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Pulmonary Hypertension in Sickle Cell Anemia

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41. Cardiovascular manifestations of sickle cell disease. (Abstract)

Cardiovascular manifestations of sickle cell disease. Sickle cell disease (SCD) is the most frequent genetic haemoglobinopathy worldwide. Early childhood mortality has dramatically decreased in high-income countries, and most patients now survive beyond the 5th decade. However, in the aging SCD population, the morbidity related to chronic organ damage, especially kidney and heart, has become a major concern. While pulmonary hypertension has attracted most attention, it appears (...) that this condition is frequently linked to left heart failure (HF). Accordingly, SCD-associated cardiomyopathy is emerging as a major cause of reduced quality of life and early mortality in these patients. The diagnosis of this particular phenotype of high-output HF is challenging. Exercise intolerance and dyspnoea in SCD patients are linked to multiple causes including chronic anaemia. Moreover, echocardiographic features are unusual and can be misinterpreted. The classical diagnosis algorithm for HF

2019 European Heart Journal

42. Sickle cell disease: current treatment and emerging therapies. (Abstract)

consequences across multiple organ systems, including acute pain episodes, chronic pain syndromes, acute chest syndrome, anemia, stroke and silent cerebral infarcts, cognitive dysfunction, pulmonary hypertension, and a wide range of other clinical consequences. Hydroxyurea was the only approved treatment for SCD for nearly 2 decades; in 2017, L-glutamine oral powder was approved for the prevention of the acute complications of SCD. During the last several years there has been a dramatic increase (...) Sickle cell disease: current treatment and emerging therapies. 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

2019 American Journal Of Managed Care

43. MKSAP: 24-year-old woman with sickle cell anemia

chest syndrome (ACS), surgical interventions, secondary prevention of stroke or ACS, and, possibly, prevention of priapism, pulmonary hypertension, and nonhealing ulcers. Transfusion is not indicated for uncomplicated pregnancy, routine painful episodes, minor surgery not requiring anesthesia, or asymptomatic anemia. Erythrocyte exchange transfusion is indicated for acute ischemic stroke, ACS with significant hypoxia, and multiorgan failure/hepatopathy as well as in persons in whom simple (...) , in SCD, erythropoietin levels are typically high to augment bone marrow erythrocyte production in response to chronic hemolysis. Therefore, ESAs are not indicated for treatment of the anemia associated with SCD. Key Point In patients with sickle cell disease, including pregnant patients, transfusion is not indicated for uncomplicated pregnancy, routine painful episodes, minor surgery not requiring anesthesia, or asymptomatic anemia. This content is excerpted from with permission from the (ACP). Use

2017 KevinMD blog

44. Quantifying the Presence of Lung Disease and Pulmonary Hypertension in Children With Sickle Cell Disease

of acute chest syndrome (ACS) (dyspnea, fever, wheezing, hypoxia, cough, chest pain). In addition, we will track any respiratory or cardiac symptoms or therapies for each subject 6 years after enrollment up to age 18 years using the registry. As standard of care, refer any child identified as having lung disease or pulmonary hypertension to a pediatric pulmonologist and/or cardiologist for monitoring, treatment and ongoing care. Condition or disease Intervention/treatment Phase Sickle Cell Disease Drug (...) Peterson-Carmichael, MD Duke University More Information Go to Layout table for additonal information Responsible Party: Duke University ClinicalTrials.gov Identifier: Other Study ID Numbers: Pro00040933 First Posted: July 11, 2013 Last Update Posted: December 3, 2014 Last Verified: December 2014 Keywords provided by Duke University: sickle cell disease pulmonary hypertension airway hyperreactivity acute chest syndrome Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Lung

2013 Clinical Trials

45. Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology Full Text available with Trip Pro

Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology Cardiopulmonary complications are the leading cause of mortality in sickle cell anemia (SCA). Elevated tricuspid regurgitant jet velocity, pulmonary hypertension, diastolic, and autonomic dysfunction have all been described, but a unifying pathophysiology and mechanism explaining the poor prognosis and propensity to sudden death has been elusive. Herein, SCA mice underwent a longitudinal comprehensive cardiac (...) analysis, combining state-of-the-art cardiac imaging with electrocardiography, histopathology, and molecular analysis to determine the basis of cardiac dysfunction. We show that in SCA mice, anemia-induced hyperdynamic physiology was gradually superimposed with restrictive physiology, characterized by progressive left atrial enlargement and diastolic dysfunction with preserved systolic function. This phenomenon was absent in WT mice with experimentally induced chronic anemia of similar degree

2016 Proceedings of the National Academy of Sciences of the United States of America

46. Biochemical surrogate markers of hemolysis do not correlate with directly measured erythrocyte survival in sickle cell anemia. Full Text available with Trip Pro

Biochemical surrogate markers of hemolysis do not correlate with directly measured erythrocyte survival in sickle cell anemia. Hemolysis is a key feature of sickle cell anemia (HbSS). Direct quantitation of hemolysis could be used as an objective outcome in clinical trials of new therapeutics for HbSS and would also enable better human studies of the pathogenesis of complications of HbSS that are ostensibly hemolysis-related, such as pulmonary hypertension. However, contemporary human studies (...) in HbSS have used only surrogate markers of hemolysis rather than direct measurements of RBC survival. We directly quantified hemolysis in HbSS by measuring survival of an age cohort of RBCs labeled with a stable isotope, administered orally as 15 N-glycine, a metabolic precursor of heme. The atomic excess of 15 N in heme extracted from blood was monitored by mass spectrometry over time. We performed 13 labeling experiments in 11 individuals with HbSS. Mean RBC survival was 31.9 days (range 14.1-53.6

2016 American journal of hematology

47. Simple chronic transfusion therapy, a crucial therapeutic option for sickle cell disease, improves but does not normalize blood rheology: What should be our goals for transfusion therapy? Full Text available with Trip Pro

Simple chronic transfusion therapy, a crucial therapeutic option for sickle cell disease, improves but does not normalize blood rheology: What should be our goals for transfusion therapy? Sickle cell anemia is characterized by a mutation resulting in the formation of an abnormal beta-hemoglobin called hemoglobin S. Hemoglobin S polymerizes upon deoxygenation, causing impaired red blood cell deformability and increased blood viscosity at equivalent hematocrits. Thus, sickle cell disease (...) is a hemorheologic disease that results in various pathologic processes involving multiple organ systems including the lungs, heart, kidneys and brain. Red blood cell mechanics and the perturbations on blood flow-endothelial interaction underlie much of the pathology found in sickle cell disease. Transfusion therapy is one of the few therapeutic options available to patients, acting as both primary and secondary prevention of stroke. Transfusion therapy, both simple and exchange, is also used for unremitting

2018 Clinical hemorheology and microcirculation

48. Efficacy of a Decision Aid for Hydroxyurea in Sickle Cell Disease

Studies a U.S. FDA-regulated Drug Product: No Studies a U.S. FDA-regulated Device Product: No Keywords provided by Lakshmanan Krishnamurti, Emory University: Blood Disorders Hydroxyurea (HU) Decision Aid Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Hydroxyurea Antineoplastic Agents Antisickling Agents Enzyme Inhibitors Molecular Mechanisms (...) receiving pretest surveys; and 2) subjects that do not receive pretest surveys. Condition or disease Intervention/treatment Phase Sickle Cell Disease Other: Training for use of web based Decision Aid Other: No training for use of web based Decision Aid Other: Pretest surveys and posttest surveys Other: Only posttest surveys Not Applicable Detailed Description: Hydroxyurea (HU) has been demonstrated to be efficacious in reducing complications such as vasocclusive pain crises and acute chest syndrome

