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Journal articles on the topic 'Sickle cell disease treatment'

Author: Grafiati

Published: 4 June 2021

Last updated: 15 February 2022

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1

Dassy, Cylia, Manou Saramba, and Dongchi Zhao. "Treatment Options for Complications of Sickle-cell Disease in Children." International Journal of Medical Reviews and Case Reports 4, Reports in Microbiology, Infecti (2020): 1. http://dx.doi.org/10.5455/ijmrcr.sickle-cell-disease-children.

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2

Ekeke, G. I., and G. O. Ibeh. "Sialic acid in sickle cell disease." Clinical Chemistry 34, no. 7 (July 1, 1988): 1443–46. http://dx.doi.org/10.1093/clinchem/34.7.1443.

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Abstract Neuraminic (sialic) acid concentrations in serum from normal and sickle cell (HbSS) subjects were determined for discrete age groups from childhood through adolescence. Values in sickle cell disease were consistently lower over the entire age range. We further investigated the effect of exogenous sialic acid on the rate of sickling reversion of HbSS erythrocytes and demonstrated that this compound in millimole per liter concentrations could revert pre-sickled erythrocytes to their normal morphology in a concentration-dependent manner. When subjected to partial de-sialation with sialidase (EC 3.2.1.18), the HbSS erythrocytes not only sickled faster upon deoxygenation, they also reverted more slowly on treatment with phenylalanine (a more efficient anti-sickling agent than sialic acid) than did untreated cells. We conclude that, in sickle cell disease, erythrocyte sialic acid content could play a significant role, not only in the control of the sickling rate in vivo, but also, after sickling has occurred, in the rate of recovery from a sickling crisis.

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3

Buchanan, George R., Michael R. DeBaun, Charles T. Quinn, and Martin H. Steinberg. "Sickle Cell Disease." Hematology 2004, no. 1 (January 1, 2004): 35–47. http://dx.doi.org/10.1182/asheducation-2004.1.35.

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Abstract 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 marshaling the potentially beneficial effects of nitric oxide are other new and exciting approaches.

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4

Steinberg, Martin H. "Hydroxyurea Treatment for Sickle Cell Disease." Scientific World JOURNAL 2 (2002): 1706–28. http://dx.doi.org/10.1100/tsw.2002.295.

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High fetal hemoglobin (HbF) levels inhibit the polymerization of sickle hemoglobin (HbS) and reduce the complications of sickle cell disease. Pharmacologic agents that can reverse the switch from γ- to β-chain synthesis — γ-globin chains characterize HbF, and sickle β-globin chains are present in HbS — or selectively increase the proportion of adult erythroid precursors that maintain the ability to produce HbF are therapeutically useful. Hydroxyurea promotes HbF production by perturbing the maturation of erythroid precursors. This treatment increases the total hemoglobin concentration, reduces the vaso-occlusive complications of pain and acute chest syndrome, and attenuates mortality in adults. It is a promising beginning for pharmacologic therapy of sickle cell disease. Still, its effects are inconsistent, trials in infants and children are ongoing, and its ultimate value — and peril — when started early in life are still unknown.

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5

Blake, Kathryn, and John Lima. "Asthma in Sickle Cell Disease: Implications for Treatment." Anemia 2011 (2011): 1–15. http://dx.doi.org/10.1155/2011/740235.

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Objective. To review issues related to asthma in sickle cell disease and management strategies.Data Source. A systematic review of pertinent original research publications, reviews, and editorials was undertaken using MEDLlNE, the Cochrane Library databases, and CINAHL from 1947 to November 2010. Search terms were [asthma] and [sickle cell disease]. Additional publications considered relevant to the sickle cell disease population of patients were identified; search terms included [sickle cell disease] combined with [acetaminophen], [pain medications], [vitamin D], [beta agonists], [exhaled nitric oxide], and [corticosteroids].Results. The reported prevalence of asthma in children with sickle cell disease varies from 2% to approximately 50%. Having asthma increases the risk for developing acute chest syndrome , death, or painful episodes compared to having sickle cell disease without asthma. Asthma and sickle cell may be linked by impaired nitric oxide regulation, excessive production of leukotrienes, insufficient levels of Vitamin D, and exposure to acetaminophen in early life. Treatment of sickle cell patients includes using commonly prescribed asthma medications; specific considerations are suggested to ensure safety in the sickle cell population.Conclusion. Prospective controlled trials of drug treatment for asthma in patients who have both sickle cell disease and asthma are urgently needed.

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6

Gardner, Renée V. "Sickle Cell Disease: Advances in Treatment." Ochsner Journal 18, no. 4 (2018): 377–89. http://dx.doi.org/10.31486/toj.18.0076.

