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Pulmonary Hypertension in Sickle Cell Anemia

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461. 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

462. 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

463. Evaluation of the Prevalence of Pulmonary Hypertension in Adult Patients With Sickle Cell Disease

, 2012 Last Verified: November 2012 Keywords provided by Assistance Publique - Hôpitaux de Paris: Doppler Echocardiography Pulmonary hypertension Sickle cell disease 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 (...) Evaluation of the Prevalence of Pulmonary Hypertension in Adult Patients With Sickle Cell Disease Evaluation of the Prevalence of Pulmonary Hypertension in Adult Patients 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). Please remove

2007 Clinical Trials

464. Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. (Full text)

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

2007 American Journal of Respiratory and Critical Care Medicine

465. Evaluation of Hydroxyurea Plus L-arginine or Sildenafil to Treat Sickle Cell Anemia

Iongitudinally. Once a maximal Hb-F raising effect of hydroxyurea has been established, oral L-arginine (the substrate for NO synthase) and sildenafil (Viagra, a phosphodiesterase inhibitor that potentates cGMP dependent signaling) will be added to determine the ability of other agents to enhance HbF synthesis, especially in hydroxyurea non-responders or partial-responders. Additionally, we have found that up to 33% of patients with sickle cell disease also have secondary pulmonary hypertension, measured (...) , priapism, aseptic necrosis of the hip, and/or pulmonary hypertension. Patients must be able to provide informed consent. EXCLUSION CRITERIA: Patients who have hemoglobin S and A (trait) or hemoglobin A-only (non-sickle cell). Patients must not be on a chronic transfusion program, defined as regular transfusions every 2-8 weeks. Patients must not be pregnant or breast feeding. Patients on chronic nitrates, such as nitroglycerin. Contacts and Locations Go to Information from the National Library

2003 Clinical Trials

466. Right ventricular abnormalities in sickle cell anemia: evidence of a progressive increase in pulmonary vascular resistance. (PubMed)

Right ventricular abnormalities in sickle cell anemia: evidence of a progressive increase in pulmonary vascular resistance. To assess the effects of sickle cell anemia (SCA) on the right ventricle (RV).Echocardiograms of 32 children with SCA were compared with age-matched healthy controls. RV measurements included diastolic area index, fractional area change, free-wall mass index, ejection time corrected for heart rate (ET(c)), and tricuspid regurgitation (TR) gradient.SCA subjects had elevated (...) = .42; P < .05). TR gradients > 2.5 m/sec, consistent with pulmonary hypertension, were found in 5 (16%) of SCA subjects, all older than 9 years.RV preload and systolic function do not worsen during childhood in SCA; however, RV mass index and the prevalence of pulmonary hypertension increase consistent with rising pulmonary vascular resistance.

2006 Journal of Pediatrics

467. Nitric Oxide and Transfusion Therapy for Sickle Cell Patients With Pulmonary Hypertension

, and Blood Institute (NHLBI) Collaborator: Mallinckrodt 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: This study will test whether inhaling nitric oxide (NO) gas mixed with room air can improve pulmonary hypertension (high blood pressure in the lungs) in patients with sickle cell anemia. Patients with sickle cell disease 18 years of age or older may (...) months. Patients have an echocardiogram and blood tests every week and do a 6-minute walk test every 2 weeks. Condition or disease Intervention/treatment Phase Sickle Cell Anemia Pulmonary Hypertension Drug: Nitric Oxide Device: INO Pulse - NO Delivery System Phase 1 Detailed Description: Sickle cell anemia is an autosomal recessive disorder and the most common genetic disease affecting African-Americans. Approximately 0.15% of African-Americans are homozygous for sickle cell disease, and 8% have

2001 Clinical Trials

468. Diastolic dysfunction and pulmonary hypertension in sickle cell anemia: is there a role for L-carnitine treatment? (PubMed)

Diastolic dysfunction and pulmonary hypertension in sickle cell anemia: is there a role for L-carnitine treatment? Clinical manifestations of cardiovascular abnormalities in patients with sickle cell (SC) anemia are well documented. Many variables were assessed in our study before and after administration of L-carnitine to randomly selected 37 SC disease (SCD) children for a period of 6 months. Variables such as weight, height, serum ferritin levels, units of blood transfused and the number (...) of veno-occlusive crises all showed significant improvement after the 6 months of therapy with L-carnitine. Our study also showed that cardiac diastolic function and pulmonary hypertension are common in pediatric SCD patients. These two disorders showed some improvement after L-carnitine administration. Therefore, L-carnitine deserves a rigorous large-scale randomized clinical trial to evaluate its potential benefits as treatment for SCD patients with cardiac complications.Copyright 2006 S. Karger AG

2006 Acta haematologica

469. Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia

Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia - 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. Secondary Pulmonary (...) of Diabetes and Digestive and Kidney Diseases (NIDDK) 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: The purpose of this study is to determine how often people with sickle cell anemia develop pulmonary hypertension a serious disease in which blood pressure in the artery to the lungs is elevated. Men and women 18 years of age and older with sickle cell

2001 Clinical Trials

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