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Pulmonary Hypertension in Sickle Cell Anemia

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141. FG-4592 (ASP1517) in the Treatment of Anemia in Chronic Kidney Disease Patients Not Requiring Dialysis

to a maximum of 160.0 kg. Exclusion criteria: Subject has received any ESA treatment within 12 weeks prior to randomization. Subject has had more than one dose of IV iron within 12 weeks prior to randomization. Subject has received a RBC transfusion within 8 weeks prior to randomization. Subject has a known history of myelodysplastic syndrome or multiple myeloma. Subject has a known hereditary hematologic disease such as thalassemia or sickle cell anemia, pure red cell aplasia, or other known causes (...) , seizure, or a thrombotic/thromboembolic event (e.g., pulmonary embolism) within 12 weeks prior to randomization. Uncontrolled hypertension or two or more blood pressure (BP) values of systolic BP (SBP) greater than or equal to 160 mmHg or diastolic BP (DBP) greater than or equal to 95 mmHg confirmed by repeat measurement within 2 weeks prior to randomization. Subject has a diagnosis or suspicion (e.g., complex kidney cyst of Bosniak Category 2F or higher) of renal cell carcinoma on renal ultrasound

2013 Clinical Trials

142. Sickle Cell Disease

crisis occurs when marrow erythropoiesis slows during acute infection due to human parvovirus, during which an acute erythroblastopenia may occur. Acute chest syndrome results from pulmonary microvascular occlusion and is a common cause of death, with mortality rates of up to 10%. It occurs in all age groups but is most common in childhood. Repeated episodes predispose to chronic . In children, acute sequestration of sickled cells in the spleen may occur, exacerbating anemia. , a serious complication (...) of ADP Receptor ADP receptors are integral proteins embedded in a platelet membrane. When ADP binds, it causes a conformational change in the fibrinogen receptor, which allows fibrinogen to bind to the receptors... SOCIAL MEDIA Add to Any Platform Loading , MD, PhD, Johns Hopkins School of Medicine Click here for Patient Education NOTE: This is the Professional Version. CONSUMERS: Topic Resources Sickle cell disease (a hemoglobinopathy—see ) causes a chronic hemolytic anemia occurring almost

2013 Merck Manual (19th Edition)

143. Changes in Bi-ventricular Function After Hematopoietic Stem Cell Transplant as Assessed by Speckle Tracking Echocardiography. (PubMed)

Changes in Bi-ventricular Function After Hematopoietic Stem Cell Transplant as Assessed by Speckle Tracking Echocardiography. Hematopoietic stem cell transplant (HSCT) is a therapeutic option for patients with sickle cell disease (SCD) and severe acquired aplastic anemia (SAA). HSCT may have beneficial effects on ventricular function in damaged myocardium. We hypothesized improvement in ventricular performance and pulmonary hypertension following HSCT with strain echocardiography in SCD and SAA (...) initial decline (from - 11.1 to - 17.5, P = 0.009) but was comparable to baseline (P = 0.43). Other measurements of bi-ventricular function did not change significantly. Tricuspid regurgitation velocities as surrogates for pulmonary hypertension improved in the subset of patients with baseline elevated values although data points were limited. Abnormal myocardial systolic function was detected at baseline with strain imaging. HSCT was associated with initial worsening longitudinal strain values

2017 Pediatric Cardiology

144. Losartan to Reverse Sickle Nephropathy

to Brief Summary: Sickle cell disease causes kidney damage with increasing age, leading to chronic kidney disease and renal failure in nearly one third of patients with sickle cell disease. Currently, there is no treatment for sickle cell related kidney disease. Condition or disease Intervention/treatment Phase Nephropathy Sickle Cell Anemia Drug: Losartan Phase 2 Detailed Description: The purpose of this research study is to see if losartan can help reduce or reverse damage done to the kidneys (...) of children and adults with Sickle Cell Anemia (SCA) and Sickle Beta-zero (HbSβ0) Thalassemia. Study Design Go to Layout table for study information Study Type : Interventional (Clinical Trial) Actual Enrollment : 36 participants Intervention Model: Single Group Assignment Masking: None (Open Label) Primary Purpose: Treatment Official Title: A Phase II Trial of Losartan to Reverse Sickle Nephropathy Study Start Date : February 2012 Actual Primary Completion Date : November 2015 Actual Study Completion