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7

Meier, Emily Riehm. "Treatment Options for Sickle Cell Disease." Pediatric Clinics of North America 65, no. 3 (June 2018): 427–43. http://dx.doi.org/10.1016/j.pcl.2018.01.005.

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8

Brugnara, Carlo, and Martin H. Steinberg. "Developing treatment for sickle cell disease." Expert Opinion on Investigational Drugs 11, no. 5 (May 2002): 645–59. http://dx.doi.org/10.1517/13543784.11.5.645.

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9

Redding-Lallinger, Rupa, and Christine Knoll. "Sickle Cell Disease—Pathophysiology and Treatment." Current Problems in Pediatric and Adolescent Health Care 36, no. 10 (November 2006): 346–76. http://dx.doi.org/10.1016/j.cppeds.2006.07.002.

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10

Rosse, Wendell F., Mohandas Narla, Lawrence D. Petz, and Martin H. Steinberg. "New Views of Sickle Cell Disease Pathophysiology and Treatment." Hematology 2000, no. 1 (January 1, 2000): 2–17. http://dx.doi.org/10.1182/asheducation.v2000.1.2.2.

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Abstract This review addresses several areas of concern in the care of patients with sickle cell disease. In Sections I and II, the fundamental pathogenetic mechanisms of sickle cell disease and their clinical consequences are discussed. Dr. Narla presents the evidence for abnormal cell adhesiveness by SS cells and Dr. Rosse examines the role of the increased whole blood viscosity. In Section III, Dr. Petz reviews common and uncommon alloimmune consequences of transfusion in sickle cell disease and discusses the diagnosis and management of sickle cell patients with hyperhemolysis after transfusion. In Section IV, Dr. Steinberg gives an update on the use of hydroxyurea in the treatment of sickle cell disease, including the SC and S-β thalassemia variants.

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11

Rosse, Wendell F., Mohandas Narla, Lawrence D. Petz, and Martin H. Steinberg. "New Views of Sickle Cell Disease Pathophysiology and Treatment." Hematology 2000, no. 1 (January 1, 2000): 2–17. http://dx.doi.org/10.1182/asheducation.v2000.1.2.20000002.

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This review addresses several areas of concern in the care of patients with sickle cell disease. In Sections I and II, the fundamental pathogenetic mechanisms of sickle cell disease and their clinical consequences are discussed. Dr. Narla presents the evidence for abnormal cell adhesiveness by SS cells and Dr. Rosse examines the role of the increased whole blood viscosity. In Section III, Dr. Petz reviews common and uncommon alloimmune consequences of transfusion in sickle cell disease and discusses the diagnosis and management of sickle cell patients with hyperhemolysis after transfusion. In Section IV, Dr. Steinberg gives an update on the use of hydroxyurea in the treatment of sickle cell disease, including the SC and S-β thalassemia variants.

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12

Steinberg, Martin H. "Sickle Cell Disease: Present and Future Treatment." American Journal of the Medical Sciences 312, no. 4 (October 1996): 166–74. http://dx.doi.org/10.1016/s0002-9629(15)41800-2.

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13

Bunn, H. Franklin. "Pathogenesis and Treatment of Sickle Cell Disease." New England Journal of Medicine 337, no. 11 (September 11, 1997): 762–69. http://dx.doi.org/10.1056/nejm199709113371107.

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14

Jagadeeswaran, Ramasamy, and Angela Rivers. "Evolving treatment paradigms in sickle cell disease." Hematology 2017, no. 1 (December 8, 2017): 440–46. http://dx.doi.org/10.1182/asheducation-2017.1.440.

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AbstractSickle cell disease (SCD) is an inheritable hemoglobinopathy characterized by polymerization of hemoglobin S in red blood cells resulting in chronic hemolytic anemia, vaso-occlusive painful crisis, and multiorgan damage. In SCD, an increased reactive oxygen species (ROS) generation occurs both inside the red blood cells and inside the vascular lumen, which augment hemolysis and cellular adhesion. This review discusses the evolving body of literature on the role of ROS in the pathophysiology of SCD as well as some emerging therapeutic approaches to SCD with a focus on the reduction of ROS.

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15

Steinberg, Martin H. "Sickle Cell Disease: Present and Future Treatment." American Journal of the Medical Sciences 312, no. 4 (October 1996): 166–74. http://dx.doi.org/10.1097/00000441-199610000-00004.

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16

Field, Joshua J. "Can selectin and iNKT cell therapies meet the needs of people with sickle cell disease?" Hematology 2015, no. 1 (December 5, 2015): 426–32. http://dx.doi.org/10.1182/asheducation-2015.1.426.

