The Experts below are selected from a list of 216 Experts worldwide ranked by ideXlab platform
F Galacteros - One of the best experts on this subject based on the ideXlab platform.
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clinical and laboratory factors associated with the severity of proliferative Sickle Cell retinopathy in patients with Sickle Cell hemoglobin c sc and homozygous Sickle Cell ss disease
Medicine, 2011Co-Authors: Nicolas Leveziel, Sylvie Bastujigarin, Franck Lalloum, Giuseppe Querques, P Benlian, M Binaghi, G Coscas, Gisele Soubrane, Dora Bachir, F GalacterosAbstract:AbstractProliferative Sickle Cell retinopathy (PSCR) is the most frequent vision-threatening complication of Sickle Cell disease (SCD). We investigated the relationship between the severity of Sickle Cell retinopathy in heterozygous (SC) or homozygous (SS) adult SCD patients and the clinical and lab
Nicolas Leveziel - One of the best experts on this subject based on the ideXlab platform.
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clinical and laboratory factors associated with the severity of proliferative Sickle Cell retinopathy in patients with Sickle Cell hemoglobin c sc and homozygous Sickle Cell ss disease
Medicine, 2011Co-Authors: Nicolas Leveziel, Sylvie Bastujigarin, Franck Lalloum, Giuseppe Querques, P Benlian, M Binaghi, G Coscas, Gisele Soubrane, Dora Bachir, F GalacterosAbstract:AbstractProliferative Sickle Cell retinopathy (PSCR) is the most frequent vision-threatening complication of Sickle Cell disease (SCD). We investigated the relationship between the severity of Sickle Cell retinopathy in heterozygous (SC) or homozygous (SS) adult SCD patients and the clinical and lab
Martin H. Steinberg - One of the best experts on this subject based on the ideXlab platform.
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Sickle Cell Disease.
The New England journal of medicine, 2017Co-Authors: Frédéric B Piel, Martin H. Steinberg, David C. ReesAbstract:Sickle Cell disease is an increasing global health problem. Estimates suggest that every year approximately 300,000 infants are born with Sickle Cell anemia, which is defined as homozygosity for the Sickle hemoglobin (HbS) gene (i.e., for a missense mutation [Glu6Val, rs334] in the β-globin gene [HBB]) and that this number could rise to 400,000 by 2050.1 Although early diagnosis, penicillin prophylaxis, blood transfusion, transcranial Doppler imaging, hydroxyurea, and hematopoietic stem-Cell transplantation can dramatically improve survival and quality of life for patients with Sickle Cell disease, our understanding of the role of genetic and nongenetic factors in explaining the remarkable phenotypic diversity of this mendelian disease is still limited. Better prediction of the severity of Sickle Cell disease could lead to more precise treatment and management. Beyond well-known modifiers of disease severity, such as fetal hemoglobin (HbF) levels and α-thalassemia, other genetic variants might affect specific subphenotypes. Similarly, although the influence of altitude and temperature has long been reflected in advice to patients with Sickle Cell disease, recent studies of nongenetic factors, including climate and air quality, suggest more complex associations between environmental factors and clinical complications.2 New treatments and management strategies accounting for these genetic and nongenetic factors could substantially and rapidly improve the quality of life and reduce health care costs for patients with Sickle Cell disease
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Sickle Cell disease
Hematology. American Society of Hematology. Education Program, 2004Co-Authors: George R. Buchanan, Michael R. Debaun, Charles T. Quinn, Martin H. SteinbergAbstract:Much progress has been made during the past several decades in gaining understanding about the natural history of Sickle Cell disease and management approaches aimed at treating or even preventing certain disease complications. The characterization of the human genome now offers the opportunity to understand relationships regarding how gene polymorphisms as well as how environmental factors affect the Sickle Cell disease phenotype, i.e., the individual patient's overall clinical severity as well as their specific organ function. This chapter explores some of these recent advances in knowledge. In Section I, Dr. Michael DeBaun characterizes the problem of silent stroke in Sickle Cell disease, comparing and contrasting its clinical and neuroimaging features with overt stroke. Combined, these events affect virtually 40% of children with Sickle Cell anemia. New understanding of risk factors, associated clinical findings, and imaging technologies are impacting substantially on treatment options. The appreciable cognitive dysfunction and other sequelae of silent infarct demand more effective treatments and ultimate prevention. In Section II, Dr. Charles Quinn addresses the conundrum of why some patients with Sickle Cell disease do well whereas others fare poorly. Some risk factors have been known for years, based upon careful study of hundreds of patients by the Cooperative Study for Sickle Cell Disease and investigators studying the Jamaican newborn cohort. Other prognostic measures have only recently been defined. Dr. Quinn devotes special attention to stroke and chest syndrome as organ-related complications but also describes attempts to measure overall disease severity and to predict survival. Recently, investigators have attempted to predict factors responsible for early mortality in children and following onset of pulmonary hypertension in adults. In Section III, Dr. Martin Steinberg reviews pharmacologic approaches to Sickle Cell disease and the rationale for their use. In addition to the inhibition of hemoglobin S polymerization, newer targets have been defined during the past one to two decades. These include the erythrocyte membrane, changes in the red Cell intraCellular content (especially loss of water), endothelial injury, and free radical production. Hydroxyurea treatment attracted the greatest interest, but many uncertainties remain about its long-term benefits and toxicities. Newer "anti-sickling" agents such as decitabine and short-chain fatty acids also receive attention. Prevention of red Cell dehydration, "anti-endothelial" therapy, and marshalling the potentially beneficial effects of nitric oxide are other new and exciting approaches.
