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Pulmonary Hypertension in Sickle Cell Anemia

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441. Safety Study of Gleevec® in Children With Pulmonary Hypertension

hypertension with pulmonary capillary wedge pressure > 12 mmHg) detected at right heart catheterization. Chronic thromboembolic pulmonary hypertension, congenital or acquired deficiencies of blood coagulation, deficient thrombocyte function, thrombocytopenia < 40,000/μl, or Sickle Cell anemia. Pregnancy, breast feeding, or lack of safe contraception (hormonal contraception, IUD, bilateral tubal ligation, hysterectomy) in premenopausal women. Hepatic insufficiency with transaminase levels >4-fold the upper (...) Safety Study of Gleevec® in Children With Pulmonary Hypertension Safety Study of Gleevec® in Children With Pulmonary Hypertension - 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. Safety Study of Gleevec®

2007 Clinical Trials

442. A Study of Aspirin and Simvastatin in Pulmonary Arterial Hypertension

or coordination Negative pregnancy test at screening visit for women of childbearing potential If female, willing to use adequate form of birth control Exclusion Criteria: PAH related to other etiologies Diagnosis of sickle cell disease Clinically significant untreated sleep apnea, as diagnosed by polysomnography Left-sided valvular disease (more than moderate mitral valve stenosis or insufficiency or aortic stenosis or insufficiency), pulmonary artery or valve stenosis, or ejection fraction less than 45 (...) A Study of Aspirin and Simvastatin in Pulmonary Arterial Hypertension A Study of Aspirin and Simvastatin in Pulmonary Arterial Hypertension - 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. A Study of Aspirin

2006 Clinical Trials

443. Cardiopulmonary Function Assessment and NO-Based Therapies for Patients With Hemolysis-Associated Pulmonary Hypertension

Posted : July 14, 2006 Last Update Posted : April 5, 2019 Sponsor: National Heart, Lung, and Blood Institute (NHLBI) Information provided by: National Institutes of Health Clinical Center (CC) Study Details Study Description Go to Brief Summary: This study will evaluate new treatments for people who have pulmonary hypertension, or high blood pressure in the lungs, caused by sickle cell anemia or thalassemia. Patients ages 18 and older with a diagnosis of sickle cell disease or thalassemia, who have (...) pressure-and to make sure that the blood pressure does not increase when medication is stopped. Patients whose symptoms have improved as a result of breathing NO may wish to continue with that therapy. Condition or disease Intervention/treatment Phase Pulmonary Hypertension Drug: Nitric Oxide/INP Pulse Delivery Drug: Nitric Oxide/INO Pulse Delivery Phase 1 Detailed Description: Sickle cell anemia is an autosomal recessive disorder and the most common genetic disease affecting African-Americans

2006 Clinical Trials

444. Therapeutic Application of Intravascular Nitrite for Sickle Cell Disease

as an oxygen- and pH dependent nitrite reductase. To date we have observed that nitrite infusions in animal models significantly reduce liver and cardiac ischemia-reperfusion injury and infarction in mouse models, prevent cerebral vasospasm after subarachnoid hemorrhage in primates, and decrease pulmonary hypertension in newborn hypoxic sheep. The current protocol is designed as a phase I/II trial to address the hypothesis that nitrite infusions will vasodilate the circulation in patients with sickle cell (...) that the patients breathe out. Then the procedure for administering normal saline, sodium nitroprusside, and L-NMMA will be repeated, as will a blood test. This study will not have a direct benefit for participants. However, it is hoped that the information gained from the study will help to develop treatment options for patients with sickle cell disease. Condition or disease Intervention/treatment Phase Sickle Cell Anemia Drug: L-NMMA Drug: Sodium Nitrite Phase 1 Detailed Description: Sickle cell disease

2004 Clinical Trials

445. Natural History of Sickle Cell Disease and Other Hemolytic Disorders

) ): Hemoglobin Acute Chest Syndrome Treatment Options Nitric Oxide Pulmonary Hypertension Sickle Cell Disease Sickle Cell Trait Red Blood Cell Disorder Thalassemia Paroxysmal Nocturnal Hemoglobinuria Hemolytic Disorders Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn (...) Natural History of Sickle Cell Disease and Other Hemolytic Disorders Natural History 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. Natural History of Sickle Cell Disease The safety