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AbstractRecent insights into the pathogenesis of microvascular occlusion downstream of the sickled red cell have revealed new therapeutic targets for sickle cell disease (SCD). After the formation of sickle cells, tissue injury spurs inflammation, which leads to receptor-mediated contacts between sickle cells, leukocytes, and vascular endothelium. Specifically, selectins decelerate sickled red cells and leukocytes in the circulation to facilitate endothelial adhesion and other cell–cell interactions, ultimately leading to vascular occlusion. Invariant NKT (iNKT) cells, activated during reperfusion, generate a broad inflammatory response, which further increases cellular adhesion and vascular occlusion. Novel therapies are in development that target selectins and iNKT cells to prevent or interrupt the vicious cycle of adhesion and inflammation. Although the therapies hold promise for the treatment of SCD, an underappreciated threat to their development is poor access to care for people with SCD. Unless the majority of people with SCD have access to consistent, high-quality care, they will not have the opportunity to participate in a clinical trial or receive any new therapy, regardless of its efficacy.

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17

Nwagu, Marcellinus Uchechukwu, Ologo Thompson, and Akinola Oyekemi. "Advanced metastatic breast carcinoma in sickle cell disease." Universa Medicina 38, no. 2 (April 29, 2019): 139. http://dx.doi.org/10.18051/univmed.2019.v38.139-143.

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Background<br />Breast cancer is the leading cancer in women leading to over 400,000 deaths per year worldwide. It begins in the breast tissue and can metastasize to other organs if early diagnosis and treatment is not instituted. Women with sickle cell disease are usually spared from breast cancer and other solid tumours due to the tumoricidal effect of sickled erythrocytes. Breast cancers are rare among these group of patients. Despite its rare occurrence, this paper was to emphasize the need for breast cancer screening among female sickle cell disease patients who have positive family history of breast cancer.<br /><br />Case description<br />OO was a 30-year old woman with sickle cell disease who presented to the hospital one and half years ago with a seven months history of right breast swelling and pains. She had lost her mother to breast cancer about 15 years ago. Mammography and histology of breast biopsy confirmed diagnosis of invasive ductal carcinoma of the right breast. Financial constraint was a major challenge in managing this patient as she was unable to buy her chemotherapy. She developed features suggestive of metastasis such as seizures and hepatomegaly. She was stabilized and discharged home but we lost her to follow up. She died at home.<br /><br />Conclusion <br />Breast cancer is rare among females with sickle cell disease; any of them with a family history should be routinely screened for early diagnosis and treatment.

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18

Jing Li, Si-Yeon Jeong, Bei Xiong, Alan Tseng, Andrew B. Mahon, Steven Isaacman, Victor R. Gordeuk, and Jaehyung Cho. "Repurposing pyridoxamine for therapeutic intervention of intravascular cell-cell interactions in mouse models of sickle cell disease." Haematologica 105, no. 10 (October 31, 2019): 2407–19. http://dx.doi.org/10.3324/haematol.2019.226720.

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Adherent neutrophils on vascular endothelium positively contribute to cell-cell aggregation and vaso-occlusion in sickle cell disease. In the present study, we demonstrated that pyridoxamine, a derivative of vitamin B6, might be a therapeutic agent to alleviate intravascular cell-cell aggregation in sickle cell disease. Using real-time intravital microscopy, we found that one oral administration of pyridoxamine dose-dependently increased the rolling influx of neutrophils and reduced neutrophil adhesion to endothelial cells in cremaster microvessels of sickle cell disease mice challenged with hypoxia-reoxygenation. Short-term treatment also mitigated neutrophil-endothelial cell and neutrophil-platelet interactions in the microvessels and improved the survival of sickle cell disease mice challenged with tumor necrosis factor-α. The inhibitory effects of pyridoxamine on intravascular cell-cell interactions were potentiated by co-treatment with hydroxyurea. We observed that long-term (5.5 months) oral treatment with pyridoxamine significantly diminished the adhesive function of neutrophils and platelets and down-regulated the expression of E-selectin and intercellular adhesion molecule-1 on the vascular endothelium in tumor necrosis factor-α-challenged sickle cell disease mice. Ex vivo studies revealed that the surface amount of αMβ2 integrin was significantly decreased in stimulated neutrophils isolated from sickle cell disease mice treated with pyridoxamine-containing water. Studies using platelets and neutrophils from sickle cell disease mice and patients suggested that treatment with pyridoxamine reduced the activation state of platelets and neutrophils. These results suggest that pyridoxamine may be a novel therapeutic and a supplement to hydroxyurea to prevent and treat vaco-occlusion events in sickle cell disease.

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19

Javazon, Elisabeth H., Mohamed Radhi, Bagirath Gangadharan, Jennifer Perry, and David R. Archer. "Hematopoietic Stem Cell Function in a Murine Model of Sickle Cell Disease." Anemia 2012 (2012): 1–9. http://dx.doi.org/10.1155/2012/387385.

