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Condition: Sickle Cell Anemia
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Total 297 results found since Jan 2013.

Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service-part ii
Trans Am Clin Climatol Assoc. 2022;132:236-248.ABSTRACTIn the United States, primary stroke prevention in children with sickle cell anemia (SCA) is the standard of care and includes annual transcranial Doppler ultrasound to detect elevated velocities. For children with velocities ≥200 cm/sec, initial monthly blood transfusion therapy for at least a year, followed by the option of hydroxyurea therapy, results in a significant decline in the stroke rate. In Africa, no prior strategy exists for primary stroke prevention because regular blood transfusion therapy is not feasible. We completed a randomized controlled trial for...
Source: Transactions of the American Clinical and Climatological Association - October 5, 2022 Category: General Medicine Authors: Michael R Debaun Source Type: research

Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service-part ii
Trans Am Clin Climatol Assoc. 2022;132:236-248.ABSTRACTIn the United States, primary stroke prevention in children with sickle cell anemia (SCA) is the standard of care and includes annual transcranial Doppler ultrasound to detect elevated velocities. For children with velocities ≥200 cm/sec, initial monthly blood transfusion therapy for at least a year, followed by the option of hydroxyurea therapy, results in a significant decline in the stroke rate. In Africa, no prior strategy exists for primary stroke prevention because regular blood transfusion therapy is not feasible. We completed a randomized controlled trial for...
Source: Transactions of the American Clinical and Climatological Association - October 5, 2022 Category: General Medicine Authors: Michael R Debaun Source Type: research

Stroke in sickle cell disease and the promise of recent disease modifying agents
Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting approximately 100,000 individuals in the United States. Cerebrovascular disease is among the most common and debilitating complications of SCA, with 53% experiencing silent cerebral infarct by age 30 and 3.8% experiencing overt stroke by age 40  years. This review highlights the burden of cerebrovascular disease in SCD, including both stroke and silent cerebral infarct (SCI). We then discuss the pathophysiology of stroke and cerebral fat embolism in the absence of a patent foramen ovale.
Source: Journal of the Neurological Sciences - September 8, 2022 Category: Neurology Authors: Ava Runge, Danielle Brazel, Zahra Pakbaz Tags: Review Article Source Type: research

Academic Performance of Children With Sickle Cell Disease in the United States: A Meta-Analysis
Conclusion: Students with sickle cell disease demonstrate notable academic difficulties and are at high risk for grade retainment. Development of academic interventions and increased access to school support services are needed for this vulnerable population.Systematic Review Registration:https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42020179062.
Source: Frontiers in Neurology - December 13, 2021 Category: Neurology Source Type: research

Hematopoietic Cell Transplant Outcomes among Medicaid and Privately Insured Patients with Sickle Cell Disease
Sickle cell disease (SCD) is the most common inherited hemoglobin disorder affecting approximately 100,000 people in the United States (U.S.), a majority of whom are Black/African American, with an incidence rate of 1 in every 365 births.1 SCD is a chronic health condition associated with acute painful vaso-occlusive crisis (VOC), acute chest syndrome (ACS), stroke, chronic organ damage and culminates in a life expectancy that is less than half that of the general American population.2-4
Source: Biology of Blood and Marrow Transplantation - April 21, 2021 Category: Hematology Authors: Tatenda G. Mupfudze, Christa Meyer MS, Jaime M. Preussler, Lih-Wen Mau, Yung-Tsi Bolon, Patricia Steinert, Staci D. Arnold, Wael Saber, Lakshmanan Krishnamurti Source Type: research

Hematopoietic Cell Transplantation Outcomes among Medicaid and Privately Insured Patients with Sickle Cell Disease
Sickle cell disease (SCD) is the most common inherited hemoglobin disorder, affecting approximately 100,000 people in the United States, a majority of whom are Black/African American, with an incidence rate of 1 in every 365 births [1]. SCD is a chronic health condition associated with acute painful vaso-occlusive crisis (VOC), acute chest syndrome (ACS), stroke, and chronic organ damage and culminates in a life expectancy that is less than one-half that of the general American population [2 –4].
Source: Biology of Blood and Marrow Transplantation - April 21, 2021 Category: Hematology Authors: Tatenda G. Mupfudze, Christa Meyer, Jaime M. Preussler, Lih-Wen Mau, Yung-Tsi Bolon, Patricia Steinert, Staci D. Arnold, Wael Saber, Lakshmanan Krishnamurti Source Type: research

Allogenic Hematopoietic Stem Cell Transplantation in Sickle Cell Disease
Sickle cell disease (SCD) is one of the most common monogenic disorders worldwide and affects approximately 100,000 people in the United States alone. SCD can cause numerous complications, including anemia, pain, stroke, and organ failure, which can lead to death. Although there are a few disease-modifying treatments available to patients with SCD, the only current curative option is a hematopoietic stem cell transplant (HSCT). In this review, we will discuss the different approaches to allogeneic HSCT in the treatment of SCD and the outcomes of these approaches.
Source: Transfusion and Apheresis Science - January 10, 2021 Category: Hematology Authors: Dana K. Furstenau, John F. Tisdale Source Type: research

