative study revealed that the educational intervention significantly improved attitudes (raising concerns, using confidentiality guidance), subjective norms (raising concerns, reflective practice, using confidentiality guidance), perceived control (raising concerns, using confidentiality guidance), and intentions (using confidentiality guidance) (Group and Time interaction; Fs ≥ 3.996, ps ≤ .047, ηp2 ≥ .020). Non-UK graduate doctors' subjective norms towards raising concerns and confidentiality guidance increased significantly after the intervention (Fs ≤ 6.602, ps ≥ .011, ηp2 = .032 F = 6.602, p = .011, ηp2 = .032), but not UK graduates (p > .05). Interviews revealed that doctors had positive views about professional behaviours but also mentioned numerous barriers to actually engage in more complex, context dependent behaviours. CONCLUSIONS: This study demonstrates that an educational intervention was successful in improving the TPB variables of three professional behaviours. It also revealed that teaching professionalism does not happen in isolation and, therefore, personal and contextual factors are crucial to consider. To change complex professional behaviours, barriers at all levels i.e., personal, organisational and system, should be addressed.

Smart-Contract Aware Ethereum and Client-Fog-Cloud Healthcare System.

The Internet of Medical Things (IoMT) is increasingly being used for healthcare purposes. IoMT enables many sensors to collect patient data from various locations and send it to a distributed hospital for further study. IoMT provides patients with a variety of paid programmes to help them keep track of their health problems. However, the current system services are expensive, and offloaded data in the healthcare network are insecure. The research develops a new, cost-effective and stable IoMT framework based on a blockchain-enabled fog cloud. The study aims to reduce the cost of healthcare application services as they are processing in the system. The study devises an IoMT system based on different algorithm techniques, such as Blockchain-Enable Smart-Contract Cost-Efficient Scheduling Algorithm Framework (BECSAF) schemes. Smart-Contract Blockchain schemes ensure data consistency and validation with symmetric cryptography. However, due to the different workflow tasks scheduled on other nodes, the heterogeneous, earliest finish, time-based scheduling deals with execution under their deadlines. Simulation results show that the proposed algorithm schemes outperform all existing baseline approaches in terms of the implementation of applications.

Effectiveness and Quality of Life with Montelukast in Asthma - A double-blind randomized control trial.

OBJECTIVE: To determine the role of montelukast - a leukotriene receptor antagonist (LTRA) - in improving the quality of life (QOL) and asthma control of adult patients with mild to moderate persistent asthma. METHODS: Randomized, double-blind, placebo-controlled, non-crossover trial was conducted from March 2017 till November 2018 in three hospitals of Karachi and Hyderabad. Adults of age 15 years or more with mild to moderate persistent asthma. Treatment group was administered tablet montelukast 10mg once daily; the other group was given a similar looking placebo; as an adjuvant to the current medication. QOL was assessed with Asthma Quality of Life Questionnaire - Standard (AQLQ-S) before and after the treatment. Asthma control was monitored via Asthma Control Test (ACT). RESULTS: After 4 weeks, the mean ± SD of overall QOL on AQLQ-S improved from 3.74±0.88 to 5.06±0.89 for montelukast group and from 3.58±0.92 to 4.71±0.97 for placebo group (p=0.02). The improvement in sub-domains of symptoms, activity, and emotional functions was not significant; however, the sub-domain "environmental stimuli" significantly improved with 5.06±0.89 for montelukast group and 4.71±0.97 for placebo group (p=0.02). The mean ± SD of ACT, after four weeks, for montelukast group was 18.19±2.91 and for placebo group 17.28±3.36. Only on ACT, Montelukast did not show any statistically insignificant results. CONCLUSION: The role of montelukast in improving QOL of adult patients with mild to moderate persistent asthma is quite beneficial. It improves patient quality of life. It has the ease of once daily oral administration and also eradicates side effects associated with long-term adherence to steroids.

Impact of Body Mass Index on outcomes in hospitalized heart failure patients with reduced versus preserved ejection fraction: a 1,699,494-individual analysis from the United States National Inpatient Sample.