2018 Clinical Trials

49. Sleep Apnea in Sickle Cell Disease

severity of patients and the frequency of VOC. Condition or disease Intervention/treatment Phase Sickle Cell Disease Diagnostic Test: polysomnography and oxygen saturation exam Biological: calculation of VOC frequency between the first polysomnography and the end of the first year of continuous positive airway pressure treatment Biological: Blood samples Other: Physiological measurements Other: Continuous Positive Airway Pressure Not Applicable Study Design Go to Layout table for study information (...) of microvascular reactivity and autonomic nervous system activity Experimental: SS patients apneic Homozygous sickle cell patients after one year of continuous positive airway pressure treatment Each patient will undergo the following: polysomnography and oxygen saturation exam calculation of VOC rate within the two previous years Blood samples Physiological measurements Diagnostic Test: polysomnography and oxygen saturation exam Measurement of the Apnea/hypopnea index (AHI) and oxygen saturation Biological

2018 Clinical Trials

50. Hemodynamic Characteristics and Predictors of Pulmonary Hypertension in Patients With Sickle Cell Disease. Full Text available with Trip Pro

in TRV often observed in patients with sickle cell disease is rarely associated with a high pulmonary vascular resistance and that multiple factors-including the compensatory high output state associated with anemia, pulmonary venous hypertension, and pulmonary vasculopathy-can contribute to an elevated pulmonary arterial pressure in these patients.Copyright © 2012 Elsevier Inc. All rights reserved. (...) Hemodynamic Characteristics and Predictors of Pulmonary Hypertension in Patients With Sickle Cell Disease. Pulmonary hypertension is a common co-morbidity of sickle cell disease with an associated increased mortality risk, but its etiology is not well-understood. To evaluate the hemodynamic characteristics, clinical predictors, and cardiovascular manifestations of elevated pulmonary arterial pressure in this population, we performed noninvasive hemodynamic assessments of 135 patients

2012 American Journal of Cardiology

51. Mesenchymal Stromal Cells for Haplo Hematopoietic Cell Transplantation for Sickle Cell Disease

lasting > 24 hours; History of ≥2 episodes of acute chest syndrome (ACS) in the 2-year period preceding enrollment despite the institution of supportive care measures (i.e. asthma therapy and/or hydroxyurea); History of ≥3 severe pain crises per year in the 2-year period preceding enrollment despite the institution of supportive care measures (i.e. a pain management plan and/or treatment with hydroxyurea); Administration of regular red blood cell (RBC) transfusion therapy, defined as receiving ≥8 (...) , and/or pulmonary hypertension (PH). A diagnosis of pulmonary hypertension (PH) will be made by finding of mean pulmonary artery pressure (mPAP) <25 mm Hg on right heart catheterization. In patients unable and/or unwilling to undergo cardiac catheterization, patients will be excluded with the following constellation of findings based upon presumptive diagnosis of PH (PPV of 62%): TRJ velocity >2.5 m/sec AND either N-terminal pro-brain natriuretic peptide (NT-pro-BNP) ≥160 pg/ml OR 6-minute walk distance <333 m

2017 Clinical Trials

52. Iron Deficiency and Anaemia in Adults

ratio BP blood pressure CD coeliac disease CKD chronic kidney disease COPD chronic obstructive pulmonary disease CRP C-reactive protein EPO endogenous erythropoietin ESA erythropoietin stimulating agents FBC full blood count FID functional iron deficiency GFR glomerular filtration rate GI gastrointestinal Hb haemoglobin HFrEF heart failure with reduced ejection fraction HFpEF heart failure with preserved ejection fraction HMB heavy menstrual bleeding HRC hypochromic red cells IBD inflammatory bowel (...) . In situations where there is anticipated blood loss, intraoperative cell salvage can be used to return salvaged red blood cells back to the patient (Thakrar et al., 2017). Post-operative care Following major surgery, up to 90% of patients may become anaemic and recent changes to transfusion thresholds have resulted in more patients being discharged with anaemia. Simple interventions, such as reducing the number and volume of blood samples and avoiding the use of postoperative drains, can have a significant

