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Pulmonary Hypertension in Sickle Cell Anemia

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41. Hydroxyurea in the Emergency Room to Lessen Pain in Sickle Cell Crisis

or disease Intervention/treatment Phase Anemia, Sickle Cell Anemia; Sickle-Cell, With Crisis Drug: Hydroxyurea Phase 2 Detailed Description: Sickle cell anemia (SCA) is a hereditary hemoglobinopathy; complications of the disease include, spleen enlargement, acute chest syndrome, pulmonary hypertension, stroke and cumulative damage to multiple organs, and painful vaso-occlusive crises (VOC). In Brazil, about 3,500 children are born each year with DF, and the number of individuals with sickle cell disease (...) Assignment Masking: None (Open Label) Primary Purpose: Treatment Official Title: Protocol for the Administration of Hydroxyurea During Painful Vaso-occlusive Crisis in Sickle Cell Anemia Study Start Date : November 2016 Estimated Primary Completion Date : April 2017 Estimated Study Completion Date : July 2017 Resource links provided by the National Library of Medicine related topics: related topics: available for: resources: Arms and Interventions Go to Arm Intervention/treatment No Intervention: Control

2017 Clinical Trials

42. Placenta growth factor mediated gene regulation in sickle cell disease. (PubMed)

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

43. Clinical 'pearls' of maternal critical care Part 2: sickle-cell disease in pregnancy. (PubMed)

Clinical 'pearls' of maternal critical care Part 2: sickle-cell disease in pregnancy. The current review outlines the challenges in managing pregnant women with sickle-cell anemia, who are at risk of becoming critically ill during pregnancy.Sickle obstetric patients pose unique challenges to the anesthetist and intensivist. We discuss the role of prophylactic transfusions for specific indications like acute anemia and twin pregnancies. The management and prevention of vaso-occlusive crises (...) and chest crisis are also outlined. The role of the multidisciplinary team cannot be overstated.Massive obstetric hemorrhage in this population is difficult, and unique considerations such as cell-saver technology and tranexamic acid usage are discussed. Secondary complications such as pulmonary hypertension and stroke are also considered, with a summary of the latest treatment guidelines.This is a challenging cohort of pregnant patients who have a significantly increased morbidity and mortality

2017 Current Opinion in Anaesthesiology

44. Heart Arteries and Sickle Cell Disease / Coeur Artères DREpanocytose

table for additional information Studies a U.S. FDA-regulated Drug Product: No Studies a U.S. FDA-regulated Device Product: No Keywords provided by Xavier Jouven, Cardiologie et Développement: Sub-Saharan Africa Sickle Cell Disease Vasculopathy Nephropathy Pulmonary hypertension cardiac disease hemolysis pulse wave velocity blood viscosity Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases (...) . In addition to repeated acute ischemic insults due to the red blood cells sickling in the microcirculation, a chronic vasculopathy leads to organ injuries, such as kidney disease, stroke, pulmonary hypertension, retinopathy, bone infarcts, and leg ulcers. CADRE is a multinational prospective observational study undertaken in five countries in sub-Saharan Africa. Patients with SCD will be recruited through outpatients' clinics in public, university and private hospitals and research centers in five

2017 Clinical Trials

45. Intravascular hemolysis and the pathophysiology of sickle cell disease (PubMed)

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 (...) clinical complications in sickle cell disease and other chronic hereditary or acquired hemolytic anemias.

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2017 The Journal of clinical investigation

46. Cardiac Structural and Functional Changes Evaluated by Transthoracic and Tissue Doppler Echocardiography in Adult Patients with Sickle Cell Disease (PubMed)

Cardiac Structural and Functional Changes Evaluated by Transthoracic and Tissue Doppler Echocardiography in Adult Patients with Sickle Cell Disease One of the most common genetic blood disorders, resulting from inherited red blood cell disorders, is sickle cell disease (SCD) which is responsible for high death in adult patients with left ventricular diastolic dysfunction and pulmonary hypertension. Tissue Doppler Echocardiography (TDE) and transthoracic echocardiography (TTE) are two useful (...) tools to assess the risk of SCD. The present study was conducted to evaluate the cardiac functions and structure using TDE and TTE among adult patients suffering from sickle cell anemia compare to normal samples.The current study was performed on 30 SCD patients with a mean age 18-40 years and healthy cases at Mazandaran Heart Center, Iran. The left and right ventricular functions were assessed using M-mode, two-dimensional (2D), and tricuspid regurgitation jet velocity (TRJV) data and TDE derived