BACKGROUND: Obesity's effect on outcomes in heart failure (HF) patients with reduced versus maintained ejection fraction (HFrEF and HFpEF) remains debatable. We evaluated hospital outcomes and healthcare expenditures in these patients based on their Body Mass Index (BMI). METHODS: Using the USA National Inpatient Sample (NIS) database, patients >18 years admitted with a primary diagnosis of HFrEF or HFpEF between January 1, 2004, and August 31, 2015, were studied. Patients were stratified into the following BMI categories: underweight, normal weight, overweight, obese, and morbidly obese. Adjusted multivariable analyses using Poisson regression models were used to study the association between BMI and hospital outcomes and healthcare costs. RESULTS: Overall, 1,699,494 patients were included. After full adjustment, obesity (OR=1.84; 95% CI: 1.22-2.76) and morbid obesity (OR=1.81; 95% CI: 1.22-2.70) increased the odds of in-hospital mortality compared with normal weight. When stratified per ejection fraction, underweight patients had higher odds of in-hospital mortality in HFrEF (OR=1.46; 95% CI: 1.06-2.01). Obese and morbidly obese patients had higher odds of in-hospital mortality in both HFrEF and HFpEF. Furthermore, obese and morbidly obese patients had a longer mean adjusted length of stay and higher health care expenses. CONCLUSIONS: Being underweight is associated with increased risk of in-hospital mortality in HFrEF patients. Obesity and morbid obesity increase the risk of in-hospital mortality and higher healthcare costs in both HFrEF and HFpEF. These findings have clinical significance for HF patients, and further research is needed to investigate the ideal weight for HF patients.

Trends in mortality related to kidney failure and diabetes mellitus in the United States: a 1999-2020 analysis.

BACKGROUND: Kidney failure ranks as the tenth leading cause of mortality in the United States (US), frequently arising as a complication associated with diabetes mellitus (DM). METHODS: Trends in DM and kidney failure mortality were assessed using a cross-sectional analysis of death certificates from the Centers for Disease Control and Prevention Wide-Ranging Online Data for Epidemiologic Research (CDC WONDER) database. Crude and age-adjusted mortality rates (AAMR) per 100,000 people and annual percent change (APC) in age-adjusted mortality rate with 95% CI were obtained and measured across different demographic and geographic subgroups. RESULTS: Between 1999 and 2020, a total of 325,515 deaths occurred related to kidney failure and DM. The overall age-adjusted mortality rate showed no significant change between 1999 and 2012, after which it declined until 2015 - 64.8 (95% CI - 75.6 to - 44.8) and has been steadily increasing since. Men had consistently higher age-adjusted mortality rates than women throughout the study duration (overall age-adjusted mortality rate men: 8.1 vs. women: 5.9). Non-Hispanic (NH) Black or African American individuals had the highest overall age-adjusted mortality rate (13.9), followed by non-Hispanic American Indian or Alaskan Native (13.7), Hispanic or Latino (10.3), non-Hispanic Asian or Pacific Islander (6.1), and non-Hispanic White (6.0). Age-adjusted mortality rate also varied by region (overall age-adjusted mortality rate: West:7.5; Midwest: 7.1; South: 6.8; Northeast: 5.8), and non metropolitan areas had higher overall age-adjusted mortality rate (7.5) than small/medium (7.2) and large metropolitan areas (6.4). CONCLUSION: After an initial decline, mortality rose across all the demographic groups from 2015 to 2020, revealing notable disparities in gender, race, and region.

Cantharidin Impedes Activity of Glutathione S-Transferase in the Midgut of Helicoverpa armigera Hübner.

Previous investigations have implicated glutathione S-transferases (GSTs) as one of the major reasons for insecticide resistance. Therefore, effectiveness of new candidate compounds depends on their ability to inhibit GSTs to prevent metabolic detoxification by insects. Cantharidin, a terpenoid compound of insect origin, has been developed as a bio-pesticide in China, and proves highly toxic to a wide range of insects, especially lepidopteran. In the present study, we test cantharidin as a model compound for its toxicity, effects on the mRNA transcription of a model Helicoverpa armigera glutathione S-transferase gene (HaGST) and also for its putative inhibitory effect on the catalytic activity of GSTs, both in vivo and in vitro in Helicoverpa armigera, employing molecular and biochemical methods. Bioassay results showed that cantharidin was highly toxic to H. armigera. Real-time qPCR showed down-regulation of the HaGST at the mRNA transcript ranging from 2.5 to 12.5 folds while biochemical assays showed in vivo inhibition of GSTs in midgut and in vitro inhibition of rHaGST. Binding of cantharidin to HaGST was rationalized by homology and molecular docking simulations using a model GST (1PN9) as a template structure. Molecular docking simulations also confirmed accurate docking of the cantharidin molecule to the active site of HaGST impeding its catalytic activity.