2018 Royal College of Nursing

53. Nonmyeloablative Haploidentical Peripheral Blood Mobilized Hematopoietic Precursor Cell Transplantation for Sickle Cell Disease

, and toxicity at a number of transplant centers. Our ongoing protocol for patients with severe congenital anemias, particularly sickle cell disease (SCD), and an HLA-matched sibling donor has had excellent preliminary results. None of the patients who engrafted had sickle-related events or any evidence of graft versus host disease (GVHD). There was no significant toxicity associated with the conditioning regimen. An additional protocol is ongoing for patients with high risk of graft rejection which employs (...) ) ( National Heart, Lung, and Blood Institute (NHLBI) ): Peripheral Blood Stem Cells Host-Donor Chimerism Graft Versus Host Disease Haploidentical Donor Apheresis Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Cyclophosphamide Sirolimus Pentostatin Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs Antirheumatic Agents

2017 Clinical Trials

54. Study of HLA-Haploidentical Stem Cell Transplantation to Treat Clinically Aggressive Sickle Cell Disease

Acute chest syndrome with recurrent hospitalizations, defined as ≥ 2 lifetime events 1.5 Red-cell alloimmunization (≥ 2 antibodies) during long-term transfusion therapy 1.6 Bilateral proliferative retinopathy with major visual impairment in at least one eye 1.7 Osteonecrosis of 2 or more joints 1.8 Sickle cell nephropathy, defined by a GFR < 90mL/min/1.73m2 or the presence of macroalbuminuria (urine albumin > 300 mg/g creatinine) 1.9 Pulmonary hypertension, defined by a mean pulmonary arterypressure (...) , Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Cyclophosphamide Fludarabine phosphate Sirolimus Everolimus Thymoglobulin Fludarabine Mycophenolic Acid Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs Antirheumatic Agents Antineoplastic Agents, Alkylating Alkylating Agents Molecular Mechanisms of Pharmacological Action Antineoplastic Agents Myeloablative Agonists Antimetabolites

2017 Clinical Trials

55. Nonmyeloablative Stem Cell Transplant in Children With Sickle Cell Disease and a Major ABO-Incompatible Matched Sibling Donor

of priapism in males history of osteonecrosis pulmonary hypertension as documented by tricuspid regurgitation jet velocity (TRV) > 2.5 m/s on echocardiogram red cell allo-immunization (≥ 2 antibodies) during long term transfusion therapy Sickle complications should be present despite the use of hydroxyurea, but this is not an absolute requirement, if the treating team considers the patient to be at high risk for further crisis episodes. Exclusion Criteria: Patients who are unable to comply with or follow (...) ) in pediatric patients with sickle cell disease (SCD) who have a matched related major ABO-incompatible donor. The nonmyeloablative regimen will use alemtuzumab, total body irradiation (TBI) and sirolimus for immune suppression. This study will expand the access of HSCT for patients with SCD who are currently not eligible because of donor restrictions. Condition or disease Intervention/treatment Phase Sickle Cell Disease Stem Cell Transplant Complications Red Blood Cell Disorder Pure Red Cell Aplasia Drug

2017 Clinical Trials

56. Haplo-Identical Transplantation for Severe Aplastic Anemia and Hypo-Plastic MDS Using Peripheral Blood Stem Cells and Post-Transplant Cyclophosphamide for GVHD Prophylaxis

Haplo-Identical Transplantation for Severe Aplastic Anemia and Hypo-Plastic MDS Using Peripheral Blood Stem Cells and Post-Transplant Cyclophosphamide for GVHD Prophylaxis Haplo-Identical Transplantation for Severe Aplastic Anemia and Hypo-Plastic MDS Using Peripheral Blood Stem Cells and Post-Transplant Cyclophosphamide for GVHD Prophylaxis - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search (...) for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Haplo-Identical Transplantation for Severe Aplastic Anemia and Hypo-Plastic MDS Using Peripheral Blood Stem Cells and Post-Transplant Cyclophosphamide for GVHD Prophylaxis The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has