2011 Clinical Trials

145. 4 Week Switch Study in Hemodialysis-dependent Subjects With Anemia Associated With Chronic Kidney Disease

haematological disease including those affecting platelets, the coagulation disorders (e.g., Protein C or S deficiency) or red blood cells (e.g. sickle cell anemia, myelodysplastic syndromes, haematological malignancy, myeloma, haemolytic anemia) or any other cause of anemia other than renal disease. Liver disease: Current liver disease, known hepatic or biliary abnormalities (with the exception of Gilbert's syndrome or asymptomatic gallstones) or evidence at Screening of abnormal liver function tests (...) taking hypertension medication(s) before screening and dialysis, if required. DBP >105 mmHg or SBP>170 mmHg for subjects who are asked to hold hypertension medication(s) before screening and dialysis. Thrombotic Disease: History of thrombotic disease (e.g., venous thrombosis such as deep vein thrombosis or pulmonary embolism, or arterial thrombosis such as new onset or worsening limb ischemia requiring intervention), or other thrombosis related condition except shunt thrombosis) within the prior 6

2012 Clinical Trials

146. 4 Week Correction Study in Subjects With Anemia Associated With Chronic Kidney Disease Who Are Not Undergoing Dialysis

). Hematological disease: Any hematological disease including those affecting platelets, the coagulation disorders (e.g., Protein C or S deficiency) or red blood cells (e.g. sickle cell anemia, myelodysplastic syndromes, hematological malignancy, myeloma, hemolytic anemia) or any other cause of anemia other than renal disease. Liver disease: Current liver disease, known hepatic or biliary abnormalities (with the exception of Gilbert's syndrome or asymptomatic gallstones) or evidence at Screening of abnormal (...) : Class III/IV heart failure, as defined by the New York Heart Association (NYHA) functional classification system. Hypertension: Poorly controlled hypertension, whether due to inadequate treatment, or lack of treatment, defined as DBP >100 mmHg or SBP>160 mmHg. Thrombotic disease: History of thrombotic disease (e.g., venous thrombosis such as deep vein thrombosis or pulmonary embolism, or arterial thrombosis such as new onset or worsening limb ischemia requiring intervention), or other thrombosis

2012 Clinical Trials

147. Pulmonary Hypertension

, methamphetamines, L-tryptophan, and dasatinib ( ). Patients with hereditary causes of , such as , are at high risk of developing pulmonary hypertension (10% of cases based on right heart catheterization criteria). The mechanism is related to intravascular hemolysis and release of cell-free hemoglobin into the plasma, which scavenges nitric oxide, generates reactive oxygen species, and activates the hemostatic system. Other risk factors for pulmonary hypertension in sickle cell disease include , liver (...) sclerosis, sickle cell disease, or HIV infection with pulmonary arterial hypertension have a worse prognosis than those without pulmonary arterial hypertension. For example, patients with sickle cell disease and pulmonary hypertension have a 40% 4-year mortality rate. Treatment Avoidance of activities that may exacerbate the condition (eg, cigarette smoking, high altitude, pregnancy, use of sympathomimetics) Idiopathic and familial pulmonary arterial hypertension: IV epoprostenol ; inhaled, oral, sc