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Previous studies have shown that the sickle environment is highly enriched for reactive oxygen species (ROS). We examined the oxidative effects of sickle cell disease on hematopoietic stem cell function in a sickle mouse model.In vitrocolony-forming assays showed a significant decrease in progenitor colony formation derived from sickle compared to control bone marrow (BM). Sickle BM possessed a significant decrease in the KSL (c-kit+, Sca-1+, Lineage−) progenitor population, and cell cycle analysis showed that there were fewer KSL cells in the G0phase of the cell cycle compared to controls. We found a significant increase in both lipid peroxidation and ROS in sickle-derived KSL cells.In vivoanalysis demonstrated that normal bone marrow cells engraft with increased frequency into sickle mice compared to control mice. Hematopoietic progenitor cells derived from sickle mice, however, demonstrated significant impairment in engraftment potential. We observed partial restoration of engraftment by n-acetyl cysteine (NAC) treatment of KSL cells prior to transplantation. Increased intracellular ROS and lipid peroxidation combined with improvement in engraftment following NAC treatment suggests that an altered redox environment in sickle mice affects hematopoietic progenitor and stem cell function.

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20

Raghupathy, Radha, Deepa Manwani, and Jane A. Little. "Iron Overload in Sickle Cell Disease." Advances in Hematology 2010 (2010): 1–9. http://dx.doi.org/10.1155/2010/272940.

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In sickle cell disease transfusions improve blood flow by reducing the proportion of red cells capable of forming sickle hemoglobin polymer. This limits hemolysis and the endothelial damage that result from high proportions of sickle polymer-containing red cells. Additionally, transfusions are used to increase blood oxygen carrying capacity in sickle cell patients with severe chronic anemia or with severe anemic episodes. Transfusion is well-defined as prophylaxis (stroke) and as therapy (acute chest syndrome and stroke) for major complications of sickle cell disease and has been instituted, based on less conclusive data, for a range of additional complications, such as priapism, vaso-occlusive crises, leg ulcers, pulmonary hypertension, and during complicated pregnancies. The major and unavoidable complication of transfusions in sickle cell disease is iron overload. This paper provides an overview of normal iron metabolism, iron overload in transfused patients with sickle cell disease, patterns of end organ damage, diagnosis, treatment, and prevention of iron overload.

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21

Swerdlow, Paul S. "Red Cell Exchange in Sickle Cell Disease." Hematology 2006, no. 1 (January 1, 2006): 48–53. http://dx.doi.org/10.1182/asheducation-2006.1.48.

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Abstract Red cell exchange transfusions remain an effective but possibly underutilized therapy in the acute and chronic treatment of sickle cell disease. In sickle cell disease, increased blood viscosity can cause complications when the hemoglobin exceeds 10 g/dL even if this is due to simple transfusion. Red cell exchange can provide needed oxygen carrying capacity while reducing the overall viscosity of blood. Acute red cell exchange is useful in acute infarctive stroke, in acute chest and the multi-organ failure syndromes, the right upper quadrant syndrome, and possibly priapism. Neither simple or exchange transfusions are likely to hasten resolution of an acute pain episode.

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22

Saganuwan, Saganuwan Alhaji. "The Pattern of Sickle Cell Disease in Sickle Cell Patients from Northwestern Nigeria." Clinical Medicine Insights: Therapeutics 8 (January 2016): CMT.S38164. http://dx.doi.org/10.4137/cmt.s38164.

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Sickle cell disease is caused due to a genetic disorder, which accounts for people dying at an early age in Nigeria. A retrospective study of sickle cell disease patients was carried out with a view to determining the disease pattern in sickle cell patients from the Northwestern Nigeria. Case notes of 319 sickle cell patients were collected and reviewed retrospectively. The prevalence of sickle cell trait, comorbidity of sickle cell disease and malaria, and the effects of sickle cell disease and age on the weight and hematological parameters of sickle cell patients were determined and analyzed. Results showed the prevalence rate of sickle cell trait to be 61.8% (197) and that of non-sickle cell trait to be 38.2% (122). The sickle cell trait comprised 96 males (48.7%) and 101 females (51.3%). Among these patients, 51 (41.8%) males and 71 (58.2%) females had malaria. However, 35.4% (113) of sickle cell patients and 7.5% (24) of malaria patients showed anemia. Genotyping revealed 32 AS (16.2%), 102 SS (51.8%), SS+F (3.6%), and 56 SC (28.4%). The associated prevalence rates of clinical signs were pain/crisis 45.1% (89), pneumonia 28.4% (56), gastric disorders 9.1% (18), central nervous system (CNS) disorders 4.1% (8), renal diseases 2.5% (5), musculo-skeletal disorders 2.5% (5), conjunctivitis 0.5% (1), acute chest syndrome 0.5% (1), cholecystitis 0.5% (1), hemophilia 0.5% (1), fever 0.5% (1), priapism 2.0% (4), splenomegaly 2.0% (4), and epistaxis 1.5% (3). Few patients lived up to 49 years. There was significant difference ( P < 0.05) in hematological parameters of the patients from various age groups. The use of anti-sickling, hematonic, analgesic, anti-inflammatory, and antimalarial drugs in the treatment of the affected disease in patients might have improved their quality of life.