Management of Heart Failure With Reduced Ejection Fraction.

Heart failure with reduced ejection fraction (HFrEF) is a complex and progressive clinical condition characterized by dyspnea and functional impairment. HFrEF has a high burden of mortality and readmission rate making it one of the most significant public health challenges. Basic treatment strategies include diuretics for symptom relief and use of quadruple therapy (Angiotensin receptor blocker/neprilysin inhibitors, evidence-based beta-blockers, mineralocorticoid receptor antagonists, and sodium-glucose co-transporter 2 inhibitors) for reduction in hospitalizations, all-cause mortality, and cardiovascular mortality. Despite compelling evidence of clinical benefit, guideline directed medical therapy is vastly underutilized in the real-world clinical practice. Other medications such as intravenous iron, ivabradine, hydralazine/nitrates and vericiguat may also have a role in certain subgroup of HFrEF patients. Specific groups of patients with HFrEF may also be candidates for various device therapies such as implanted cardioverter defibrillators, cardiac resynchronization therapy and trans catheter mitral valve repair. This review provides a comprehensive overview of drug and device management approaches for patients with HFrEF, recommendations for initiation and titrations of therapies, and challenges associated with guideline directed medical therapy in the management of patients with HFrEF (Graphical abstract).

Management of Heart Failure With Reduced Ejection Fraction in Patients With Diabetes Mellitus and Chronic Kidney Disease.

Heart failure (HF), diabetes, and chronic kidney disease (CKD) frequently coexist, with one comorbidity worsening the prognosis of another. ß-blockers, angiotensin-receptor-neprilysin inhibitors, renin-angiotensin-aldosterone system inhibitors, mineralocorticoid-receptor antagonists, and sodium-glucose cotransporter-2 inhibitors all have been shown to reduce mortality in patients with HF with reduced ejection fraction. However, their uptake in real-world clinical practice remains low, especially among patients who have multiple other comorbidities such as CKD and diabetes. The management of HF in patients with diabetes and CKD can be especially challenging because these patients typically are older, frail, and have multiple other comorbidities, and guideline-directed medical therapy used in HF potentially can affect renal function acutely and chronically. In this article, we discuss the available evidence for each of the foundational HF therapies in patients with diabetes and CKD, emphasizing the current challenges and outlining future directions to optimize the management of HF among these high-risk patients.

Restricted Boltzmann Machine Assisted Secure Serverless Edge System for Internet of Medical Things.

The Internet of things (IoT) is a network of technologies that support a wide variety of healthcare workflow applications to facilitate users' obtaining real-time healthcare services. Many patients and doctors' hospitals use different healthcare services to monitor their healthcare and save their records on the servers. Healthcare sensors are widely linked to the outside world for different disease classifications and questions. These applications are extraordinarily dynamic and use mobile devices to roam several locales. However, healthcare apps confront two significant challenges: data privacy and the cost of application execution services. This work presents the mobility-aware security dynamic service composition (MSDSC) algorithmic framework for workflow healthcare based on serverless, serverless, and restricted Boltzmann machine mechanisms. The study suggests the stochastic deep neural network trains probabilistic models at each phase of the process, including service composition, task sequencing, security, and scheduling. The experimental setup and findings revealed that the developed system-based methods outperform traditional methods by 25% in terms of safety and 35% in application cost.

Temporal Trends and Outcomes of Implantable Cardioverter Defibrillators in Heart Failure and Chronic Kidney Disease in the United States.