Systematic Review/Meta-Analysis on Efficacy of Allogeneic Hematopoietic Cell Transplantation in Sickle Cell Disease: An International Effort on Behalf of the Pediatric Diseases Working Party of European Society for Blood and Marrow Transplantation and the Sickle Cell Transplantation International Consortium
Sickle cell disease (SCD) is the most common inherited hemoglobinopathy, affecting nearly 100,000 individuals in the United States and approximately 300,000 worldwide, with low-income countries bearing the highest burden [1,2]. It is associated with several complications, including debilitating morbidity, organ injury, and shortened life expectancy. Progress in the overall management of SCD has been made in recent years, namely improved health maintenance surveillance programs, penicillin prophylaxis, chronic blood transfusions, vaccines, primary and secondary stroke prophylaxis, use of hydroxyurea, and approval of novel a...
Source: Biology of Blood and Marrow Transplantation - December 10, 2020 Category: Hematology Authors: Madiha Iqbal, Tea Reljic, Selim Corbacioglu, Josu de la Fuente, Eliane Gluckman, Ambuj Kumar, Farah Yassine, Ernesto Ayala, Areej El-Jawahri, Hemant Murthy, Fahad Almohareb, Shahrukh K. Hashmi, Barbara Cappelli, Ali Alahmari, Graziana Maria Scigliuolo, Ad Source Type: research

Systematic review/meta-analysis on efficacy of allogeneic HCT in sickle cell disease: an international effort on behalf of the Pediatric Diseases Working Party of EBMT and the Sickle Cell Transplantation International Consortium
Sickle cell disease (SCD) is the most common inherited hemoglobinopathy affecting nearly 100,000 individuals in the United States and approximately 300,000 worldwide, with low-income countries bearing the highest burden.1, 2 It is associated with several complications including debilitating morbidity, organ injury, and shortened life expectancy. Progress have been made in recent years to the overall management of SCD, namely improved health maintenance surveillance programs, penicillin prophylaxis, chronic blood transfusions, vaccines, primary and secondary stroke prophylaxis, use of hydroxyurea and approval of novel agent...
Source: Biology of Blood and Marrow Transplantation - December 10, 2020 Category: Hematology Authors: Madiha Iqbal, Tea Reljic, Selim Corbacioglu, Josu de la Fuente, Eliane Gluckman, Ambuj Kumar, Farah Yassine, Ernesto Ayala, Areej El-Jawahri, Hemant Murthy, Fahad Almohareb, Shahrukh K. Hashmi, Barbara Cappelli, Ali Alahmari, Graziana Maria Scigliuolo, Ad Source Type: research

Acute compartment syndrome in a patient with sickle cell disease.
We present a young man with HbSC disease who developed acute compartment syndrome. This is only the second report of this syndrome in a patient with HbSC disease. This is a very rare complication in HbSC disease, but it can have serious implications. PMID: 32777927 [PubMed - as supplied by publisher]
Source: Annals of the Royal College of Surgeons of England - August 10, 2020 Category: Surgery Authors: Cochrane E, Young S, Shariff Z Tags: Ann R Coll Surg Engl Source Type: research

Sickle cell disease: a comprehensive program of care from birth.
Authors: de Montalembert M, Tshilolo L, Allali S Abstract As more children are appropriately being diagnosed, the burden of sickle cell disease is increasing greatly in Africa and in high-resource countries such as the United States and Europe. Early management is mandatory, but newborn screening is not implemented everywhere. Point-of-care testing devices are increasingly being used in low-resource countries, showing good sensitivity and specificity. Because the diagnosis is often traumatic for the families, the announcement should be made by an experienced person. The development of care networks is urgently requ...
Source: Hematology ASH Education Program - December 7, 2019 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Pelosi ’s radical plan would leave patients with sickle cell disease behind
Sickle cell disease (SCD) is the most commonly inherited genetic blood disorder, affecting nearly 100,000 children and adults in the United States. Three million Americans carry the sickle cell trait and it is nearly25 times more common in people of African American descent. The disorder, which can cause irregularly shaped blood cells to get stuck in narrow vessels, inflicts substantial pain on patients. The resulting reduced oxygen flow creates life-threatening complications such as difficulty breathing, chest pains, blood clots and stroke.
Source: The Catalyst - November 14, 2019 Category: Pharmaceuticals Authors: Tom Wilbur Tags: Research and Development Rare Diseases Pelosi Plan Source Type: news

Sickle cell disease: current treatment and emerging therapies.
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 - October 31, 2019 Category: Health Management Authors: Neumayr LD, Hoppe CC, Brown C Tags: Am J Manag Care Source Type: research

Reducing Health Care Disparities in Sickle Cell Disease: A Review.
Authors: Lee L, Smith-Whitley K, Banks S, Puckrein G Abstract Sickle cell disease (SCD) is an inherited blood disorder most common among African American and Hispanic American persons. The disease can cause substantial, long-term, and costly health problems, including infections, stroke, and kidney failure, many of which can reduce life expectancy. Disparities in receiving health care among African Americans and other racial/ethnic minority groups in the United States are well known and directly related to poor outcomes associated with SCD. As an orphan disease-one that affects <200 000 persons nationwide-SCD ...
Source: Public Health Reports - October 11, 2019 Category: International Medicine & Public Health Tags: Public Health Rep Source Type: research