2004 Clinical Trials

446. Niacin to Improve Blood Flow in People With Sickle Cell Disease

, due in large part to scavenging of nitric oxide by cell-free hemoglobin. We recently have completed studies that directly demonstrate endothelial dysfunction in patients with sickle cell disease, characterized by decreased ACh dependent vasorelaxation in forearm blood flow studies, distinct from the nitric oxide resistance above. Further, we have found in sickle cell patients a new association between low levels of apoA-I, pulmonary hypertension and endothelial dysfunction. Raising levels of HDL (...) ) ): Acetylcholine Forearm Blood Flow HDL L-NMMA Sickle Cell Anemia 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 Niacin Acetylcholine Niacinamide Nicotinic Acids omega-N-Methylarginine Hypolipidemic Agents Antimetabolites Molecular Mechanisms of Pharmacological Action Lipid Regulating Agents Vasodilator Agents Vitamin B Complex Vitamins

2007 Clinical Trials

447. Evaluation of the Lung Capillary Blood Volume in Children With Sickle Cell Disease

- Hôpitaux de Paris Information provided by (Responsible Party): Assistance Publique - Hôpitaux de Paris Study Details Study Description Go to Brief Summary: Sickle cell disease (SCD) is the most common inherited disease of the world affecting African and Caribbean populations. SCD is caused by the homozygous inheritance of the gene for sickle hemoglobin (HbS). Most patients with SCD develop abnormal pulmonary function characterized by airway obstruction, restrictive lung disease, abnormal diffusing (...) circulation in these two populations of children. Combined with complete lung function measurements, echocardiographic assessment of pulmonary hemodynamics, and measurement of exhaled nitric oxide, these evaluations will lead to a better understanding of pathophysiology of lung injury in SCD. The study will be completes at Robert Debré Hospital, in close collaboration with Sickle Cell Disease Center and Physiology Department. Children will be included after informed consent signed, as legally prescribed

2007 Clinical Trials

448. A Phase 2 Study of the Effects of 6R-BH4 in Subjects With Sickle Cell Disease

for at least 2 years, or had a tubal ligation at least 1 year prior to Screening, or who have had a total hysterectomy. Exclusion Criteria: Requires chronic hypertransfusion therapy. Sickle cell crisis during the 30 days prior to Screening. Myocardial infarction, cerebral vascular accident, or pulmonary embolism during the 6 months prior to Screening. History of bone marrow or hematopoietic stem cell transplantation. Hepatic dysfunction (alanine aminotransferase [ALT][SGPT] > 2 times the upper limit (...) : Sickle Cell Disease SCD 6R-BH4 BH4 sapropterin dihydrochloride endothelial dysfunction Nitric Oxide NO Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Verapamil Anti-Arrhythmia Agents Calcium Channel Blockers Membrane Transport Modulators Molecular Mechanisms of Pharmacological Action Calcium-Regulating Hormones and Agents Physiological Effects

2007 Clinical Trials

449. Sildenafil to Increase Exercise Capacity in Individuals With Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension

or greater than 300 mg/dl) Severe serum sodium abnormalities (serum sodium less than 130 mEq/L or greater than 150 mEq/L) Condition that may predispose participant to priapism (e.g., sickle cell anemia, multiple myeloma, leukemia) Retinitis pigmentosa Known or suspected idiopathic hypertrophic subaortic stenosis (IHSS) Low blood pressure (systolic blood pressure [SBP] less than 100 mm Hg or diastolic blood pressure [DBP] less than 50 mm Hg) Uncontrolled systemic high blood pressure (SBP greater than 180 (...) Sildenafil to Increase Exercise Capacity in Individuals With Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension Sildenafil to Increase Exercise Capacity in Individuals With Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension - 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

2006 Clinical Trials

450. A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension

Fraction < 22% by echocardiography or Symptomatic coronary disease (demonstrable ischemia). Have any other disease that is associated with pulmonary hypertension (e.g. congenital systemic to pulmonary shunt, sickle cell anemia, schistosomiasis). Have a musculoskeletal disorder (e.g. arthritis, artificial leg, etc.) or any other disease, which is thought to limit ambulation, or be connected to a machine, which is not portable. Have uncontrolled systemic hypertension as evidenced by systolic blood (...) A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension - 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