Chronic kidney disease (CKD) and hemodialysis increase the risk of sudden cardiac death (SCD) in heart failure (HF); however, national trends in utilization and outcomes of implantable cardioverter-defibrillator (ICD) in this population remain unknown. We sought to evaluate the utilization and outcomes of ICD therapy in HF patients with CKD and end-stage renal disease (ESRD) using the National Inpatient Sample from 2009 to 2018. Hospitalizations with a discharge diagnosis of systolic HF and ICD implantation were identified and stratified by stages of kidney disease. A total of 281,219 systolic HF hospitalizations who underwent ICD implantation were included. A significant decrease in inpatient ICD implantation was observed over the past decade (3.7% in 2009 to 1.1% in 2018) regardless of renal impairment. In-hospital mortality was highest in ESRD, followed by CKD compared with patients with no CKD. Length of hospital stay and hospitalization costs were also significantly higher in patients with CKD and ESRD. The overall utilization of inpatient ICD implantation has decreased in systolic HF patients and inpatient ICD placement in CKD is associated with an increased risk of mortality and adverse clinical outcomes. This indicates that patients with renal impairment and HF represent a sicker cohort than the general HF population.

The Safety and Efficacy of GLP-1 Receptor Agonists in Heart Failure Patients: A Systematic Review and Meta-Analysis.

Evaluation of glucagon-like peptide-1 receptor agonists (GLP-1 RAs) usage in heart failure (HF) patients with or without type 2 diabetes mellitus (T2DM) could be proven to be a critical breakthrough in treatment options available for these patients. Our study focuses on understanding the safety and efficacy of GLP-1 RAs in this patient population by pooling the data from 9 randomized controlled trials (RCTs) comprising 871 subjects. As compared with the placebo, GLP-1 RAs did not improve major adverse cardiovascular events (MACE) which include cardiovascular (CV) mortality and heart failure (HF) hospitalizations, our primary outcome. CV mortality (RR = 1.03, 95% CI = 0.56-1.88, P = 0.92) and HF hospitalizations (RR = 1.18, 95%CI = 0.93-1.51, P = 0.18). Similarly, GLP-1 RAs did not improve our secondary findings of left ventricular ejection fraction (LVEF) and 6-minute walk test (6MWT). LVEF (RR = 1.96, 95%CI = -0.16-4.07, P = 0.07) or 6 MWT (RR = 8.43, 95% CI = -2.69-19.56, P = 0.14). This meta-analysis shows that GLP-1 RAs do not improve cardiovascular outcomes in HF patients with or without T2DM.

Use of abdominal compression device in colonoscopy: a systematic review and meta-analysis.

BACKGROUND/AIMS: Colonoscopy for screening is associated with unpleasant experiences for patients, and abdominal compression devices have been developed to minimize these problems. However, there is a paucity of data supporting the therapeutic benefits of this strategy. This study examined the effects of using an abdominal compression device during colonoscopy on the cecal intubation time (CIT), abdominal compression, patient comfort, and postural changes. METHODS: We searched PubMed and Scopus (from inception to November 2021) for randomized controlled trials that assessed the effects of an abdominal compression device during colonoscopy on CIT, abdominal compression, patient comfort, and postural change. A random-effects meta-analysis was performed. Weighted mean differences (WMDs) and Mantel-Haenszel odds ratios (ORs) were calculated. RESULTS: Our pooled analysis of seven randomized controlled trials revealed that abdominal compression devices significantly reduced CIT (WMD, -0.76 [-1.49 to -0.03] minutes; p=0.04), abdominal compression (OR, 0.52; 95% confidence interval [CI], 0.28-0.94; p=0.03), and postural changes (OR, 0.46; 95% CI, 0.27-0.78; p=0.004) during colonoscopy. However, our results did not show a significant change in patient comfort (WMD, -0.48; 95% CI, -1.05 to 0.08; p=0.09) when using an abdominal compression device. CONCLUSION: Our findings demonstrate that employing an abdominal compression device may reduce CIT, abdominal compression, and postural change but have no impact on patient comfort.

Incidence and Comparison of Suicide in Various Phases of the Menstrual Cycle: a Systematic Review and Meta-analysis.

