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Pulmonary Hypertension in Sickle Cell Anemia

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61. Anemia, Sickle Cell (Diagnosis)

) to identify these conditions in young patients. [ ] In the study, 17 patients (43.6%) had ambulatory hypertension, whereas 4 (10.3%) had hypertension on the basis of their clinic blood pressure. Twenty-three patients (59%) had impaired systolic blood pressure dipping, 7 (18%) had impaired diastolic blood pressure dipping, and 5 (13%) had reversed dipping. [ ] Imaging studies Imaging studies that aid in the diagnosis of sickle cell anemia in patients in whom the disease is suggested clinically include (...) In the United States, approximately 100,000 people have SCD SCD occurs in about 1 of every 16,300 Hispanic-American births Approximately 1 in 13 black or African Americans has sickle cell trait In the United States, SCD accounts for less than 1% of all new cases of end-stage renal disease (ESRD). [ ] The following factors are known to portend a greater likelihood of progression to overt renal failure: hypertension, nephrotic-range proteinuria, hematuria, severe anemia, and a Central African Republic

2014 eMedicine Emergency Medicine

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

Diseases Hypertension, Pulmonary Anemia, Sickle Cell Vascular Diseases Cardiovascular Diseases Respiratory Tract Diseases Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Albuterol Bronchodilator Agents Autonomic Agents Peripheral Nervous System Agents Physiological Effects of Drugs Anti-Asthmatic Agents Respiratory System Agents Tocolytic Agents Reproductive Control Agents Adrenergic beta-2 Receptor Agonists Adrenergic beta (...) Quantifying the Presence of Lung Disease and Pulmonary Hypertension in Children With Sickle Cell Disease Quantifying the Presence of Lung Disease and Pulmonary Hypertension in Children With Sickle Cell Disease - 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

2013 Clinical Trials

63. Nonmyeloablative Conditioning for Mismatched Hematopoietic Stem Cell Transplantation for Severe Sickle Cell Disease

for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Cyclophosphamide Fludarabine phosphate Sirolimus Everolimus Fludarabine Mycophenolic Acid Alemtuzumab Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs Antirheumatic Agents Antineoplastic Agents, Alkylating Alkylating Agents Molecular Mechanisms of Pharmacological Action Antineoplastic Agents Myeloablative Agonists (...) Nonmyeloablative Conditioning for Mismatched Hematopoietic Stem Cell Transplantation for Severe Sickle Cell Disease Nonmyeloablative Conditioning for Mismatched Hematopoietic Stem Cell Transplantation for Severe Sickle Cell Disease - 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

2016 Clinical Trials

64. Hemodynamic Characteristics and Predictors of Pulmonary Hypertension in Patients With Sickle Cell Disease. (PubMed)

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

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2012 American Journal of Cardiology

65. Bone Marrow Transplantation vs Standard of Care in Patients With Severe Sickle Cell Disease (BMT CTN 1503)

to their disease at a rate much higher than the general population. Therefore, the goal of the study is to establish that the difference in the proportion of patients surviving is not significantly more than 15% lower in the donor arm at 2-years after assignment to treatment arm. Secondary endpoints will compare changes in sickle cell disease related events (pulmonary hypertension, cerebrovascular events, renal function, avascular necrosis, leg ulcer) and functional outcomes [6-minute walk distance (6MWD (...) Leukocyte Antigen (HLA) Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Methotrexate Fludarabine phosphate Tacrolimus Busulfan Antilymphocyte Serum Thymoglobulin Fludarabine Abortifacient Agents, Nonsteroidal Abortifacient Agents Reproductive Control Agents Physiological Effects of Drugs Antimetabolites, Antineoplastic Antimetabolites Molecular

2016 Clinical Trials

66. Hydroxyurea in the Treatment of Sickle Cell Disease

Hydroxyurea 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 of Pharmacological Action Nucleic Acid Synthesis Inhibitors (...) Hydroxyurea in the Treatment of Sickle Cell Disease Hydroxyurea in the Treatment of Sickle Cell Disease - 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. Hydroxyurea in the Treatment of Sickle Cell Disease

