Sickle Cell Disease (Anemia)

Title: Sickle Cell Disease (Anemia)Category: Diseases and ConditionsCreated: 12/31/1997 12:00:00 AMLast Editorial Review: 1/10/2020 12:00:00 AM
Source: MedicineNet Chronic Pain General - Category: Anesthesiology Source Type: news

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Abstract Sickle cell disease (SCD) is among the most common genetic diseases in the United States, affecting approximately 100,000 people. In the United States, SCD is characterized by a shortened life expectancy of only about 50 years in severe subtypes, significant quality-of-life impairments, and increased healthcare utilization and spending. SCD is characterized by chronic hemolytic anemia, vaso-occlusion, and progressive vascular injury affecting multiple organ systems. The pathophysiology is directly related to polymerization of deoxygenated hemoglobin, leading to a cascade of pathologic events including ery...
Source: The American Journal of Managed Care - Category: Health Management Authors: Tags: Am J Manag Care Source Type: research
Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD) are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%. With migration they are becoming more common worldwide, making their management and care an increasing concern for health care systems.BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory haemopoietic expansion. Globally, there are over 25,000 births each year with transfusion-dependent thalassemia (TDT). The current available tr...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
CONCLUSION: Thus, our work provides evidence that despite SCA being a chronic inflammatory disease, only genes polymorphisms are not enough to change the outcome of this disease. PMID: 31448710 [PubMed - as supplied by publisher]
Source: Current Molecular Medicine - Category: Molecular Biology Authors: Tags: Curr Mol Med Source Type: research
Abstract When typically round red blood cells intermittently elongate and become lodged in the body's microcirculation, this can result in painful vaso-occlusive crises, often referred to as 'sickle cell crises'. The sickling and unsickling process can result in acute pain, chronic anaemia, ischaemic injury and multiple organ damage. One of the main concerns raised by patients with sickle cell disease is the lack of knowledge and understanding of their condition among healthcare professionals in acute care settings. Therefore, this article aims to enhance nurses' understanding of sickle cell disease and the effect...
Source: Nursing Standard - Category: Nursing Authors: Tags: Nurs Stand Source Type: research
Abstract Sickle cell disease afflicts millions of people worldwide and approximately 100,000 Americans. Complications are myriad and arise as a result of complex pathological pathways 'downstream' to a point mutation in DNA, and include red blood cell membrane damage, inflammation, chronic hemolytic anemia with episodic vaso-occlusion, ischemia and pain, and ultimately risk of cumulative organ damage with reduced patient lifespan. The National Heart, Lung, and Blood Institute's 2014 evidence-based guideline for sickle cell disease -management states additional research is needed before investigational curative the...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
CONCLUSIONS: Patients exhibiting greater daily HbA decrement were more likely to have multiple emergency department visits or admissions for sickling crises. Modulating HbA decrement may merit study as an intermediate metric for interventions to improve outcomes in hemoglobin SS disease. PMID: 31161685 [PubMed - as supplied by publisher]
Source: Transfusion - Category: Hematology Authors: Tags: Transfusion Source Type: research
In this study, we used HUT as the means to provide an all-encompassing assessment of cardiac and/or peripheral autonomic function in normal controls, SCD subjects and non-SCD subjects with chronic anemia. We hypothesized that by identifying different categories of HUT response among these subjects, we would be able to isolate the autonomic phenotypes that might place SCD subjects at increased risk for microvascular occlusion and VOC. We then employed the causal modeling approach, which utilizes signal analysis and system identification techniques, to probe and disentangle the functional mechanisms involved in the cardiovas...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
Some insurers had cited the CDC’s previous opioid guidelines and refused to pay for prescriptions for patients with cancer or sickle cell anemia, or other chronic pain conditions.
Source: WebMD Health - Category: Consumer Health News Source Type: news
Authors: Niccoli Asabella A, Altini C, Nappi AG, Lavelli V, Ferrari C, Marzullo A, Loiodice A, Rubini G Abstract Sickle cell disease (SCD) is the best known haemoglobinopathy, caused by a mutation substituting valina for glutamic acid at position 6 of the beta-globin chain of adult hemoglobin A, resulting in hemoglobin S (HbS). The homozygous HbS disease (HbSS), an autosomal recessive disorder, is the most common form and the Mediterranean area, along with sub-Saharian African and India, have the highest prevalence (1%-15%). In particular, Sicily with a prevalence of 2%-5%, is among the most interested regions. How...
Source: Hellenic Journal of Nuclear Medicine - Category: Nuclear Medicine Tags: Hell J Nucl Med Source Type: research
Conclusion: Camp offers unique opportunities for care coordination. Camps for children with SCD provide a setting for dissemination of best practices for the disease. Community-based organizations should partner in recruiting young adults with SCD as counselors. Educational material now available could be modified for use in the camp setting. All stakeholders in the care of children with SCD should work in unison to ensure these children enjoy the benefits of summer camp. PMID: 30559621 [PubMed]
Source: Ochsner Journal - Category: General Medicine Tags: Ochsner J Source Type: research
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