Background: Suicide is a global health concern. There are reproductive health-related factors that are responsible for increasing the risk of female suicide. There are a number of studies examining the association between suicide and the menstrual cycle, but still, there are no conclusive findings. Aim: We aimed to pool data from all the studies reporting data on suicides and the menstrual cycle phase to report the following outcomes: incidence of suicidal deaths in the menstrual, secretory, and proliferative phases, and to find out whether the burden of suicide in the menstrual phase in particular, was more at a young age (18-35 years) or middle age (36-50years). Methods: The PubMed database was extensively searched from inception till 12th April 2022. The data for the number of events occurring for each outcome were pooled using random-effects model and forest plots were created. Results: Five articles were shortlisted for inclusion in our analysis. Incidence of suicide in the secretory phase was highest at 45.2% [95% CI, 0.367-0.537]. The incidence of suicide, when occurring in the menstrual phase, was reported to be 68.4% (95 CI, 0.317-1.052) and 31.6% (95 CI, -0.052.3-0.68) for young-aged and middle-aged victims, respectively. Conclusion: Our results demonstrate that the menstrual phase has a lower risk of mortality due to suicide when compared to the other two phases of the menstrual cycle. Nevertheless, when suicide occurred in the menstrual phase, the incidence of suicide among the younger age-group was higher than for those in the middle age-group.

Mediterranean Diet and its Effect on Endothelial Function: A Meta-analysis and Systematic Review.

BACKGROUND: Endothelial dysfunction serves as an early marker for the risk of cardiovascular disease (CVD); therefore, it is a site of therapeutic interventions to reduce the risk of CVD. AIMS: To examine the effect of the Mediterranean diet (MedDiet), as an intervention, on structural and functional parameters of endothelial function, and how it may reduce the risk of CVD and associated mortality. METHODS: Medline database was searched for randomized controlled trials. Random-effects meta-analysis was conducted on 21 independent datasets. Meta-regression and subgroup analysis were performed to assess whether the effect of MedDiet was modified by health status (healthy subjects or with increased CVD risk), type of MedDiet intervention (alone or combined), type of parameter (functional or structural), study design (cross-over or parallel), BMI, age, and study duration. Our study used sample size, mean, and standard deviation of endothelial function measurements for both MedDiet intervention and control in the analyses. RESULTS: Inverse relationship between endothelial function and intake of MedDiet was observed (SMD: 0.34; 95% CI: 0.16, 0.52; P = 0.0001). Overall, MedDiet increased FMD by 1.39% (95% CI: 0.47, 2.19; P < 0.001). There was a significant improvement in endothelial function in both healthy patients and in those with an increased risk of CVD. No significant variation was observed in the effects of MedDiet on endothelial function, due to study design or type of intervention. CONCLUSIONS: These findings support that MedDiet can reduce the risk of CVD by improving endothelial function.

Bibliometric analysis of the literature on von Willebrand disease: Research status and trends.

BACKGROUND AND AIM: Von Willebrand disease (VWD) is considered the most prevalent inherited bleeding disorder. The current study aims to demonstrate the research status and trends on VWD worldwide. METHODS: Bibliometric analysis was used to investigate the global research productivity and trends on VWD. The publications on VWD from 1956 to 2021 were extracted using the Web of Science database. In the VWD domain, a total of 3,643 records were analyzed for authorship and collaboration patterns, yearly productivity, highly cited documents, relevant source of publication, most prolific scholars, productive countries, and organizations. RESULTS: The most productive journal, author, organization, and country were 'Haemophilia' with 439 publications, 'Favaloro EJ' with 119 publications, the 'University of Milan' with 192 publications, and the United States of America (USA) with 1,048 publications, respectively. The document with the highest citations was 'Srivastava A, 2013, Haemophilia,' which received 1,154 citations in total. In 2016, the highest number of publications shared by two author patterns was 28. With 199 publications, the year 2021 remained on the top, while the citation-wise analysis identified 2006 as the top year with 5,379 citations. CONCLUSIONS: Research productivity and publication trends on VWD revealed that the USA emerged as the most significant contributing country. The 'University of Milan' was the most significant contributing organization, while 'Favaloro EJ' was the most significant author. 'Hemophilia' was found to be the most significant journal in the field of VWD. It is recommended that researchers from countries with significant contributions to the field should collaborate with researchers from Asian countries and other countries that lack behind in research in the domain of VWD.(www.actabiomedica.it).