2016 Clinical Trials

67. Macrolide Therapy to Improve Forced Expiratory Volume in 1 Second in Adults With Sickle Cell Disease

insufficient resources to initiate the trial.) First Posted : November 9, 2016 Last Update Posted : October 2, 2018 Sponsor: Vanderbilt University Information provided by (Responsible Party): Michael DeBaun, Vanderbilt University Study Details Study Description Go to Brief Summary: Sickle cell anemia (SCA) is a life-threatening, monogenic disorder associated with early death when compared to individuals without SCA. Pulmonary complications, namely acute chest syndrome, obstructive lung disease (...) Participant Data (IPD) Sharing Statement: Plan to Share IPD: Yes Keywords provided by Michael DeBaun, Vanderbilt University: sickle cell disease pulmonary complications reduced FEV1 azithromycin lung function Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn

2016 Clinical Trials

68. Oxygen Therapy and Pregnancy in Sickle Cell Disease

-regulated Drug Product: No Studies a U.S. FDA-regulated Device Product: No Keywords provided by Assistance Publique - Hôpitaux de Paris: sickle cell disease oxygen therapy pregnancy pregnancy in sickle cell disease Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn (...) Oxygen Therapy and Pregnancy in Sickle Cell Disease Oxygen Therapy and Pregnancy in Sickle Cell Disease - 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. Oxygen Therapy and Pregnancy in Sickle Cell Disease

2016 Clinical Trials

69. Transplantation Using Reduced Intensity Approach for Patients With Sickle Cell Disease From Mismatched Family Donors of Bone Marrow

Verified: May 2018 Keywords provided by Lakshmanan Krishnamurti, Emory University: bone marrow transplant 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 Fludarabine phosphate Thiotepa Antilymphocyte Serum Thymoglobulin Fludarabine Hydroxyurea Vidarabine Immunosuppressive Agents Immunologic Factors Physiological Effects of Drugs (...) Transplantation Using Reduced Intensity Approach for Patients With Sickle Cell Disease From Mismatched Family Donors of Bone Marrow Transplantation Using Reduced Intensity Approach for Patients With Sickle Cell Disease From Mismatched Family Donors of Bone Marrow - 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

2016 Clinical Trials

70. Increased Red Blood Cell Stiffness Increases Pulmonary Vascular Resistance and Pulmonary Arterial Pressure (PubMed)

Increased Red Blood Cell Stiffness Increases Pulmonary Vascular Resistance and Pulmonary Arterial Pressure Patients with sickle cell anemia (SCD) and pulmonary hypertension (PH) have a significantly increased risk of sudden death compared to patients with SCD alone. Sickled red blood cells (RBCs) are stiffer, more dense, more frequently undergo hemolysis, and have a sixfold shorter lifespan compared to normal RBCs. Here, we sought to investigate the impact of increased RBC stiffness (...) , independent of other SCD-related biological and mechanical RBC abnormalities, on the hemodynamic changes that ultimately cause PH and increase mortality in SCD. To do so, pulmonary vascular impedance (PVZ) measures were recorded in control C57BL6 mice before and after ∼50 μl of blood (Hct = 45%) was extracted and replaced with an equal volume of blood containing either untreated RBCs or RBCs chemically stiffened with glutaraldehyde (Hct = 45%). Chemically stiffened RBCs increased mean pulmonary artery

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2016 Journal of biomechanical engineering

71. Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders. (PubMed)

Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders. Pulmonary hypertension (PH) has emerged as a major complication of several hematologic disorders, including hemoglobinopathies, red cell membrane disorders, chronic myeloproliferative disorders, and splenectomy. With the exception of sickle cell disease, there are a limited number of studies systematically evaluating the prevalence of PH using the gold standard right heart catheterization

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2013 Clinics in Chest Medicine

