Neurological Complications Associated with Hereditary Bleeding Disorders.

Purpose Of Review: Hereditary bleeding disorders may have a wide variety of clinical presentations ranging from mild mucosal and joint bleeding to severe central nervous system (CNS) bleeding, of which intracranial hemorrhage (ICH) is the most dreaded complication. In this review, we will discuss the pathophysiology of specific hereditary bleeding disorders, namely, hemophilia A, hemophilia B, and von Willebrand disease (vWD); their clinical manifestations with a particular emphasis on neurological complications; a brief overview of management strategies pertaining to neurological complications; and a review of literature guiding treatment strategies.

Recent Findings: ICH is the most significant cause of morbidity and mortality in patients with hemophilia.

A microfluidic platform for simultaneous quantification of oxygen-dependent viscosity and shear thinning in sickle cell blood.

The pathology of sickle cell disease begins with the polymerization of intracellular hemoglobin under low oxygen tension, which leads to increased blood effective viscosity and vaso-occlusion. However, it has remained unclear how single-cell changes propagate up to the scale of bulk blood effective viscosity. Here, we use a custom microfluidic system to investigate how the increase in the stiffness of individual cells leads to an increase in the shear stress required for the same fluid strain in a suspension of softer cells.

Serum magnesium level and hematoma expansion in patients with intracerebral hemorrhage.

Spontaneous intracerebral hemorrhage (ICH) is a devastating subtype of stroke that results in significant rates of mortality and morbidities. The initial hematoma volume, hematoma expansion (HE), blood pressure (BP), and coagulopathy are considered strong predictors of clinical outcomes and mortality. Low serum magnesium (Mg) levels have been shown to be associated with larger initial hematoma and greater HE.

Graph theory analysis reveals how sickle cell disease impacts neural networks of patients with more severe disease.

Sickle cell disease (SCD) is a hereditary blood disorder associated with many life-threatening comorbidities including cerebral stroke and chronic pain. The long-term effects of this disease may therefore affect the global brain network which is not clearly understood. We performed graph theory analysis of functional networks using non-invasive fMRI and high resolution EEG on thirty-one SCD patients and sixteen healthy controls.

Oral anticoagulation therapy and subsequent risk of venous thromboembolism in atrial fibrillation patients.

Objective: Oral anticoagulation (OAC) prescribed to AF patients for the prevention of cardioembolic complications likely has the added benefit of preventing venous thromboembolism (VTE). This study evaluated, among AF patients who are anticoagulated, whether type of OAC was associated with subsequent VTE risk.

Methods: Non-valvular AF patients prescribed OACs between 2010 and September 2015 were identified via the MarketScan administrative claims databases.

The Role of Serum Calcium Level in Intracerebral Hemorrhage Hematoma Expansion: Is There Any?

Spontaneous intracerebral hemorrhage (ICH) is a devastating form of stroke, with a high rate of mortality and morbidity. Even with the best current medical or surgical interventions, outcomes remain poor. The location and initial hematoma volume are strong predictors of mortality.

Comparative effectiveness of direct oral anticoagulants and warfarin in patients with cancer and atrial fibrillation.

Randomized clinical trials comparing direct oral anticoagulants (DOACs) to warfarin in cancer patients have not been performed. We evaluated the effectiveness and associated risk of DOACs vs warfarin, as well as comparisons of DOACs, in a large population of cancer patients with nonvalvular atrial fibrillation (AF). Using the MarketScan databases, we identified 16 096 AF patients (mean age, 74 years) initiating oral anticoagulant and being actively treated for cancer between 2010 and 2014.

Increased theta band EEG power in sickle cell disease patients.

Objective: Pain is a major issue in the care of patients with sickle cell disease (SCD). The mechanisms behind pain and the best way to treat it are not well understood. We studied how electroencephalography (EEG) is altered in SCD patients.

Characterization of functional brain activity and connectivity using EEG and fMRI in patients with sickle cell disease.

Sickle cell disease (SCD) is a red blood cell disorder that causes many complications including life-long pain. Treatment of pain remains challenging due to a poor understanding of the mechanisms and limitations to characterize and quantify pain. In the present study, we examined simultaneously recording functional MRI (fMRI) and electroencephalogram (EEG) to better understand neural connectivity as a consequence of chronic pain in SCD patients.

Direct oral anticoagulant use and the incidence of bleeding in the very elderly with atrial fibrillation.

Atrial fibrillation (AF) is a major risk factor for stroke in the elderly population. The use of anticoagulation in patients with AF greatly reduces the risk for stroke, but results in an increased risk of bleeding. Over the past several years, direct oral anticoagulants (DOACs, dabigatran, rivaroxaban, and apixaban) have been used in place of warfarin for stroke prevention in AF.

Antiplatelet and Anticoagulant Therapies for Prevention of Ischemic Stroke.

Ischemic stroke represents one of the leading causes of death and disability in both the United States and abroad, particularly for patients with prior ischemic stroke or transient ischemic attack (TIA). A quintessential aspect of secondary stroke prevention is the use of different pharmacological agents, mainly antiplatelets and anticoagulants. Antiplatelets and anticoagulants exhibit their effect by blocking the activation pathways of platelets and the coagulation cascade, respectively.