2013 Merck Manual (19th Edition)

148. Pulmonary Embolism (Overview)

. MULTIDETECTOR COMPUTED TOMOGRAPHY FOR ACUTE PULMONARY EMBOLISM: EMBOLIC BURDEN AND CLINICAL OUTCOME. Chest . 2012 May 24. . Restrepo CS, Artunduaga M, Carrillo JA, Rivera AL, Ojeda P, Martinez-Jimenez S, et al. Silicone pulmonary embolism: report of 10 cases and review of the literature. J Comput Assist Tomogr . 2009 Mar-Apr. 33(2):233-7. . Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest (...) ventricle. Pulmonary thromboembolism is not a disease in and of itself. Rather, it is a complication of underlying venous thrombosis. Under normal conditions, microthrombi (tiny aggregates of red cells, platelets, and fibrin) are formed and lysed continually within the venous circulatory system. Signs and symptoms The classic presentation of pulmonary embolism is the abrupt onset of pleuritic chest pain, shortness of breath, and hypoxia. However, most patients with pulmonary embolism have no obvious

2014 eMedicine.com

149. Pulmonary Embolism (Overview)

. MULTIDETECTOR COMPUTED TOMOGRAPHY FOR ACUTE PULMONARY EMBOLISM: EMBOLIC BURDEN AND CLINICAL OUTCOME. Chest . 2012 May 24. . Restrepo CS, Artunduaga M, Carrillo JA, Rivera AL, Ojeda P, Martinez-Jimenez S, et al. Silicone pulmonary embolism: report of 10 cases and review of the literature. J Comput Assist Tomogr . 2009 Mar-Apr. 33(2):233-7. . Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest (...) ventricle. Pulmonary thromboembolism is not a disease in and of itself. Rather, it is a complication of underlying venous thrombosis. Under normal conditions, microthrombi (tiny aggregates of red cells, platelets, and fibrin) are formed and lysed continually within the venous circulatory system. Signs and symptoms The classic presentation of pulmonary embolism is the abrupt onset of pleuritic chest pain, shortness of breath, and hypoxia. However, most patients with pulmonary embolism have no obvious

2014 eMedicine Emergency Medicine

150. Pulmonary Embolism (Diagnosis)

. MULTIDETECTOR COMPUTED TOMOGRAPHY FOR ACUTE PULMONARY EMBOLISM: EMBOLIC BURDEN AND CLINICAL OUTCOME. Chest . 2012 May 24. . Restrepo CS, Artunduaga M, Carrillo JA, Rivera AL, Ojeda P, Martinez-Jimenez S, et al. Silicone pulmonary embolism: report of 10 cases and review of the literature. J Comput Assist Tomogr . 2009 Mar-Apr. 33(2):233-7. . Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest (...) ventricle. Pulmonary thromboembolism is not a disease in and of itself. Rather, it is a complication of underlying venous thrombosis. Under normal conditions, microthrombi (tiny aggregates of red cells, platelets, and fibrin) are formed and lysed continually within the venous circulatory system. Signs and symptoms The classic presentation of pulmonary embolism is the abrupt onset of pleuritic chest pain, shortness of breath, and hypoxia. However, most patients with pulmonary embolism have no obvious

2014 eMedicine Emergency Medicine

151. Pulmonary Embolism (Diagnosis)

. MULTIDETECTOR COMPUTED TOMOGRAPHY FOR ACUTE PULMONARY EMBOLISM: EMBOLIC BURDEN AND CLINICAL OUTCOME. Chest . 2012 May 24. . Restrepo CS, Artunduaga M, Carrillo JA, Rivera AL, Ojeda P, Martinez-Jimenez S, et al. Silicone pulmonary embolism: report of 10 cases and review of the literature. J Comput Assist Tomogr . 2009 Mar-Apr. 33(2):233-7. . Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest (...) ventricle. Pulmonary thromboembolism is not a disease in and of itself. Rather, it is a complication of underlying venous thrombosis. Under normal conditions, microthrombi (tiny aggregates of red cells, platelets, and fibrin) are formed and lysed continually within the venous circulatory system. Signs and symptoms The classic presentation of pulmonary embolism is the abrupt onset of pleuritic chest pain, shortness of breath, and hypoxia. However, most patients with pulmonary embolism have no obvious