72. Tissue Doppler Echocardiographic Findings of Left Ventricle in Children with Sickle-Cell Anemia (PubMed)

Tissue Doppler Echocardiographic Findings of Left Ventricle in Children with Sickle-Cell Anemia Sickle-cell disease (SCD) is an inherited hemoglobin childhood disorder, frequently complicated by pulmonary hypertension and cardiac involvement. Cardiovascular events and complications are the leading cause of mortality and morbidity in patients with SCD. Tissue Doppler imaging and the myocardial performance index (Tei index), are simple indices for the assessment of the cardiac function

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2012 The Journal of Tehran University Heart Center

73. Oxidized Mutant Human Hemoglobins S and E Induce Oxidative Stress and Bioenergetic Dysfunction in Human Pulmonary Endothelial Cells (PubMed)

Oxidized Mutant Human Hemoglobins S and E Induce Oxidative Stress and Bioenergetic Dysfunction in Human Pulmonary Endothelial Cells Cell free hemoglobin (Hb), becomes oxidized in the circulation during hemolytic episodes in sickle cell disease (SCD) or thalassemia and may potentially cause major complications that are damaging to the vascular system. Hemolytic anemias are commonly associated with pulmonary hypertension (PH) and often result from dysfunction of lung endothelial cells. The aim (...) of this study was to determine the effect of different Hbs on cultured human lung endothelial function. Toward this goal, endothelial permeability, oxidative stress response parameters, glycolytic and mitochondrial bioenergetic functions were monitored in cultured human pulmonary arterial endothelial cells (HPAEC) following incubation with human adult Hb (HbA), and Hb isolated from patients with sickle cell Hb (HbS, βV6E) and HbE (βE26K) that commonly co-exist with β-thalassemia. These mutant Hbs are known

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2017 Frontiers in physiology

74. Treatment of Sickle Cell Anemia With Stem Cell Transplant

Treatment of Sickle Cell Anemia With Stem Cell Transplant Treatment of Sickle Cell Anemia With Stem Cell Transplant - 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. Treatment of Sickle Cell Anemia With Stem (...) efficacious and sufficiently gentle to apply to patients with sickle cell anemia and related disorders. It is proposed to characterize the efficacy and toxicity of this regimen in high risk patients with sickle cell anemia using criteria for patient selection that have been accepted in prior BMT trials in patients with sickle cell disease, specifically only the subset of patients whose prior clinical behavior indicates that they are at high risk for serious morbidity and early mortality. In addition

2011 Clinical Trials

75. A monocyte-TNF-endothelial activation axis in sickle transgenic mice: Therapeutic benefit from TNF blockade. (PubMed)

A monocyte-TNF-endothelial activation axis in sickle transgenic mice: Therapeutic benefit from TNF blockade. Elaboration of tumor necrosis factor (TNF) is a very early event in development of ischemia/reperfusion injury pathophysiology. Therefore, TNF may be a prominent mediator of endothelial cell and vascular wall dysfunction in sickle cell anemia, a hypothesis we addressed using NY1DD, S+SAntilles , and SS-BERK sickle transgenic mice. Transfusion experiments revealed participation (...) for an abnormal monocyte-TNF-endothelial activation axis in the sickle context. Its presence, plus the many benefits of etanercept observed here, argue that pilot testing of TNF blockade should be considered for human sickle cell anemia, a challenging but achievable translational research goal.© 2017 The Authors American Journal of Hematology Published by Wiley Periodicals, Inc.

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2017 American journal of hematology

76. Efficacy and Safety Study to Evaluate MT-6548 in Hemodialysis Subjects Not Receiving ESAs With Anemia Associated With Chronic Kidney Disease in Japan

≥ lower limit of normal during the screening period Exclusion Criteria: Anemia due to a main cause other than CKD: sickle cell disease, myelodysplastic syndrome, bone marrow fibrosis, hematologic malignancy, hemolytic anemia, thalassemia, or pure red cell aplasia Active bleeding or recent blood loss within 8 weeks prior to the screening period RBC transfusion within 8 weeks prior to the screening period Received testosterone enanthate or mepitiostane within 8 weeks prior to the screening period AST (...) Efficacy and Safety Study to Evaluate MT-6548 in Hemodialysis Subjects Not Receiving ESAs With Anemia Associated With Chronic Kidney Disease in Japan Efficacy and Safety Study to Evaluate MT-6548 in Hemodialysis Subjects Not Receiving ESAs With Anemia Associated With Chronic Kidney Disease in Japan - 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