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23

Couillard, S., M. Benkerrou, R. Girot, V. Brousse, A. Ferster, and B. Bader-Meunier. "Steroid treatment in children with sickle-cell disease." Haematologica 92, no. 3 (March 1, 2007): 425–26. http://dx.doi.org/10.3324/haematol.10800.

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24

Podmore, Adrian H. B. "Haptoglobin for the treatment of sickle cell disease." Bioscience Hypotheses 1, no. 1 (January 2008): 59–63. http://dx.doi.org/10.1016/j.bihy.2008.01.011.

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25

Steinberg, Martin H. "Pathophysiologically based drug treatment of sickle cell disease." Trends in Pharmacological Sciences 27, no. 4 (April 2006): 204–10. http://dx.doi.org/10.1016/j.tips.2006.02.007.

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26

Fantasia, Heidi Collins, and Brenna L. Morse. "Voxelotor for the Treatment of Sickle Cell Disease." Nursing for Women's Health 24, no. 3 (June 2020): 233–37. http://dx.doi.org/10.1016/j.nwh.2020.03.003.

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27

Kapoor, Sargam, Jane A. Little, and Lydia H. Pecker. "Advances in the Treatment of Sickle Cell Disease." Mayo Clinic Proceedings 93, no. 12 (December 2018): 1810–24. http://dx.doi.org/10.1016/j.mayocp.2018.08.001.

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Monfort, Jean-Benoît, and Patricia Senet. "Leg Ulcers in Sickle-Cell Disease: Treatment Update." Advances in Wound Care 9, no. 6 (June 1, 2020): 348–56. http://dx.doi.org/10.1089/wound.2018.0918.

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29

Herity, Leah B., DaleMarie M. Vaughan, Lindsey Ritenour Rodriguez, and Denise Kozella Lowe. "Voxelotor: A Novel Treatment for Sickle Cell Disease." Annals of Pharmacotherapy 55, no. 2 (July 16, 2020): 240–45. http://dx.doi.org/10.1177/1060028020943059.

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Objective: To review the pharmacological characteristics, clinical evidence, and place in therapy of voxelotor for the treatment of sickle cell disease (SCD). Data Sources: A comprehensive literature search of PubMed (1966 to April 2020) was conducted. Key search terms included GBT440, sickle cell, and voxelotor. Other sources were derived from bibliographies of articles, product labeling, manufacturer’s website, and news releases. ClinicalTrials.gov was searched for additional studies. Study Selection and Data Extraction: All English-language articles identified from the data sources were reviewed and evaluated. Case reports/series and phase 1 through 3 clinical trials were included. Data Synthesis: SCD is an inherited disorder associated with significant morbidity and early mortality. Three medications approved for SCD reduce SCD-associated complications but do not selectively ameliorate the underlying disease. Voxelotor is a novel agent that targets the pathophysiology of SCD. A phase 3 trial reported an increase in mean Hb level from baseline for voxelotor compared with placebo (1.1 vs −0.1 g/dL; P < 0.001). Voxelotor is generally well tolerated, with common adverse effects including headache, diarrhea, nausea, and arthralgia. Relevance to Patient Care and Clinical Practice: Voxelotor may be considered for patients with SCD who have continued anemia and hemolysis despite being on maximum tolerated dose of hydroxyurea or in those who are hydroxyurea intolerant. Voxelotor is costly; therefore, both cost and benefit should be weighed before prescribing. Conclusion: Voxelotor appears to be safe and effective as monotherapy or in combination with hydroxyurea for patients with SCD who are 12 years of age and older.

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Coco, Megan. "Nonpharmacologic Treatment of Pain in Sickle Cell Disease." Topics in Pain Management 34, no. 5 (December 2018): 1–8. http://dx.doi.org/10.1097/01.tpm.0000549768.92473.6d.

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El-Hazmi, Mohsen A. F., Arjum S. Warsy, Abdulkarim Al-Momen, and Mohamed Harakati. "Hydroxyurea for the Treatment of Sickle Cell Disease." Acta Haematologica 88, no. 4 (1992): 170–74. http://dx.doi.org/10.1159/000204681.