2014 eMedicine.com

152. Pulmonary hypertension: an increasingly recognized complication of hereditary hemolytic anemias and HIV infection. (PubMed)

Pulmonary hypertension: an increasingly recognized complication of hereditary hemolytic anemias and HIV infection. Modern health care has greatly increased longevity for patients with congenital hemolytic anemias (such as sickle cell disease and thalassemia) and human immunodeficiency virus (HIV) infection. It is estimated that 10% of patients with hemoglobinopathies and 0.5% of patients with HIV infection develop moderate to severe pulmonary hypertension. Pulmonary hypertension (...) is a relentlessly progressive disease leading to right heart failure and death. Worldwide, there are an estimated 30 million patients with sickle cell disease or thalassemia and 40 million patients with HIV disease. Considering the prevalence of pulmonary vascular disease in these populations, sickle cell disease and HIV disease may be the most common causes of pulmonary hypertension worldwide. In this review, the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies

2008 JAMA

153. Exercise-induced Pulmonary Hypertension in Patients With Sickle-cell Anemia

Exercise-induced Pulmonary Hypertension in Patients With Sickle-cell Anemia Exercise-induced Pulmonary Hypertension in Patients 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 (...) . Exercise-induced Pulmonary Hypertension in Patients With Sickle-cell Anemia 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: NCT00948012 Recruitment Status : Completed First Posted : July 29, 2009 Last Update Posted : July 29, 2009 Sponsor: Federal University of São Paulo Information provided by: Federal

2009 Clinical Trials

154. Sickle-cell disease

). "Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease". American Journal of Respiratory and Critical Care Medicine . 177 (6): 646–53. . : . . Paul RN, Castro OL, Aggarwal A, Oneal PA (September 2011). "Acute chest syndrome: sickle cell disease". European Journal of Haematology . 87 (3): 191–207. : . . Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009-05-28). Robbins and Cotran Pathologic Basis of Disease, Professional Edition: Expert Consult (...) breathing, and pulmonary infiltrate on a . Given that pneumonia and sickling in the lung can both produce these symptoms, the patient is treated for both conditions. It can be triggered by painful crisis, respiratory infection, bone-marrow embolisation, or possibly by atelectasis, opiate administration, or surgery. [ ] may also occur. Genetics [ ] Sickle cell disease is inherited in an pattern. Distribution of the sickle cell trait, shown in pink and purple Historical distribution of (no longer endemic

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2012 Wikipedia

155. Adenosine 2A Agonist Lexiscan in Children and Adults With Sickle Cell Disease

AND CLOSED TO ACCRUAL) Participants must have sickle cell anemia confirmed by hemoglobin analysis Participants must report that their pain is at baseline. Additionally, they cannot report an increase in dose or frequency of opioid use in the last 2 weeks prior to drug administration Age 21-70 years Participants must have the laboratory indices as outlined in the protocol Participants must have reliable IV access as determined by the investigator Women of child-bearing potential and men must agree to use (...) adequate contraception prior to study entry and for the duration of the study. Inclusion Criteria Stage III: (COMPLETE AND CLOSED TO ACCRUAL) Participants must have sickle cell anemia confirmed by hemoglobin analysis Participant is admitted to the hospital for a pain episode Age 21-70 years Participants must have the laboratory indices as outlined in the protocol Participants must have reliable IV access as determined by the investigator Women of child-bearing potential and men must agree to use

2010 Clinical Trials

156. Cardiovascular Complications of Sickle Cell Disease

to this study by ClinicalTrials.gov Identifier (NCT Number): Layout table for additonal information Responsible Party: University of Chicago ClinicalTrials.gov Identifier: Other Study ID Numbers: 16653A First Posted: January 8, 2010 Last Update Posted: August 24, 2016 Last Verified: August 2016 Keywords provided by University of Chicago: Sickle Cell Disease Cardiac magnetic resonance imaging Coronary Disease Pulmonary Hypertension Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle (...) by (Responsible Party): University of Chicago Study Details Study Description Go to Brief Summary: In this research study, we are using heart imaging exams and blood testing, in order to gain an improved understanding of the pulmonary (lung) hypertension and cardiovascular (heart) complications that often occur in sickle cell patients. Information gathered from the healthy volunteers that participate in this study will be compared to information from the sickle cell patients in this study in order to help