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2017 Acta Informatica Medica

47. Cardiorespiratory pathogenesis of sickle cell disease in a mouse model (PubMed)

at 7-8 months of age, restrictive lung disease, pulmonary hypertension, cardiac enlargement, lower total lung capacity, and pulmonary vascular congestion. All adult SAD mice demonstrated impairments in exercise capacity and response to hypoxia, with a more severe phenotype in the tachypneic mice. The model revealed distinguishable subgroups of SAD mice with cardiorespiratory pathophysiology mimicking the complications of human sickle cell disease. (...) Cardiorespiratory pathogenesis of sickle cell disease in a mouse model The nature and development of cardiorespiratory impairments associated with sickle cell disease are poorly understood. Given that the mechanisms of these impairments cannot be addressed adequately in clinical studies, we characterized cardiorespiratory pathophysiology from birth to maturity in the sickle cell disease SAD mouse model. We identified two critical phases of respiratory dysfunction in SAD mice; the first prior

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2017 Scientific reports

48. Sickle Cell Anemia (Follow-up)

, and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor (...) non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . . Olujohungbe A, Howard J. The clinical care of adult patients

2014 eMedicine.com

49. Sickle Cell Anemia (Treatment)

, and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor (...) non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . . Olujohungbe A, Howard J. The clinical care of adult patients

2014 eMedicine.com

50. Sickle Cell Anemia (Overview)

) 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.com

51. Sickle Cell Anemia (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 Pediatrics

52. Sickle Cell Anemia (Overview)

) 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 Pediatrics

53. Sickle Cell Anemia (Treatment)

, and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor (...) non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . . Olujohungbe A, Howard J. The clinical care of adult patients

2014 eMedicine Pediatrics

54. Anemia, Sickle Cell (Overview)

) 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

55. Anemia, Sickle Cell (Follow-up)

, and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor (...) non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . . Olujohungbe A, Howard J. The clinical care of adult patients

2014 eMedicine Emergency Medicine

56. Sickle Cell Anemia (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.com

57. Sickle Cell Anemia, Skeletal

. 122(6):1332-42. . Linguraru MG, Orandi BJ, Van Uitert RL, Mukherjee N, Summers RM, Gladwin MT, et al. CT and image processing non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . Cerci SS, Suslu H, Cerci C, Yildiz M, Ozbek FM, Balci TA, et al. Different findings in Tc-99m MDP bone scintigraphy of patients with sickle cell disease: report of three cases. Ann Nucl Med . 2007 Jul. 21(5):311-4. . Solav SV, Bhandari R, Solav P (...) Sickle Cell Anemia, Skeletal Sickle Cell Anemia Skeletal Imaging: Overview, Radiography, Computed Tomography Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvNDEzNTQyLW92ZXJ2aWV3 processing > Sickle Cell Anemia

2014 eMedicine Radiology

58. Anemia, Sickle Cell (Treatment)

, and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor (...) non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . . Olujohungbe A, Howard J. The clinical care of adult patients

2014 eMedicine Emergency Medicine

59. Sickle Cell Anemia (Follow-up)

, and a history of priapism. Even modestly increased pulmonary artery pressures are associated with severe reduction in exercise capacity, as assessed by both the 6-minute walk and cardiopulmonary exercise testing, and herald a poor prognosis. Both pulmonary hypertension and cardiac sequelae, such as diastolic dysfunction, have been associated with accelerated mortality in the sickle cell disease population. For symptomatic patients, hydroxyurea and chronic transfusion have been used. Enothelin-1 receptor (...) non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc . 2008. 2008:859-62. . . Olujohungbe A, Howard J. The clinical care of adult patients

2014 eMedicine Pediatrics

60. 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

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