2018 Clinical Trials

77. Efficacy and Safety Study to Evaluate MT-6548 in Hemodialysis Subjects Currently Receiving ESAs With Anemia Associated With Chronic Kidney Disease in Japan

due to a main cause other than CKD: sickle cell disease, myelodysplastic syndrome, bone marrow fibrosis, hematologic malignancy, hemolytic anemia, thalassemia, or pure red cell aplasia Active bleeding or recent blood loss within 8 weeks prior to the screening period RBC transfusion within 8 weeks prior to the screening period Received testosterone enanthate or mepitiostane within 8 weeks prior to the screening period AST, ALT, or total bilirubin >2.5 x upper limit of normal during the screening (...) Efficacy and Safety Study to Evaluate MT-6548 in Hemodialysis Subjects Currently Receiving ESAs With Anemia Associated With Chronic Kidney Disease in Japan Efficacy and Safety Study to Evaluate MT-6548 in Hemodialysis Subjects Currently Receiving ESAs With Anemia Associated With Chronic Kidney Disease in Japan - 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

2018 Clinical Trials

78. Recommendations on RBC Transfusion Support in Children With Hematologic and Oncologic Diagnoses From the Pediatric Critical Care Transfusion and Anemia Expertise Initiative. (PubMed)

transfusion over simple (nonexchange) transfusion if the child's condition is deteriorating (based on clinical judgment), otherwise a simple, nonexchange RBC transfusion is recommended. There is insufficient evidence to make recommendations on transfusion thresholds for patients with sickle cell disease prior to minor procedures, with acute stroke or with pulmonary hypertension. For patients with oncologic disease or undergoing hematopoietic stem cell transplant, a hemoglobin concentration of 7-8 g/dL (...) the Grading of Recommendations Assessment, Development, and Evaluation method.The hematologic/oncologic subgroup developed 14 recommendations (seven clinical, seven research); all achieved greater than 80% agreement. In patients with sickle cell disease, Transfusion and Anemia Expertise Initiative recommends: 1) RBC transfusion to achieve a target hemoglobin concentration of 10 g/dL rather than hemoglobin of less than 30% prior to surgical procedures requiring general anesthesia and 2) exchange

2018 Pediatric Critical Care Medicine

79. Markers of Severe Vaso-Occlusive Painful Episode Frequency in Children and Adolescents with Sickle Cell Anemia. (PubMed)

Markers of Severe Vaso-Occlusive Painful Episode Frequency in Children and Adolescents with Sickle Cell Anemia. To identify factors associated with frequent severe vaso-occlusive pain crises in a contemporary pediatric cohort of patients with sickle cell anemia (SCA) enrolled in a prospective study of pulmonary hypertension and the hypoxic response in sickle cell disease.Clinical and laboratory characteristics of children with SCA who had ≥3 severe pain crises requiring health care

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2011 Journal of Pediatrics

80. Tricuspid Regurgitant Jet Velocity as an Independent Marker for Mortality in Sickle Cell Anemia

that the mortality was related to pulmonary hypertension. It is the aim of this study to retrospectively evaluate patients who have had multiple echocardiograms and to determine if patients who had either a normalization of their TR jet velocity on a subsequent echo or had no evidence of pulmonary hypertension on right heart catheterization had a similar mortality rate to those with persistently elevated TR jet velocity. Condition or disease Intervention/treatment Sickle Cell Anemia Pulmonary Hypertension Other (...) provided below. For general information, Layout table for eligibility information Ages Eligible for Study: 18 Years and older (Adult, Older Adult) Sexes Eligible for Study: All Accepts Healthy Volunteers: No Sampling Method: Non-Probability Sample Study Population Patients records will be extracted from the complete medical record at OSUMC Criteria To limit bias in this study all patients that with sickle cell anemia and pulmonary hypertension with the pre-specified data required will be included

2011 Clinical Trials

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