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32

Vissa, Madhav, and Elliott Vichinsky. "Voxelotor for the treatment of sickle cell disease." Expert Review of Hematology 14, no. 3 (March 4, 2021): 253–62. http://dx.doi.org/10.1080/17474086.2021.1893688.

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33

Walters, Mark C., Arthur W. Nienhuis, and Elliott Vichinsky. "Novel Therapeutic Approaches in Sickle Cell Disease." Hematology 2002, no. 1 (January 1, 2002): 10–34. http://dx.doi.org/10.1182/asheducation-2002.1.10.

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Abstract In this update, selected clinical features of sickle cell disease and their management are reviewed. In addition, the current status of interventions that have curative potential for sickle cell disease is discussed, with particular attention focused on indications, methodology, recent results, and challenges to wider clinical application. In Section I, Dr. Nienhuis describes recent improvements in vector technology, safety, and replacement gene expression that are creating the potential for clinical application of this technology. In Section II, Dr. Vichinsky reviews our current understanding of the pathophysiology and treatment of pulmonary injury in sickle cell disease. The acute and chronic pulmonary complications of sickle cell disease, modulators and predictors of severity, and conventional and novel treatment of these complications are discussed. In Section III, Dr. Walters reviews the current status of hematopoietic cell transplantation for sickle cell disease. Newer efforts to expand its availability by identifying alternate sources of stem cells and by reducing the toxicity of transplantation are discussed.

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34

Ataga, Kenneth I. "Novel therapies in sickle cell disease." Hematology 2009, no. 1 (January 1, 2009): 54–61. http://dx.doi.org/10.1182/asheducation-2009.1.54.

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AbstractDespite an increased understanding of the pathophysiology of sickle cell disease (SCD), there remains a paucity of available agents for the prevention and treatment of specific SCD-related complications. Recently, there has been significant progress in the development of novel drugs for this disease. These agents, which increase the production of fetal hemoglobin, improve red blood cell hydration, increase the availability of nitric oxide and possess anti-inflammatory effects, are in varying stages of clinical development. With the complex pathophysiology of SCD, it is unlikely that a single agent will prevent or treat all the sequelae of this disease. As a result, patients may benefit from treatment with a combination of agents that possess different mechanisms of action. This overview discusses selected novel agents that appear promising in SCD.

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Ataga, Kenneth I. "Novel therapies in sickle cell disease." Hematology 2009, no. 1 (January 1, 2009): 54–61. http://dx.doi.org/10.1182/asheducation.v2009.1.54.0010054.

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Despite an increased understanding of the pathophysiology of sickle cell disease (SCD), there remains a paucity of available agents for the prevention and treatment of specific SCD-related complications. Recently, there has been significant progress in the development of novel drugs for this disease. These agents, which increase the production of fetal hemoglobin, improve red blood cell hydration, increase the availability of nitric oxide and possess anti-inflammatory effects, are in varying stages of clinical development. With the complex pathophysiology of SCD, it is unlikely that a single agent will prevent or treat all the sequelae of this disease. As a result, patients may benefit from treatment with a combination of agents that possess different mechanisms of action. This overview discusses selected novel agents that appear promising in SCD.

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36

Field, Joshua J., David G. Nathan, and Joel Linden. "Targeting iNKT cells for the treatment of sickle cell disease." Clinical Immunology 140, no. 2 (August 2011): 177–83. http://dx.doi.org/10.1016/j.clim.2011.03.002.

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37

Wang, Zhiping, Gregory Kishchenko, Yimei Chen, and Robert Josephs. "Polymerization and Three Dimensional Reconstruction of Deoxy-Sickle Cell Hemoglobin Fibers in High and Low Phosphate Buffers." Microscopy and Microanalysis 6, S2 (August 2000): 240–41. http://dx.doi.org/10.1017/s1431927600033699.

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The amino acid substitution (B Glu → G6 Val) results in the conversion of Hemoglobin A (HbA) to sickle cell hemoglobin(HbS) which is responsible for sickle cell disease. Under physiological conditions this substitution causes a reduction of the solubility of HbA from about 340 mg/ml to 165 mg/ml (for HbS). One consequence of the reduction in solubility is that HbS polymerizes to form long fiber like structures about 240Å in diameter. The formation of these fibers causes sickle cell disease. The fibers fill the red cell and cause it to assume a characteristic sickle shape. More significantly the fibers cause the red cell to become rigid and, as a result, sickled cells can block blood flow in the capillaries. Understanding the polymerization process in detail is important for understanding the pathophysiology of sickle cell disease and for developing a specific therapy that could be used in its treatment.

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Atkins, Robert C., and Mark C. Walters. "Haematopoietic cell transplantation in the treatment of sickle cell disease." Expert Opinion on Biological Therapy 3, no. 8 (December 2003): 1215–24. http://dx.doi.org/10.1517/14712598.3.8.1215.

