Ex vivo evaluation of the effect of plasma-derived factor VIII/von Willebrand factor in patients with severe hemophilia A on emicizumab prophylaxis.

Hemophilia A (HA) patients under emicizumab prophylaxis may require the concomitant use of procoagulant factors for breakthrough bleedings or immune tolerance induction (ITI). The aim of this study is to evaluate the ex vivo procoagulant effect of plasma-derived FVIII concentrates containing von Willebrand factor (pdFVIII/VWF) in samples from patients with severe HA without inhibitors on emicizumab prophylaxis. Samples from healthy controls (HC) and HA patients were drawn in sodium citrate plus corn trypsin inhibitor tubes and spiked with increasing concentrations of pdFVIII/VWF concentrates (10-400 IU/dL) (Fanhdi/Alphanate, Grifols), activated prothrombin complex concentrate (aPCC, 0.

Advances in Development of Drug Treatment for Hemophilia with Inhibitors.

Patients with hemophilia A and B who have inhibitors face limited treatment options, because replacement therapy with clotting factor VIII or IX concentrates is ineffective, particularly for patients with high-titer inhibitors. Current mainstay therapies include immune tolerance induction (through frequent injections of clotting factor VIII or IX concentrates) to eradicate inhibitors and bypassing agents (such as recombinant activated clotting factor VII and activated prothrombin complex concentrates) for the prevention and treatment of bleeding episodes. The use of these agents typically requires intravenous injections and sometimes hospitalization, which can be burdensome for patients.

Oral invasive procedures in Glanzmann thrombasthenia: a retrospective observational study.

Background: Glanzmann thrombasthenia (GT) is a very rare autosomal inherited bleeding disease affecting megakaryocyte lineage with impacts on oral health such as gingival bleeding, which requires specific management protocols. Very few clinical cases have been published in the dental and hematologic literature.

Objectives: This study focuses on a series of 21 patients affected specifically by GT and their hemorrhagic prophylaxis management with the use of recombinant activated factor VII (rFVIIa) for dental extractions and full-mouth debridement.

Integrin α1 upregulation by TF:FVIIa complex promotes cervical cancer migration through PAR2-dependent MEK1/2 activation.

Tissue factor (TF) and protease-activated receptor 2 (PAR2) have been associated with the progression of cancer, while integrins are essential for the adhesion and migration of cancer cells. This study aimed to explore the cross-talk between the TF:FVIIa complex, PAR2 signaling, and the expression of integrin α1 in cervical cancer cells. Utilizing data from The Cancer Genome Atlas (TCGA), the research examined the relationship between the TF and PAR2 genes and the integrin α1 gene (ITGA1) in reproductive cancers, revealing a positive correlation between integrin α1 expression and both TF and PAR2 genes.

Partial biochemical response of adrenal artery embolization for pheochromocytoma: A case report and review of the literature.

We present the case of a 22-year-old male with a left adrenal pheochromocytoma, initially diagnosed during a workup for thoracic pain. The patient's tumor was refractory to medical management, and surgical resection was ruled out due to high cardiovascular risk, stemming from cyanotic congenital heart disease, aortic aneurysm, and factor VII deficiency. The patient underwent adrenal artery embolization (AAE) as a salvage treatment.

Novel variants impairing Sp1 transcription factor binding in the COL7A1 promoter cause mild cases of recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is a rare and most often severe genodermatosis characterized by recurrent blistering and erosions of the skin and mucous membranes after minor trauma, leading to major local and systemic complications. RDEB is caused by loss-of-function mutations in COL7A1 encoding type VII collagen (C7), the main component of anchoring fibrils which form attachment structures stabilizing the cutaneous basement membrane zone. Most of the previously reported COL7A1 mutations are located in the coding or intronic regions.

Clinical characteristics and genomic changes of recurrent Methicillin-Resistant Staphylococcus aureus bacteremia.

Background: Recurrent or persistent methicillin-resistant Staphylococcus aureus (MRSA) bacteremia presents significant clinical challenges. Comprehensive genomic-scale studies on the genetic changes in MRSA that correspond to refractory bacteremia are lacking.

Method: From 2011 to 2019, MRSA blood isolates were collected from patients with persistent or recurrent bacteremia at a teaching hospital in southern Taiwan.

Correlation between Waist Circumference and IGF-1 Levels in an Elderly Population in Bali, Indonesia.

Background: Hyperinsulinemia due to insulin resistance is hypothesized to act as a promotor of cancer growth. In addition to the direct effects of hyperinsulinemia on cancer cells, the stimulation of tumor cell growth can also be indirectly mediated through growth factors and receptors such as insulin-like growth factor 1 (IGF-1). Increased cancer risk is also associated with increased adipose tissue, such as in abdominal obesity, due to the higher risk of insulin resistance and hyperinsulinemia.