2018 Clinical Trials

57. Imatinib and Carvedilol for High Blood Pressure in the Lungs in Adults With Sickle Cell Disease

Heart, Lung, and Blood Institute (NHLBI) Information provided by (Responsible Party): National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ) Study Details Study Description Go to Brief Summary: Background: - About one-tenth of adults with sickle cell disease have pulmonary hypertension (high blood pressure in the lungs). This condition can cause shortness of breath, pain crisis, and congestive heart failure. It may even lead to death. Researchers (...) associated with higher mortality in our cohort. These same hemodynamic profiles have previously been described and confirmed in other large cohorts as well. We propose treatment of sickle cell subjects with pulmonary arterial hypertension (PAH), defined hemodynamically by pulmonary arterial mean pressures (mPAP) of 25 mmHg or greater, with low estimated left ventricular filling pressures (low pulmonary capillary wedge pressures, or PCWP), and high pulmonary vascular resistance (PVR), with imatinib

2012 Clinical Trials

58. Chronic Pulmonary Complications of Sickle Cell Disease. Full Text available with Trip Pro

Chronic Pulmonary Complications of Sickle Cell Disease. Sickle cell disease (SCD), the most common genetic hemolytic anemia worldwide, affects 250,000 births annually. In the United States, SCD affects approximately 100,000 individuals, most of African descent. Hemoglobin S (HbS) results from a glutamate-to-valine mutation of the sixth codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is associated with the most prevalent and severe form of the disease. Other SCD genotypes (...) of these conditions has improved much over the past 10 to 15 years, there remains no specific treatment for pulmonary complications of SCD. It is unclear whether conventional treatment regimens directed at non-SCD populations have equivalent efficacy in patients with SCD. This represents a critical research need. In this review, the authors review the state-of-the-art understanding of the following pulmonary complications of SCD: (1) pulmonary hypertension; (2) venous thromboembolic disease; (3) sleep-disordered

2016 Chest

59. Placenta growth factor mediated gene regulation in sickle cell disease. Full Text available with Trip Pro

Placenta growth factor mediated gene regulation in sickle cell disease. Sickle cell anemia (SCA) is an autosomal recessive disorder caused by mutation in the β-globin gene. Pulmonary hypertension (PH), a complication of SCA, results in severe morbidity and mortality. PH is a multifactorial disease: systemic vasculopathy, pulmonary vasoconstriction, and endothelial dysfunction and remodeling. Placenta growth factor (PlGF), an angiogenic growth factor, elaborated from erythroid cells, has been (...) shown to contribute to inflammation, pulmonary vasoconstriction and airway hyper-responsiveness (AH) in mouse models of sickle cell disease. In this review, we summarize the cell-signaling mechanism(s) by which PlGF regulates the expression of genes involved in inflammation, PH and AH in cell culture and corroborate these findings in mouse models of SCA and in individuals with SCA. The role of microRNAs (miRNAs) in the post-transcriptional regulation of these genes is presented and how these miRNAs

2017 Blood reviews

60. Intravascular hemolysis and the pathophysiology of sickle cell disease Full Text available with Trip Pro

Intravascular hemolysis and the pathophysiology of sickle cell disease Hemolysis is a fundamental feature of sickle cell anemia that contributes to its pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1, asymmetric dimethylarginine, and adenine nucleotides are all products of hemolysis that promote vasomotor dysfunction, proliferative vasculopathy, and a multitude of clinical complications of pulmonary and systemic vasculopathy, including pulmonary (...) sickle vaso-occlusion and acute lung injury in murine models of sickle cell disease. Intravascular hemolysis can impair NO bioavailability and cause oxidative stress, altering redox balance and amplifying physiological processes that govern blood flow, hemostasis, inflammation, and angiogenesis. These pathological responses promote regional vasoconstriction and subsequent blood vessel remodeling. Thus, intravascular hemolysis represents an intrinsic mechanism for human vascular disease that manifests

2017 The Journal of clinical investigation

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