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39

Rivolo, Massimo. "SICKLE as a holistic treatment approach to sickle cell disease related ulcers." British Journal of Nursing 27, no. 20 (November 8, 2018): S6—S10. http://dx.doi.org/10.12968/bjon.2018.27.sup20.s6.

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Achieng, Benta, Elton Akpabio, Joseph Balikwisha, Monica Omondi, Yuanita Hongo, Peter Chappell, and Harry van der Zee. "Treating Sickle Cell Disease in Africa." Homœopathic Links 31, no. 02 (June 2018): 120–26. http://dx.doi.org/10.1055/s-0038-1660439.

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SummarySickle cell disease (SCD) is a condition causing lots of complications and suffering. In the past decades, treatment modalities have been developed that increase life expectancy and the quality of life of SCD patients. In Africa, these are not available, resulting in SCD patients to die young after many hospitalisations for serious and often painful crises. A new homeopathy-based remedy for SCD has been used with good results since 2013.

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Adams, Robert J., Kwaku Ohene-Frempong, and Winfred Wang. "Sickle Cell and the Brain." Hematology 2001, no. 1 (January 1, 2001): 31–46. http://dx.doi.org/10.1182/asheducation-2001.1.31.

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Abstract Sickle cell disease affects many organ systems, but one of the major morbidities is brain disease, especially stroke. In this paper, the etiology, diagnosis, treatment, and prevention of clinical stroke, as well as so-called “silent stroke,” are examined. Risk factors, diagnostic tools, and data from prevention and treatment studies as well as issues pertaining to neuropsychological function, especially in younger patients, are discussed and current best options for treatment considered.

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Javazon, Elisabeth H., Leslie S. Kean, Jennifer Perry, Jessica Butler, and David R. Archer. "Effects of Sickle Cell Disease on Hematopoietic Stem Cell Function and the Bone Marrow Microenvironment." Blood 108, no. 11 (November 16, 2006): 1227. http://dx.doi.org/10.1182/blood.v108.11.1227.1227.

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Abstract Gene therapy and stem cell transplantation are attractive potential therapies for sickle cell disease (SCD). Previous studies have shown that the sickle environment is highly enriched for reactive oxygen species (ROS), but have not addressed whether or not the increased ROS may alter the bone marrow (BM) microenvironment or affect stem cell function. Using the Berkeley sickle mouse model, we examined the effects of sickle cell disease on hematopoietic stem cell function and the bone marrow microenvironment. We transplanted C57BL/6 (control) BM into C57BL/6 and homozygous sickle mice. Recipients received 2 × 106 BM cells and a conditioning regimen consisting of busulfan, anti-asialo GM1, and co-stimulation blockade (anti-CD40L and CTLA4-Ig). Following transplantation, sickle mice demonstrated increased donor cell engraftment in the peripheral blood compared to normal mice (58.3% vs. 33.1%, respectively). Similarly, BMT in a fully allogeneic system also resulted in enhanced engraftment in sickle recipients. Next we analyzed whether or not engraftment defects exist within the BM stem cell population of sickle mice. In vitro colony forming assays showed a significant decrease in progenitor colony formation in sickle compared to control BM. By flow cytometry, we determined that there was a significant decrease in the KSL (c-Kit+, Sca-1+, Lineage−) progenitor population within the BM of sickle mice. Cell cycle analysis of the KSL population demonstrated that significantly fewer sickle KSL cells were in G0 phase compared to control, suggesting that there are fewer quiescent stem cells in the BM of sickle mice. To assess the potential role of ROS and glutathione depletion in sickle mice, we tested the engraftment efficiency of KSL cells from untreated and n-acetyl-cysteine (NAC) treated control, hemizygous sickle (hemi), and sickle mice in a competitive repopulation experiment. Peripheral chimerism showed an engraftment defect from both hemizygous and homozygous sickle mice such that control KSL cells engrafted > hemi > sickle at a ratio of 1 : 0.4 : 0.25. Treatment with NAC for four months prior to transplantation partially restored KSL engraftment (control : hemi : sickle; 1 : 0.97 : 0.56 ). We have demonstrated that congenic and allogeneic BMT into sickle mice result in increased donor cell engraftment in the sickle recipients. Both the decreased number of KSL cells and the decreased percentage of quiescent KSL cells in the sickle mice indicate that more stem cells in the transgenic sickle mouse model are mobilized from the BM environment. The engraftment defect of sickle KSL cells that was partially ameliorated by NAC treatment suggests that an altered redox environment in sickle mice may contribute to the engraftment deficiencies that we observed.