Efficacy and safety of recombinant activated factor VII in Glanzmann thrombasthenia: A systematic literature review.

Background: Platelet transfusion is considered the standard treatment for preventing or controlling severe haemorrhage in Glanzmann thrombasthenia (GT). However, platelet transfusion can have detrimental effects, including the production of anti-GPIIb/IIIa isoantibodies or anti-HLA antibodies (Ab) and platelet transfusion refractoriness. Recombinant activated factor VII (rFVIIa) has been proposed as an alternative treatment to platelet transfusion.

Updated recommendations for warfarin reversal in the setting of four-factor prothrombin complex concentrate.

Introduction: Warfarin (vitamin K antagonist) remains an established anticoagulant for patients at high risk of arterial and venous thromboembolism. The prompt reversal of the anticoagulant effect of warfarin is necessary in the context of major bleeding or emergency surgery because of its extended inhibition of vitamin K-dependent coagulation factors for days. The mainstay of urgent warfarin reversal has been vitamin K administration, and infusion of a three-factor prothrombin complex concentrate (3FPCC) and the option for the addition of fresh frozen plasma as a source of factor VII.

Unlocking the Mycobacteroides abscessus pan-genome using computational tools: insights into evolutionary dynamics and lifestyle.

Mycobacteroides abscessus is a non-tuberculous mycobacteria implicated in causing lung infections. It is difficult to control owing to resistance to antibiotics and disinfectants. This work was aimed at comprehending: the pan-genome architecture, evolutionary dynamics, and functionalities of pan-genome components linked to COGs and KEGG.

Assessing attitudes toward research and plagiarism among medical students: a multi-site study.

Background: Research involves the systematic collection and analysis of data to enhance understanding of a particular phenomenon. Participation in medical research is crucial for advancing healthcare practices. However, there has been limited focus on understanding the factors that motivate medical students to engage in research.

Comparison of surgical management and outcomes of acute right colic and sigmoid diverticulitis: a French national retrospective cohort study.

Background: Acute right colic diverticulitis (ARD) is less frequent in Western countries than acute sigmoid diverticulitis (ASD). We aimed to compare the management of ARD and ASD operated on in emergency.

Methods: All consecutive patients who had emergency surgery for ASD and ARD (2010-2021) were included in a retrospective, multicenter, cohort study.

Review of Inherited Coagulation Disorders.

Most invasive dental procedures elicit some degree of bleeding which ultimately leads to clotting and eventual hemostasis. However, patients with inherited coagulation disorders may exhibit prolonged or, in some cases, excessive bleeding requiring multiple perioperative interventions. Von Willebrand disease is the most common inherited coagulopathy and often manifests via easy bruising, epistaxis, or prolonged bleeding.

Frequency and Association of Polymorphisms in F2, F7, and PROS1 Coagulation Genes with Disease Severity in Coronavirus Disease 2019.

Abnormal transcriptomic profiles of coagulation genes have been linked to coagulopathies in patients with coronavirus disease 2019 (COVID-19). The objective of the present study was to explore the frequency of genotypes and potential association of polymorphisms in genes encoding coagulation factors with the disease severity in COVID-19 patients.The patients were clinically categorized into four groups of COVID-19 disease severity (asymptomatic, mild, moderate, and severe).

Biophysical and socioeconomic characterization of Agewmariam experimental watershed in Northern Ethiopia: insights and management options.

Watershed characterization is essential for sustainable watershed management and effective resource utilization, particularly in assessing changes resulting from interventions. This study investigates the biophysical and socio-economic conditions of the Agewmariam watershed, aiming to develop land capability and suitability maps while identifying viable management options. Biophysical data, including land slope, soil properties, erosion severity, stoniness/rockiness cover, and vegetation cover, were collected through field surveys and analyzed using overlay analysis in ArcGIS.

Clinical, Laboratory, and Molecular Characteristics of Inherited Vitamin K-Dependent Coagulation Factors Deficiency.

Vitamin K-dependent coagulation factors deficiency (VKCFD) is a rare autosomal recessive genetic disease characterized by impaired levels of multiple coagulation factors (II, VII, IX, and X) and natural anticoagulants (proteins C and S). VKCFD is part of familial multiple coagulation factor deficiencies, reporting overall 50 affected families thus far. Disease manifestations are quite heterogeneous, bleeding symptoms may vary, and even, although generally mild, some patients may succumb to fatal outcomes.