Effect of oral and vaginal hormonal contraceptives on inflammatory blood biomarkers.

The use of combined hormonal contraceptives has been reported to increase the level of C-reactive protein (CRP). We assessed the effect of hormonal contraceptive use on inflammatory cytokines including CRP, monocyte chemotactic protein-1, soluble tumor necrosis factor (sTNF), interleukin-6 (IL-6), and soluble CD40 ligand. We used 79 female subjects (19 to 30 years old) who were combined oral contraceptives users (n = 29), combined vaginal contraceptive users (n = 20), and nonusers (n = 30) with CRP values of ≤1 (n = 46) or ≥3 (n = 33).

Calciphylaxis in catastrophic antiphospholipid antibody syndrome.

Antiphospholipid antibody syndrome (APS) is a multisystem disorder characterized by vascular thrombosis and presence of circulating autoantibodies. The presence of APS can predispose to macrovascular as well as microvascular thrombotic events. Renal involvement is a common occurrence especially in the background of systemic lupus erythematosus.

Risk of bleeding during long-term anticoagulation with warfarin: a tertiary care center experience.

The risk of recurrent venous thromboembolism (VTE) must be weighed against the risk of bleeding in deciding to keep patients on extended anticoagulation with vitamin K antagonists (VKAs). Most of the studies of risk of bleeding on VKAs are randomized controlled trials of highly selected patients followed for less than 1 year. We sought to determine the rate of bleeding in 'real world' patients on long-term anticoagulation with VKAs for VTE.

Monitoring anticoagulation in patients with an unreliable prothrombin time/international normalized ratio: factor II versus chromogenic factor X testing.

The international normalized ratio (INR) can be unreliable in patients with lupus anticoagulants (LACs) or other conditions affecting baseline testing. Alternative methods to assess anticoagulation on warfarin through measures of vitamin K-dependent factor activity by clot based or chromogenic assays may be necessary. In this patient population, the ideal method is unknown.

Intracerebral hemorrhage: a review of coagulation function.

Intracerebral hemorrhage (ICH) is associated with a higher mortality rate among stroke subtypes. The amount of hematoma at baseline and subsequent expansion are considered strong independent markers for determining poor clinical outcome. Even though reduction in blood pressure to prevent and control the amount of bleeding in ICH has received considerable amount of attention, the impact of coagulopathy and platelet dysfunction, on the bleeding diathesis has not been extensively investigated.

Oral versus vaginal combined hormonal contraceptives' effect on coagulation and inflammatory biomarkers among young adult women.

In order to compare the effect of combined oral contraceptive (COC) and combined vaginal contraceptive (CVC) methods on the inflammation and procoagulation, we recruited female participants in 3 groups: control participants, COC users, and CVC users. We measured different blood biomarkers. The users of both COC and CVC had higher levels of C-reactive protein (P < .

Evaluation of the coagulation profile among oral and vaginal combined hormonal contraceptive users using sonoclot coagulation analyzer.

Combined hormonal contraceptives possess an inherent risk of thrombus-related events. The purpose of this study is to elucidate alterations in the coagulation profile among young women using combined oral contraceptive (COC) or combined vaginal contraceptive (CVC) compared to a normal, healthy, female control group using the Sonoclot coagulation analyzer. We enrolled 159 participants (64 control individuals, 51 COC users, and 44 CVC users).

Risk factors for heart attack, stroke, and venous thrombosis associated with hormonal contraceptive use.

The search for a safe and effective method of contraception has been ongoing for centuries. During the last century, a variety of hormonal contraceptives, including combined hormonal oral contraceptives (COCs), have been introduced into the market. COCs have evolved through modifications of different hormonal components to minimize the risk of thrombotic events including stroke, myocardial infarction, and venous thrombosis.

A prothrombotic phenotype in the Copenhagen rat strain.

Introduction: Experimental and epidemiologic studies have identified several potential genetic components for increased thrombotic risk. Studies of thrombosis often use rat models without considering the effect of strain differences on thrombotic propensity.

Materials And Methods: A comparison of in vivo thrombotic occlusion after small-vessel anastomosis was made between age/weight-matched male Copenhagen and Lewis rats.

Rituximab-induced remission of a gastric MALT lymphoma.

Gastric MALT lymphoma is usually associated with H. pylori infection, and responds to treatment with antibiotics and a proton pump inhibitor. We report a case of H.

Genetic mapping and characterization of the bleeding disorder in the fawn-hooded hypertensive rat.

Release of platelet dense granule contents occurs in response to vascular injury, playing an important role in platelet aggregation and primary hemostasis. Abnormalities of the platelet dense granules results in a bleeding disorder of variable severity termed "storage pool defect" (SPD). We have examined the fawn-hooded hypertensive (FHH) rat as a model of SPD in order to genetically map the locus (Bd) responsible for prolonged bleeding.