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Beckman, Joan D., John D. Belcher, Julie V. Vineyard, Chunsheng Chen, Julia Nguyen, M. Osita Nwaneri, M. Gerard O'Sullivan, Evin Gulbahce, Robert P. Hebbel, and Gregory M. Vercellotti. "Inhaled carbon monoxide reduces leukocytosis in a murine model of sickle cell disease." American Journal of Physiology-Heart and Circulatory Physiology 297, no. 4 (October 2009): H1243—H1253. http://dx.doi.org/10.1152/ajpheart.00327.2009.

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Carbon monoxide (CO) has anti-inflammatory properties. We previously reported that acute treatments with inhaled CO inhibit vascular inflammation and hypoxia-induced vasoocclusion in sickle cell disease mouse models. Therefore, we hypothesized that chronic CO inhalation would decrease vascular inflammation and organ pathology in a sickle cell disease mouse model. The treatment of sickle cell disease mice with 25 or 250 parts/million inhaled CO for 1 h/day, 3 days/wk for 8–10 wk significantly decreased the total mean white blood cell, neutrophil, and lymphocyte counts in peripheral blood. Eight weeks of 250 parts/million CO treatments reduced staining for myeloid and lymphoid markers in the bone marrow of sickle mice. Bone marrow from treated sickle mice exhibited a significant decrease in colony-forming unit granulocyte-macrophage during colony-forming cell assays. Anti-inflammatory signaling pathways phospho-Akt and phospho-p38 MAPK were markedly increased in CO-treated sickle livers. Importantly, CO-treated sickle mice had a significant reduction in liver parenchymal necrosis, reflecting the anti-inflammatory benefits of CO. We conclude that inhaled CO may be a beneficial anti-inflammatory therapy for sickle cell disease.

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Boisson, Camille, Minke A. E. Rab, Elie Nader, Céline Renoux, Celeste Kanne, Jennifer Bos, Brigitte A. van Oirschot, et al. "Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease." Cells 10, no. 4 (April 5, 2021): 811. http://dx.doi.org/10.3390/cells10040811.

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(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment.

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Inusa, Baba, Lewis Hsu, Neeraj Kohli, Anissa Patel, Kilali Ominu-Evbota, Kofi Anie, and Wale Atoyebi. "Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment." International Journal of Neonatal Screening 5, no. 2 (May 7, 2019): 20. http://dx.doi.org/10.3390/ijns5020020.

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Sickle cell disease (SCD) is a monogenetic disorder due to a single base-pair point mutation in the β-globin gene resulting in the substitution of the amino acid valine for glutamic acid in the β-globin chain. Phenotypic variation in the clinical presentation and disease outcome is a characteristic feature of the disorder. Understanding the pathogenesis and pathophysiology of the disorder is central to the choice of therapeutic development and intervention. In this special edition for newborn screening for haemoglobin disorders, it is pertinent to describe the genetic, pathologic and clinical presentation of sickle cell disease as a prelude to the justification for screening. Through a systematic review of the literature using search terms relating to SCD up till 2019, we identified relevant descriptive publications for inclusion. The scope of this review is mainly an overview of the clinical features of pain, the cardinal symptom in SCD, which present following the drop in foetal haemoglobin as young as five to six months after birth. The relative impact of haemolysis and small-vessel occlusive pathology remains controversial, a combination of features probably contribute to the different pathologies. We also provide an overview of emerging therapies in SCD.

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Rosse, W. F. "New Views of Sickle Cell Disease Pathophysiology and Treatment." Hematology 2000, no. 1 (January 1, 2000): 2–17. http://dx.doi.org/10.1182/asheducation-2000.1.2.

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Brugnara, Carlo, Lucia De Franceschi, and Yves Beuzard. "Erythrocyte-active agents and treatment of sickle cell disease." Seminars in Hematology 38, no. 4 (October 2001): 324–32. http://dx.doi.org/10.1016/s0037-1963(01)90026-5.

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Hendricks-Ferguson, Verna, and Martha A. Nelson. "Treatment of Cholelithiasis in Children with Sickle Cell Disease." AORN Journal 77, no. 6 (June 2003): 1169–82. http://dx.doi.org/10.1016/s0001-2092(06)60979-4.

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Conran, Nicola, and David C. Rees. "Prasugrel hydrochloride for the treatment of sickle cell disease." Expert Opinion on Investigational Drugs 26, no. 7 (June 12, 2017): 865–72. http://dx.doi.org/10.1080/13543784.2017.1335710.

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Reed, MD, W., and E. P. Vichinsky, MD. "NEW CONSIDERATIONS IN THE TREATMENT OF SICKLE CELL DISEASE." Annual Review of Medicine 49, no. 1 (February 1998): 461–74. http://dx.doi.org/10.1146/annurev.med.49.1.461.

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