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Showing "Provide Access Crizanlizumab Sickle Cell Disease Patients" PubMed Articles 1–25 of 77,000+

Association of the Δ Mutant Genotype with Sickle Cell Disease in Egyptian Patients.

Sickle cell disease is considered the most common single base mutation in the world, with >250,000 new patients being discovered each year. It consists of a wide spectrum of clinical presentations and complications. The is the mutant genotype of C-C chemokine receptor 5 (CCR5). It is widely distributed due to several micro organisms that target macrophages in different populations. Theoretically, confers an advantage to sickle cell disease patients. The chronic inflammatory response is the main pathogenes...

The increased neopterin content in turkish pediatric patients with sickle cell anemia.

In the present study, the possible activation of cellular immunity in SCD patients was investigated. As immune activation parameters, neopterin concentrations and kynurenine/tryptophan ratio for tryptophan degradation in 35 pediatric patients with sickle cell disease (31 HbSS and 4 HbSß) were determined. Our results have shown that neopterin levels (both urinary and serum) are increased in pediatric patients with sickle cell disease. The increase in neopterin concentration was accompanied by significantly ...

Evaluation of Ocular Complications by Using Optical Coherence Tomography in Children With Sickle Cell Disease Eye Findings in Children With Sickle Cell Disease.

The aim of this study was to compare optical coherence tomography (OCT) findings in pediatric patients with sickle cell disease (SCD) and healthy individuals and to investigate associations between these data and the patients' systemic findings.

Prevalence of enuresis and its impact in quality of life of patients with sickle cell disease.

Evidence indicates an increase in the prevalence of enuresis in individuals with sickle cell disease. The present study aims to evaluate the prevalence and impact of enuresis on quality of life in individuals with sickle cell disease.

Multicenter Evaluation of HemoTypeSC as a Point-of-Care Sickle Cell Disease Rapid Diagnostic Test for Newborns and Adults Across India.

Sickle cell anemia is the commonest genetic disorder in India, and the frequency of the sickle cell gene is very high in the remote tribal areas where facilities are generally limited. Therefore, a rapid and affordable point-of-care test for sickle cell disease is needed.

How I treat sickle cell disease with hematopoietic cell transplantation.

Sickle cell disease (SCD) leads to significant morbidity and early mortality, and hematopoietic cell transplantation (HCT) is the only widely available cure, with impacts seen on SCD-related organ dysfunction. Outcomes are excellent following matched related donor (MRD) HCT, leading to significantly expanded application of this treatment over the past decade. The majority of SCD patients lack a MRD, but outcomes following alternative donor HCT continue to improve on clinical trials. Within this framework, w...

Correlates of Cognitive Function in Sickle Cell Disease: A Meta-Analysis.

To provide a comprehensive quantitative review of biological, environmental, and behavioral correlates across domains of cognitive function in sickle cell disease (SCD).

American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support.

Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes.

Reflections of Healthcare Experiences of African Americans With Sickle Cell Disease or Cancer: A Qualitative Study.

The experiences of African American adult patients before, during, and after acute care utilization are not well characterized for individuals with sickle cell disease (SCD) or cancer.

Characteristics and outcomes of osteomyelitis in children with sickle cell disease: A 10-year single-center experience.

Patients with sickle cell disease (SCD) are at increased risk for osteomyelitis (OM). Diagnosis of OM in SCD is challenging as the clinical presentation is similar to a vasoocclusive crisis (VOC) with no diagnostic gold standard. We report characteristics and outcomes of OM in SCD patients treated at our center over 10-year period.

Web-Based Technology to Improve Disease Knowledge Among Adolescents With Sickle Cell Disease: Pilot Study.

Advancements in treatment have contributed to increased survivorship among children with sickle cell disease (SCD). Increased transition readiness, encompassing disease knowledge and self-management skills before transfer to adult care, is necessary to ensure optimal health outcomes. The Sickle Cell Transition E-Learning Program (STEP) is a public, Web-based, 6-module tool designed to increase transition readiness for youth with SCD.

Preference-based measure of health-related quality of life and its determinants in sickle cell disease in Nigeria.

Health-related quality of life (HRQL) and economic burden are important issues for people with sickle cell disease (SCD) owing to better survival due to medical advances. Preference-based or utility information is necessary to make informed economic decisions on treatment and alternative therapies. This study aimed to assess preference-based measures of HRQL in sickle cell patients.

Prevalence of serologic markers of transfusion and sexually transmitted infections and their correlation with clinical features in a large cohort of Brazilian patients with sickle cell disease.

Patients with sickle cell disease (SCD) often require red blood cell (RBC) transfusion for clinical complications, so may be exposed to transfusion-transmitted infections (TTIs). The prevalence of markers for human immunodeficiency virus (HIV), hepatitis C virus (HCV) and B (HBV), human T-cell lymphotropic virus (HTLV-1/2), Chagas disease, and syphilis in an SCD cohort in Brazil were studied.

