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Pulmonary Hypertension in Sickle Cell Anemia

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161. Hematopoietic Stem Cell Transplantation (Diagnosis)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine Pediatrics

162. Hematopoietic Stem Cell Transplantation (Overview)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine.com

163. Hematopoietic Stem Cell Transplantation (Treatment)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine.com

164. Hematopoietic Stem Cell Transplantation (Follow-up)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine.com

165. Hematopoietic Stem Cell Transplantation (Treatment)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine Pediatrics

166. Hematopoietic Stem Cell Transplantation (Overview)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine Pediatrics

167. Hematopoietic Stem Cell Transplantation (Diagnosis)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine.com

168. Hematopoietic Stem Cell Transplantation (Follow-up)

Aplastic anemia Pure red-cell aplasia Paroxysmal nocturnal hemoglobinuria Fanconi anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency (SCID) Wiskott-Aldrich syndrome Hemophagocytic lymphohistiocytosis Inborn errors of metabolism Epidermolysis bullosa Severe congenital neutropenia Shwachman-Diamond syndrome Diamond-Blackfan anemia Leukocyte adhesion deficiency HSCT-related morbidity and mortality Complications associated with HSCT include both early and late effects. Early-onset (...) HSCT Sickle cell anemia - Sickle cell anemia is potentially curable with allogeneic HSCT Genetic disorders - Many genetic immunologic or hematopoietic disorders are potentially curable with allogeneic HSCT Results of HSCT in patients with acquired immunodeficiency syndrome (AIDS) have not been found to be very encouraging. However, transfecting the hematopoietic stem cells with retroviral vectors that cleave the ribonucleic acid (RNA) of the human immunodeficiency virus (HIV) is in the experimental

2014 eMedicine Pediatrics

169. A Pilot Study of Chronic Red Blood Cell Transfusion in Sickle Cell Disease-Associated Pulmonary Hypertension

Keywords provided by University of North Carolina, Chapel Hill: 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 (...) A Pilot Study of Chronic Red Blood Cell Transfusion in Sickle Cell Disease-Associated Pulmonary Hypertension A Pilot Study of Chronic Red Blood Cell Transfusion in Sickle Cell Disease-Associated 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

2009 Clinical Trials

170. Anticoagulation For Pulmonary Hypertension in Sickle Cell Disease

of North Carolina, Chapel Hill ClinicalTrials.gov Identifier: Other Study ID Numbers: 09-1596 First Posted: December 21, 2009 Results First Posted: February 21, 2014 Last Update Posted: May 9, 2016 Last Verified: January 2014 Keywords provided by University of North Carolina, Chapel Hill: sickle cell disease pulmonary hypertension coagulation activation platelet activation endothelial activation Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Hypertension, Pulmonary Anemia (...) Anticoagulation For Pulmonary Hypertension in Sickle Cell Disease Anticoagulation For Pulmonary Hypertension 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. Anticoagulation

2009 Clinical Trials

171. Nonmyeloablative Haploidentical Peripheral Blood Mobilized Hematopoietic Precursor Cell Transplantation for Severe Congenital Anemias Including Sickle Cell Disease and Beta-Thalassemia

transfusion therapy; OR B. Sickle cell-related renal insufficiency defined by a creatinine level greater than or equal to 1.5 times the upper limit of normal and kidney biopsy consistent with sickle cell nephropathy OR nephrotic syndrome OR creatinine clearance less than 50mL/min OR requiring peritoneal or hemodialysis; OR C. Pulmonary hypertension defined as tricuspid regurgitant jet velocity (TRV) of greater than or equal to 2.5 m/s at baseline (without vaso-occlusive crisis); OR D. Sickle hepatopathy (...) Nonmyeloablative Haploidentical Peripheral Blood Mobilized Hematopoietic Precursor Cell Transplantation for Severe Congenital Anemias Including Sickle Cell Disease and Beta-Thalassemia Nonmyeloablative Haploidentical Peripheral Blood Mobilized Hematopoietic Precursor Cell Transplantation for Severe Congenital Anemias Including Sickle Cell Disease and Beta-Thalassemia - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration