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Management of Sickle Cell Disease
The New England Journal of Medicine, 1999Co-Authors: Martin H. SteinbergAbstract:One of every 600 black people in the United States has Sickle Cell anemia. In addition, Sickle Cell–hemoglobin C disease and Sickle Cell–β-thalassemia, which are other common genotypes of Sickle Cell disease, together are as common as Sickle Cell anemia. Sickle hemoglobin (hemoglobin S, α2 β2S) accounts for over half the hemoglobin in patients with these disorders. Eight percent of black Americans are heterozygous carriers of the Sickle Cell trait; about 40 percent of their hemoglobin is hemoglobin S. They do not have anemia and need neither treatment nor occupational restrictions. About 5 percent have hematuria at . . .
Michael F. Chicella - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Sickle Cell pain.
Pharmacotherapy, 2002Co-Authors: Karen F. Marlowe, Michael F. ChicellaAbstract:Sickle Cell disease affects 70,000 Americans who experience an average of 0.8 painful episodes each year. The pathophysiology of Sickle Cell pain is not completely understood. The disease is characterized by both acute and chronic pain syndromes. Patients with Sickle Cell pain often encounter barriers to receiving appropriate care, including lack of continuity of care and perceived opiate addiction. Studies describing pharmacotherapy for Sickle Cell pain have been primarily retrospective and uncontrolled. In analyzing the available literature regarding pathophysiology, assessment, and treatment of Sickle Cell pain, we found a need for increased practitioner education and intervention to improve the level of care provided to patients with this disease.
Harold Dauerman - One of the best experts on this subject based on the ideXlab platform.
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Endothelial Function in Patients with Sickle Cell Anemia During and After Sickle Cell Crises
Journal of Thrombosis and Thrombolysis, 2005Co-Authors: Arnon Blum, Shay Yeganeh, Aviva Peleg, Fina Vigder, Konstantin Kryuger, Ahmed Khatib, Khalid Khazim, Harold DauermanAbstract:Background: Prior studies have demonstrated increased adherence of Sickle Cell erythrocytes to vascular endothelial Cells. While decreased production of nitric oxide and increased production of adhesion molecules have been implicated in this pathophysiology, the relative contribution of these mechanisms during acute Sickle Cell crises as compared to steady state conditions have not been elucidated. Methods and results: We studied 10 consecutive young adult patients presenting with a Sickle Cell crisis. Endothelial function was evaluated by a non-invasive brachial artery shear stress method. Serum levels of adhesion molecules were obtained during the crisis. Both brachial artery responsiveness and serum levels of adhesion molecules were then repeated at steady state. Ten age and gender matched volunteers served as a control group. Impaired endothelial function and impaired endothelium-independent vasodilatation were observed in all Sickle Cell patients during both steady state and during crisis. Flow mediated dilation (FMD)% was 3.25 ± 2.76% during crisis, 4.57 ± 4.11 at steady state, compared with the control group FMD of 11.64 ± 7.69% ( p < 0.001). Flow independent dilation was 10.35 ± 11.3% during crisis, 10.03 ± 6.52% at steady state, compared with control group FID of 24.17 ± 11.87% ( p < 0.001). Levels of Cell adhesion molecules and markers of inflammation were increased in Sickle Cell crisis patients compared with the control group: sCD40 ligand levels during the acute crisis were over twice the level of normal matched volunteers ( p = 0.02), and similarly significant increases were seen for E-selectin ( p = 0.008), ICAM-1 ( p = 0.037) and VCAM-1 levels ( p = 0.01). The levels of each of these biomarkers was not significantly increased during acute crises as compared to patients’ recovery state. Conclusions: Sickle Cell anemia patients have severe systemic endothelial dysfunction as demonstrated by both brachial artery assessment and increased serum levels of adhesion molecules. These abnormalities characterize not only the Sickle Cell crisis but also the steady state pathophysiology of Sickle Cell anemia.