2010 Clinical Trials

157. L-Arginine and Sickle Cell Disease

Bittar, Hospital de Clínicas de Porto Alegre ClinicalTrials.gov Identifier: Other Study ID Numbers: 04-487 cep@hcpa.ufrgs.br First Posted: June 11, 2010 Last Update Posted: June 11, 2010 Last Verified: June 2010 Keywords provided by Hospital de Clinicas de Porto Alegre: sickle cell disease L-arginine hemolysis nitric oxide pulmonary arterial hypertension Additional relevant MeSH terms: Layout table for MeSH terms Anemia, Sickle Cell Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic (...) Indústria e Comércio Ltda. (donation of L-arginine) Information provided by: Hospital de Clinicas de Porto Alegre Study Details Study Description Go to Brief Summary: One of the main problems in sickle cell disease is the decreased bioavailability of nitric oxide and arginine. This study was designed to assess if treating sickle cell disease patients with L-arginine would improve pulmonary arterial pressure and other aspects. Condition or disease Intervention/treatment Phase Sickle Cell Disease Drug: L

2010 Clinical Trials

158. Pulmonary Hypertension and Imatinib

is to determine whether circulating molecular and cellular biomarkers are predictive of imatinib effect on pulmonary artery hypertension. Condition or disease PAH Detailed Description: We hypothesize that bone marrow progenitor cells are mobilized into the circulation in PAH, home to the lungs and differentiate into mast cells, which promote vascular remodeling and vasoconstriction through release of renin and chymase. As a corollary to this, we hypothesize that anti cKit tyrosine kinase inhibitor (TKI (...) ), imatinib, provides clinical benefit to patients through inhibition of mast cell progenitor proliferation, mobilization and differentiation. To test this, we will determine if mast cell progenitors and mast cell biomarkers are related to imatinib clinical response. This will be an ancillary study, part of a placebo-controlled, double-blind multi center clinical trial of imatinib in pulmonary arterial hypertension. Study Design Go to Layout table for study information Study Type : Observational Estimated

2010 Clinical Trials

159. Glutamine Therapy for Hemolysis-Associated Pulmonary Hypertension

: Pulmonary Hypertension Sickle Cell Disease Thalassemia Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Hypertension, Pulmonary Anemia, Sickle Cell Thalassemia Hemolysis Vascular Diseases Cardiovascular Diseases Lung Diseases Respiratory Tract Diseases Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Pathologic Processes (...) ): Children's Hospital & Research Center Oakland Study Details Study Description Go to Brief Summary: The primary hypothesis of this study is that glutamine supplementation will improve the erythrocyte glutamine/glutamate ratio, a biomarker of oxidative stress, hemolysis and pulmonary hypertension (PH) in sickle cell disease (SCD) and thalassemia (Thal) patients with PH. PH is defined as a tricuspid regurgitant jet velocity (TRV) on Doppler echocardiography > 2.5 m/s. We also predict that glutamine therapy

2010 Clinical Trials

160. Effect of BMPR-2 Gene Mutations on Hemodynamic Response by Iloprost Inhalation in Pulmonary Arterial Hypertension

The patients who changes medication administered during ventavis treatment The patients with allergic reaction to ventavis The patients with other systemic disease (ex. Leukemia, MM, Sickle cell anemia, significant liver disease) Contacts and Locations Go to Information from the National Library of Medicine To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor. Please refer to this study by its ClinicalTrials.gov (...) Effect of BMPR-2 Gene Mutations on Hemodynamic Response by Iloprost Inhalation in Pulmonary Arterial Hypertension Effect of BMPR-2 Gene Mutations on Hemodynamic Response by Iloprost Inhalation 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

2010 Clinical Trials

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