2009 Clinical Trials

172. Evolution of novel small-molecule therapeutics targeting sickle cell vasculopathy. (PubMed)

Evolution of novel small-molecule therapeutics targeting sickle cell vasculopathy. A 34-year-old African American woman with sickle cell disease and history of relatively severe hemolysis, chronic leg ulcers, and mild pulmonary hypertension presented with a new ischemic stroke. Recent research has suggested a syndrome of hemolysis-associated vasculopathy in patients with sickle cell disease, which features severe hemolytic anemia and leads to scavenging of nitric oxide and its biochemical (...) precursor l-arginine. This diminished bioavailability of nitric oxide promotes a hemolysis-vascular dysfunction syndrome, which includes pulmonary hypertension, cutaneous leg ulceration, priapism, and ischemic stroke. Additional correlates of this vasculopathy include activation of endothelial cell adhesion molecules, platelets, and the vascular protectant hemeoxygenase-1. Some known risk factors for atherosclerosis are also associated with sickle cell vasculopathy, including low levels

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2008 JAMA

173. Reversible posterior leukoencephalopathy syndrome in sickle cell anemia (PubMed)

Reversible posterior leukoencephalopathy syndrome in sickle cell anemia A 10-year-old African American girl with sickle-cell anemia developed headaches and seizures associated with hypertension during hospitalization for a pulmonary abscess. Hypertension developed after multiple transfusions, associated with abnormally high hematocrit and headache. Magnetic resonance imaging was consistent with posterior leukoencephalopathy. Neurologic signs, hypertension, and high hematocrit resolved after (...) erythrocytapheresis. Magnetic resonance imaging, 1 month after the episode, produced normal results. Because reversible posterior leukoencephalopathy syndrome was only described in sickle-cell anemia during severe acute chest syndrome, this report documents that milder illness can be associated with reversible posterior leukoencephalopathy syndrome in sickle-cell anemia, and also highlights subtle signs that may herald serious neurologic events in high-risk patients. Examination of the pathophysiology

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2009 Pediatric Neurology

174. Repeat Dose Safety and Efficacy Study for Compound to Treat Anemia

hematologic disease, including, but not limited to sickle cell disease, hemosiderosis, hemochromatosis, myelodysplastic syndrome, hematologic malignancy, myeloma Post-renal transplantation patients with functioning transplant. (Failed transplant subjects back on hemodialysis are eligible). Acute peptic ulcer disease or history of chronic rectal bleeding. History of malignancy tumor within 5 years prior to Screening or are receiving medication for cancer. Non-melanoma skin cancer within the past 5 years (...) Repeat Dose Safety and Efficacy Study for Compound to Treat Anemia Repeat Dose Safety and Efficacy Study for Compound to Treat 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. Repeat Dose Safety

2010 Clinical Trials

175. Management of Stroke in Neonates and Children

stroke, and updates in regard to areas of childhood stroke that have not received close attention such as sickle cell disease. Each section is highlighted with considerations for clinical practice, attendant controversies, and knowledge gaps. This statement provides the practicing provider with much-needed updated information in this field. Much has transpired since the last scientific statement on pediatric stroke was published 10 years ago. Although stroke has long been recognized as an adult (...) by sickle cell disease (SCD), which amplifies stroke risk >200-fold. Hemorrhagic stroke in children can be intracerebral hemorrhage (ICH), IVH, or SAH. Hemorrhagic stroke makes up about half of pediatric stroke, with an incidence of ≈1 to 1.7 in 100 000 per year. , Global Perspective Krishnamurthi et al used all available global data on stroke incidence, prevalence, and mortality to evaluate differences in stroke between developed and developing countries and changes from 1990 to 2013. In 2013

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2019 American Heart Association

176. Heart Disease and Stroke Statistics

Cardiac Arrest, Ventricular Arrhythmias, and Inherited Channelopathies e377 18. Subclinical Atherosclerosis e401 19. Coronary Heart Disease, Acute Coronary Syndrome, and Angina Pectoris e415 20. Cardiomyopathy and Heart Failure e438 21. Valvular Diseases e455 22. Venous Thromboembolism (Deep Vein Thrombosis and Pulmonary Embolism), Chronic Venous Insufficiency, Pulmonary Hypertension e472 23. Peripheral Artery Disease and Aortic Diseases e481 Outcomes 24. Quality of Care e497 25. Medical Procedures (...) disease. Admissions for endocarditis related to injection drug use have risen in recent years in parallel with the opioid drug crisis. The prevalence of documented intravenous drug use among people admitted to a hospital because of endocarditis in the National (Nationwide) Inpatient Sample rose from 4.3% in 2008 to 10% in 2014. Venous Thromboembolism (Deep Vein Thrombosis and Pulmonary Embolism), Chronic Venous Insufficiency, Pulmonary Hypertension (Chapter 22) Traditional atherosclerotic risk factors