Alteration of humoral, cellular and cytokine immune response to inactivated influenza vaccine in patients with Sickle Cell Disease.

Patients suffering from Sickle Cell Disease (SCD) are at increased risk for complications due to influenza virus. Annual influenza vaccination is strongly recommended but few clinical studies have assessed its immunogenicity in individuals with SCD. The aim of this study was to explore the biological efficacy of annual influenza vaccination in SCD patients by characterizing both their humoral and cell-mediated immunity against influenza antigen. We also aimed to investigate these immunological responses amo...

Mechanisms of alloimmunization in sickle cell disease.

Red blood cell (RBC) transfusion is an important treatment for some complications of sickle cell disease (SCD). On the contrary, transfusion may lead to alloimmunization to RBC antigens, with such alloantibodies putting patients at risk for acute or delayed hemolysis, and increasing the difficulty of finding compatible RBCs. Patients with SCD are more susceptible to developing RBC alloantibodies than other multiply transfused patient populations, for reasons that are not completely understood. In this revie...

Implementation of an Emergency Department Screening and Care Management Referral Process for Patients With Sickle Cell Disease.

The purpose of the project was to describe the implementation and evaluation of a care management referral program from emergency departments (EDs) to care management services for patients with sickle cell disease (SCD).

Diversity of RH and transfusion support in Brazilian sickle cell disease patients with unexplained Rh antibodies.

Genetic diversity in the RH genes among sickle cell disease (SCD) patients is well described but not yet extensively explored in populations of racially diverse origin. Transfusion support is complicated in patients who develop unexpected Rh antibodies. Our goal was to describe RH variation in a large cohort of Brazilian SCD patients exhibiting unexpected Rh antibodies (antibodies against RH antigens to which the patient is phenotypically positive) and to evaluate the impact of using the patient's RH genoty...

Prevalence of Bacteremia in Febrile Patients With Sickle Cell Disease: Meta-Analysis of Observational Studies.

Pneumococcal vaccination has decreased the bacteremia rate in both the general pediatric and sickle cell disease (SCD) populations. Despite this decrease, and an increasing concern for antibiotic resistance, it remains standard practice to obtain blood cultures and administer antibiotics in all febrile (>38.5°C) patients with SCD. We conducted a systematic review and meta-analysis of the available studies of the prevalence of bacteremia in febrile patients with SCD.

The interaction of valine and glucose and the influence on the spectrin-actin complex in sickle cell disease.

Sickle cell disease (SCD), the most common severe monogenic disease in the world, is known for the hallmark vaso-occlusive crises that cause great suffering and degradation of health for these patients. In 1949, the discovery of the abnormal sickle cell hemoglobin protein (HbS) β-globin chain revealed a mutation where glutamic acid is replaced with a valine (β6Glu→Val). From this discovery came the pathophysiological mechanism based on the abnormal polymerization of deoxy-HbS. While an important discove...

American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease.

Prevention and management of end-organ disease represent major challenges facing providers of children and adults with sickle cell disease (SCD). Uncertainty and variability in the screening, diagnosis, and management of cardiopulmonary and renal complications in SCD lead to varying outcomes for affected individuals.

Chronic pain persists in adults with sickle cell disease despite regular red cell transfusions.

Pain affects over 50% of adults with sickle cell disease (SCD), and this pain is largely managed outside of the hospital. While chronic transfusion therapy is used to decrease the rate of acute pain events in patients with SCD, less is known about its impact on the day-to-day experience of pain. To address this knowledge gap, we provided pain diaries to patients with SCD receiving chronic transfusion.

Exploring the Needs of Adolescents With Sickle Cell Disease to Inform a Digital Self-Management and Transitional Care Program: Qualitative Study.

Accessible self-management interventions are critical for adolescents with sickle cell disease to better cope with their disease, improve health outcomes and health-related quality of life, and promote successful transition to adult health care services. However, very few comprehensive self-management and transitional care programs have been developed and tested in this population. Internet and mobile phone technologies can improve accessibility and acceptability of interventions to promote disease self-man...

Acute Care Utilization at End of Life in Sickle Cell Disease: Highlighting the Need for a Palliative Approach.

People with sickle cell disease (SCD) have a life expectancy of

Male sickle cell patients, compensated transpubertal hypogonadism and normal final growth.

Investigate the gonadal hormonal function in sickle cell individuals.

Systematic review of L-glutamine for prevention of vaso-occlusive pain crisis in patients with sickle cell disease.

L-glutamine was approved by the United States Food and Drug Administration (FDA) for sickle cell disease (SCD) in 2017. Vaso-occlusive crisis (VOC) occurs in persons with SCD and is associated with acute pain episodes. This systematic review summarizes the evidence for L-glutamine in the prevention of VOC and associated pain in patients with SCD.

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