2003 Clinical Trials

451. Phase III Randomized Study of UT-15 in Patients With Primary Pulmonary Hypertension

greater than 15 mm Hg OR LVEF less than 40% by MUGA or angiography OR LV shortening fraction less than 22% by echocardiography OR Symptomatic coronary disease (demonstrable ischemia) Other: Not pregnant or nursing Negative pregnancy test Fertile patients must use effective contraception Mentally and physically capable of using an infusion pump HIV negative No other disease associated with pulmonary hypertension (sickle cell anemia, schistosomiasis) No musculoskeletal disorder (arthritis, artificial (...) Phase III Randomized Study of UT-15 in Patients With Primary Pulmonary Hypertension Phase III Randomized Study of UT-15 in Patients With Primary Pulmonary Hypertension - 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

1999 Clinical Trials

452. Endothelial Cell Dysfunction in Pulmonary Hypertension

histopathology similar to the primary form or PAH. Clinical conditions causing pulmonary hypertension with histopathology similar to the primary form are listed below. i. Eisenmenger Syndrome ii. Collagen vascular disease iii. Liver disease with portal hypertension iv. Toxin induced injury (anorexic agents, rapeseed oil) v. HIV disease vi. Sickle cell disease EXCLUSION CRITERIA FOR PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION: Pregnant women (all women of childbearing age will be required to have (...) Endothelial Cell Dysfunction in Pulmonary Hypertension Endothelial Cell Dysfunction in Pulmonary Hypertension - 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. Endothelial Cell Dysfunction in Pulmonary

2004 Clinical Trials

453. Hydroxyurea and Erythropoietin to Treat Sickle Cell Anemia

the two drugs can lower blood pressure in the lungs. Patients 18 years of age and older with sickle cell anemia and kidney disease or pulmonary hypertension, or both, may be eligible for this study. Candidates are screened with a medical history, physical examination, blood tests, a 6-minute walk test (test to see how far the subject can walk in 6 minutes), and echocardiogram (ultrasound of the heart to measure blood pressure in the lungs). Participants undergo the following tests and procedures (...) Center (CC): Erythropoietin Therapy Fetal Hemoglobin Induction Sickle Cell Disease Pulmonary Hypertension Chronic Kidney Disease SCD Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Kidney Diseases Renal Insufficiency, Chronic Hypertension, Pulmonary Anemia, Sickle Cell Vascular Diseases Cardiovascular Diseases Urologic Diseases Renal Insufficiency Lung Diseases Respiratory Tract Diseases Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases

2005 Clinical Trials

454. Heart Disease in Sickle Cell Anemia

be injected to enhance the quality of the images. Invasive electrocardiographic (reveal) monitoring: This procedure permits study of the heart rhythms over a long time period. A small device is placed just under the skin on the left side of the chest. It can be left in for up to 14 months to monitor the heartbeat continuously during this time. Condition or disease Sickle Cell Anemia Pulmonary Hypertension Detailed Description: Sickle cell anemia is an inherited blood disorder primarily affecting groups (...) with PAH and if they contribute to SD. Improved understanding of the etiology and mechanisms of SD in SCA may allow the development and testing of therapies for the primary prevention of SD. Study Design Go to Layout table for study information Study Type : Observational Enrollment : 120 participants Official Title: Are Left Ventricular Abnormalities Associated With Pulmonary Hypertension in Sickle Cell Anemia? Study Start Date : June 2, 2005 Study Completion Date : October 2, 2007 Resource links

2005 Clinical Trials

455. Endothelial Function in Patients With Sickle Cell Anemia Before and After Sildenafil

: placebo viagra versus placebo will be given to patients with sickle cell anemia Drug: placebo Outcome Measures Go to Primary Outcome Measures : endothelial function before and after viagra treatment to patients with sickle cell anemia without pulmonary hypertension [ Time Frame: January 2009 till January 2011 ] Eligibility Criteria Go to Information from the National Library of Medicine Choosing to participate in a study is an important personal decision. Talk with your doctor and family members (...) Padeh Medical Center, Poriya Information provided by: The Baruch Padeh Medical Center, Poriya Study Details Study Description Go to Brief Summary: The investigators would like to study the endothelial function in sickle cell patients without pulmonary hypertension in an in vivo method during a steady state condition before and after sildenafil treatment for 1 month, and to study the effects of this nitric oxide donor by measuring the Flow Mediated Dilatation, by measuring endothelial progenitor stem

2008 Clinical Trials

456. Fludarabine, Cyclophosphamide, and Total-Body Irradiation Followed By Donor Bone Marrow Transplant in Treating Patients With Sickle Cell Anemia and Other Blood Disorders