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2019 American Heart Association

177. Vascular imaging

– intracranial arteries Includes atherosclerotic disease, Moyamoya disease, sickle cell anemia, and idiopathic progressive arteriopathy of childhood. Advanced imaging is considered medically necessary when the results of imaging are essential to establish a diagnosis and/or direct management. IMAGING STUDY - CTA or MRA brain Trigeminal neuralgia Advanced imaging is considered medically necessary when the results of imaging are essential to establish a diagnosis and/or direct management. IMAGING STUDY - CTA (...) treatment decisions. ? Hematoma ? Pulmonary arterial hypertension ? Pulmonary arteriovenous malformation ? Pulmonary sequestration ? Subclavian steal syndrome ? Superior vena cava syndrome ? Systemic venous thrombosis or occlusion ? Thoracic outlet syndrome IMAGING STUDY - CTA or MRA chest - CT or MRI chest (alternative modalities for evaluation of superior vena cava syndrome and thoracic outlet syndrome) Abdomen and Pelvis Aneurysm of the abdominal aorta ADULT Advanced imaging is considered medically

2019 AIM Specialty Health

178. Chronic kidney disease

Follow us: © 2017 Trip Database Ltd. company number 04316414. Trip is proud to be made in the UK. 2018 18. Interventions for chronic kidney disease in people with sickle cell disease . BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent (...) and chronic kidney insufficiency . Up to 12% of people who develop sickle cell nephropathy will develop end-stage renal 2017 19. Cost-effectiveness of screening and optimal management for diabetes, hypertension, and chronic kidney disease : a modeled analysis Cost-effectiveness of screening and optimal management for diabetes, hypertension, and chronic kidney disease : a modeled analysis Cost-effectiveness of screening and optimal management for diabetes, hypertension, and chronic kidney disease

2018 Trip Latest and Greatest

179. Society of Interventional Radiology Consensus Guidelines for the Periprocedural Management of Thrombotic and Bleeding Risk in Patients Undergoing Percutaneous Image-Guided Interventions—Part II: Recommendations.

• Mechanical mitral heart valve • Active cancer Hypertension (systolic BP > 160 mm Hg) 1 Abnormal renal function (dialysis, renal transplantation, serum Cr > 200 μmol/L) 1 Abnormal liver function (cirrhosis or bilirubin > 2× ULN, AST or ALT > 3× ULN) 1 Prior stroke 1 History of major bleeding or predisposition to bleeding (anemia) 1 Labile INR (VKA) defined as time in therapeutic range < 60% 1 Age > 65 y 1 Concomitant use of antiplatelet agent or NSAID 1 History of alcohol or drug use (> 8 drinks per week (...) nonsevere thrombophilia (eg, heterozygous Factor V Leiden) HTN history 1 • Rheumatic valvular heart disease Vascular disease history 1 • Recurrent idiopathic VTE Age 65–74 y 1 • VTE within 3 mo Age > 75 y 2 • VTE of any duration with severe thrombophilia (eg, homozygous factor V Leiden or positive antiphospholipid antibodies) History of stroke/TIA/thromboembolism 2 • Cancer-associated thrombosis CHF = congestive heart failure; HTN = hypertension; mo = months; TIA = transient ischemic attack; VTE

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2019 Society of Interventional Radiology

180. Male Sexual Dysfunction

and impotence in homozygous sickle cell disease. Arch Intern Med, 1980. 140: 1434. 10. Lionnet, F., et al. Hemoglobin sickle cell disease complications: a clinical study of 179 cases. Haematologica, 2012. 97: 1136. 11. Olujohungbe, A.B., et al. A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study. J Androl, 2011. 32: 375. 12. Wespes, E., et al., EAU Guidelines Panel (...) in the general population. Urology, 2001. 57: 970. 6. Kulmala, R.V., et al. Priapism, its incidence and seasonal distribution in Finland. Scand J Urol Nephrol, 1995. 29: 93. 7. Furtado, P.S., et al. The prevalence of priapism in children and adolescents with sickle cell disease in Brazil. Int J Hematol, 2012. 95: 648. 8. Adeyoju, A.B., et al. Priapism in sickle-cell disease; incidence, risk factors and complications - an international multicentre study. BJU Int, 2002. 90: 898. 9. Emond, A.M., et al. Priapism

2019 European Association of Urology

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