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Lexiscan Sickle Cell Disease PubMed articles on BioPortfolio. Our PubMed references draw on over 21 million records from the medical literature. Here you can see the latest Lexiscan Sickle Cell Disease articles that have been published worldwide.
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A pseudoxanthoma elasticum (PXE)-like phenotype develops in a subset of patients with inherited hemoglobinopathies. Although PXE tissue changes are thought to develop in the absence of ABCC6 mutations in patients with beta-thalassemia, ABCC6 mutations have not been well evaluated among sickle cell disease patients with PXE-like disease. To our knowledge, we describe the first patient with sickle cell disease, PXE skin findings, and two confirmed pathogenic ABCC6 mutations. This case suggests that ABCC6 test...
Oxidative stress contributes to the complex pathophysiology of sickle cell disease. Oral therapy with pharmaceutical-grade l-glutamine (USAN, glutamine) has been shown to increase the proportion of the reduced form of nicotinamide adenine dinucleotides in sickle cell erythrocytes, which probably reduces oxidative stress and could result in fewer episodes of sickle cell-related pain.
Sickle cell disease is a genetic disease commonly affecting people of African, Indian, and Mediterranean descent. Patients with this chronic disease often require lifelong red blood cell transfusions. Formation of alloantibodies and autoantibodies are well-known complications that can arise with multiple transfusions. Another rare, but serious complication associated with transfusion is hyperhemolysis syndrome. The acquisition of new and/or rare alloantibodies can make it more difficult to find compatible b...
Salmonella infections are a common bacterial cause of invasive disease in people with sickle cell disease especially children, and are associated with high morbidity and mortality rates. Although available in some centres, people with sickle cell anaemia are not routinely immunized with salmonella vaccines. This is an update of a previously published Cochrane Review.
Pneumococcal carriage is the precursor for development of pneumococcal disease, and is also responsible for transmission of the organism from person-to-person. Individuals with Sickle Cell Disease (SCD) are more likely to develop invasive disease with S. pneumoniae compared to their healthy counterparts and the presentation of disease in the former is usually abrupt and severe. In Africa, little is known about the pneumococcus in relation to people with SCD Sickle Cell Disease (SCD). The aim of the study wa...
To compare pregnancy outcomes in women with sickle cell disease from recent deliveries with a similar group delivered earlier.
High blood cholesterol is associated with atherogenesis and endothelial dysfunction. The latter is present in hemolytic diseases, such as sickle cell anemia, whose carriers have hypocholesterolemia and low incidence of coronary artery disease.
Sickle cell disease (SCD) is the most common inheritable hematologic disorder in the USA and is associated with ischemic organ diseases. SCD-associated ischemic bowel disease is increasing being recognized, and studies on the hospitalization outcomes of such patients are limited.
To identify associations between severity of sickle cell retinopathy (SCR) and other clinical, laboratory, or treatment factors relevant to sickle cell disease (SCD).
Identifying sickle cell disease patients at high risk of complications could lead to personalized treatment and better prognosis but despite many advances prediction of the clinical course of these patients remains elusive. We propose a system-type approach to discover profiles of multiple, common biomarkers that correlate with morbidity and mortality in sickle cell disease. We used cluster analysis to discover 17 signatures of 17 common circulating biomarkers in 2320 participants of the Cooperative Study o...
Sickle cell disease (SCD) is a multisystem disease, and the predominant articular manifestation is osteonecrosis (ON). Total hip arthroplasty (THA) is technically challenging, and the complication rates are high. In this retrospective study, we have analysed the outcome of THA in a cohort of patients with SCD at our institution.
Sickle cell disease is a common, life-threatening genetic disorder that is best managed when diagnosed early by newborn screening. However, sickle cell disease is most prevalent in low-resource regions of the world where newborn screening is rare and diagnosis at the point-of-care is challenging. In many such regions, the majority of affected children die, undiagnosed, before the age of five years. A rapid and affordable point-of-care test for sickle cell disease is needed. The diagnostic accuracy of HemoTy...
The phenotypic expression of sickle cell disease (SCD) is a complex pathophysiologic condition. However, sickle erythrocytes might be the cause for multiple sources of pro-oxidant processes with consequent linked to chronic and systemic oxidative stress. Herein, we explored the SCD phenomena could be the result in formation of oxidative stress as well as inflammation in children.
To describe how nurses identify the pain in children with sickle cell disease (SCD) and to list the strategies used by them in the evaluation and control of pain.
Sickle cell disease (SCD) is the most frequent life-threatening genetic hemoglobinopathy in the world and occurs due to the synthesis of abnormal hemoglobin S (HbS). HbS-containing red blood cells (RBCs) are fragile, leading to hemolysis and anemia, and adhere to the endothelium, leading to hemorheological and hemodynamical disturbances. In its deoxygenated form, HbS may polymerize, leading to sickling of RBCs and potentially to vaso-occlusive crises. Recent findings observed that sickle cell disease patien...
Exchange transfusion is a mainstay in the treatment of sickle cell anemia. Transfusion recipients with sickle cell disease (SCD) can be transfused over 10 units per therapy, an intervention that replaces circulating sickle red blood cells (RBCs) with donor RBCs. Storage of RBCs makes the intervention logistically feasible. The average storage duration for units transfused at the Duke University Medical Center is approximately 2 weeks, a time window that should anticipate the accumulation of irreversible sto...
This article introduces a simple approach to adopt in the treatment of sickle cell disease (SCD) related ulcerations based on the acronym SICKLE, standing for Skin assessment, Infection control, Compression, Keep moving keep debriding, Local strapping and Endless support. This case report explains and describes each step used in the treatment of this challenging disease in a 24-year-old patient, achieving complete healing in 6 months in long-lasting SCD ulceration previously treated without results with man...
Fetal hemoglobin (HbF) is the major modifier for sickle cell disease (SCD) severity. HbF is modulated mainly by three major quantitative trait loci (QTL) on chromosomes 2, 6, and 11.
This study aimed to explore how parents communicate with children affected by sickle cell disease, a condition associated with social and cultural complexities that pose risks to open parent-child communication.
Worldwide, sickle cell disease is recognized as one of the major causes of morbidity and mortality. Caregivers and patients with such chronic illnesses experience economic, physical, social and psychological distresses which may lead to chronic sorrow. Chronic sorrow is viewed as a normal reaction to loss, however it can progress to a pathological state such as depression if the coping styles are ineffective. Therefore, the aim of this study was to explore the existence of chronic sorrow, triggers and copin...
The present systematic review aims to identify and analyze the available evidence on the safety and efficacy of surgical revascularization for pediatric patients with sickle cell disease (SCD) and moyamoya disease (MMD).
To develop patient-reported experience measure surveys for patients with sickle cell disease (SCD) to understand their healthcare and lived experience in the UK and for their use in future to inform healthcare service development.
Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this re...
Sickle cell disease is an inherited disorder of hemoglobin, resulting in abnormal red blood cells. These are rigid and may block blood vessels leading to acute painful crises and other complications. Recent research has focused on therapies to rehydrate the sickled cells by reducing the loss of water and ions from them. Little is known about the effectiveness and safety of such drugs. This is an updated version of a previously published review.
Hemolytic transfusion reactions are a rare, yet serious complication of red blood cell (RBC) transfusion. Patients with sickle cell disease (SCD) are at an increased risk for such reactions, because they are prone to make alloantibodies against transfused RBCs, complicating this integral part of their disease management. These reactions may be missed, and the patient's state may be attributed to vaso-occlusive crisis (VOC), with misguided therapy ensuing.