Fludarabine, Cyclophosphamide, and Total-Body Irradiation Followed By Donor Bone Marrow Transplant in Treating Patients With Sickle Cell Anemia and Other Blood Disorders Non-Myeloablative Bone Marrow Transplant for Patients With Sickle Cell Anemia and Other Blood Disorders - 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. Non-Myeloablative Bone Marrow Transplant for Patients With Sickle Cell Anemia and Other Blood Disorders 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 been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT00489281 Recruitment Status

2007 Clinical Trials

457. Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. (PubMed)

Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. Although pulmonary hypertension (PH) is a common complication of sickle cell disease (SCD) associated with high mortality, there exist few data characterizing hemodynamics and cardiopulmonary function in this population.To characterize hemodynamics and cardiopulmonary function in patients with SCD with and without PH.Patients with SCD with PH (n = 26) were compared with control subjects (...) with SCD but without PH (n = 17), matched for age, hemoglobin levels, and fetal hemoglobin levels.Upon catheterization, 54% of the patients with PH had pulmonary arterial hypertension, and 46% had pulmonary venous hypertension. When compared with control subjects, patients with PH exhibited lower six-minute-walk distance (435 +/- 31 vs. 320 +/- 20 m, p = 0.002) and oxygen consumption (50 +/- 3% vs. 41 +/- 2% of predicted, p = 0.02), and also had mild restrictive lung disease and more perfusion

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2007 American Journal of Respiratory and Critical Care Medicine

458. Efficacy and Safety of Bosentan in Sickle Cell Disease (SCD) Patients Diagnosed With Pulmonary Hypertension (PH)

indexed to this study by ClinicalTrials.gov Identifier (NCT Number): Layout table for additonal information Responsible Party: Sebastien Roux, MD, Actelion ClinicalTrials.gov Identifier: Obsolete Identifiers: NCT00307372 Other Study ID Numbers: AC-052-369 ASSET-2 First Posted: April 12, 2006 Last Update Posted: January 16, 2012 Last Verified: January 2012 Keywords provided by Actelion: Pulmonary Hypertension Sickle Cell Disease Sickle cell anemia bosentan ASSET ASSET-2 Additional relevant MeSH terms (...) : Layout table for MeSH terms Hypertension Hypertension, Pulmonary Anemia, Sickle Cell Vascular Diseases Cardiovascular Diseases Lung Diseases Respiratory Tract Diseases Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Bosentan Antihypertensive Agents Endothelin Receptor Antagonists Molecular Mechanisms of Pharmacological Action

2006 Clinical Trials

459. Pulmonary Hypertension, Hypoxia and Sickle Cell Disease

Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ) Study Details Study Description Go to Brief Summary: The study will look at the risk factors for pulmonary hypertension (high blood pressure in the lungs) in children and adolescents with sickle cell anemia (SCA) and examine the role of hypoxia (oxygen shortage) in the disease. In patients with SCA, red blood cells become sickle-shaped and tend to form clumps that get stuck in blood vessels, blocking blood flow to the limbs (...) function). Transcranial doppler (brain ultrasound study to measure brain blood flow). Lung function tests. 6-minute walk (measure of the distance covered in 6 minutes of walking). In addition, patients are followed by telephone or by clinic visits every 6 months for a review of their medical history and medications. A physical examination is also done at 12 months. Condition or disease Chuvash Polycythemia Cerebrovascular Disease Pulmonary Hypertension Sickle Cell Anemia Detailed Description

2007 Clinical Trials

460. Sildenafil Therapy for Pulmonary Hypertension and Sickle Cell Disease

complications of sickle cell anemia. Pulmonary hypertension (PH) has now been identified as a marker of mortality in adults with sickle cell disease. Sildenafil has been proven beneficial in pulmonary hypertension (PH) and recent phase I/II studies from the intramural National Institutes of Health (NIH) suggest it is well tolerated and efficacious in the SCD population. Furthermore, a number of recent studies have suggested that nitric oxide (NO) based therapies may have a favorable impact on sickle red (...) by Mark Gladwin, National Heart, Lung, and Blood Institute (NHLBI): Interventional Study 6-Minute Walk Sickle Cell Anemia Sildenafil/Viagra Tricuspid Regurgitant Velocity Sickle Cell Disease Pulmonary Hypertension Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Hypertension, Pulmonary Anemia, Sickle Cell Vascular Diseases Cardiovascular Diseases Lung Diseases Respiratory Tract Diseases Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases

2007 Clinical Trials

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