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Pulmonary Hypertension in Sickle Cell Anemia

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361. Non-Myeloablative Conditioning and Bone Marrow Transplantation

. Patients with an HLA-matched related donor will proceed to a matched BMT. Age 2-70 years Good performance status (ECOG 0 or 1; Karnofsky and Lansky 70-100) Patients and donors must be able to sign consent forms. First degree relative should be willing to donate Patients must be geographically accessible and willing to participate in all stages of treatment. Eligible diagnoses: Patients with sickle cell anemia such as sickle cell anemia (Hb SS), Hb Sβ° thalassemia, Hb Sβ+thalassemia, Hb SC disease, Hb (...) Center Investigators Layout table for investigator information Principal Investigator: Adetola A Kassim, MD Vanderbilt-Ingram Cancer Center More Information Go to Additional Information: Layout table for additonal information Responsible Party: Adetola A. Kassim, Associate Professor of Medicine; Clinical Director, Sickle Cell Anemia Program; Medical Oncologist, Vanderbilt-Ingram Cancer Center ClinicalTrials.gov Identifier: Other Study ID Numbers: VICCNC BMT 12108 First Posted: May 9, 2013 Last Update

2013 Clinical Trials

362. Study to Determine How Cialis Effects the Renal Function in Response to Volume Expansion in Preclinical Systolic Cardiomyopathy (Aim2)

taking the following selective alpha blockers and who are unable to stop for the duration of the study; Alfuzosin Prazosin Doxazosin Tamsulosin Terazosin Silodosin Patients with retinitis pigmentosa, previous diagnosis of nonischemic optic neuropathy, untreated proliferative retinopathy or unexplained visual disturbance Patients with sickle cell anemia, multiple myeloma, leukemia or penile deformities placing them at risk for priapism (angulation, cavernosal fibrosis or Peyronie's disease) Patients (...) with an allergy to iodine. Patients on PDEV inhibition for pulmonary hypertension Patients on PDEV inhibition for erectile dysfunction who are not willing to stop the medication for the duration of the study Valve disease (> moderate aortic or mitral stenosis; > moderate aortic or mitral regurgitation) Obstructive Hypertrophic cardiomyopathy Infiltrative or inflammatory myocardial disease (amyloid, sarcoid) Pericardial disease Have experienced a myocardial infarction or unstable angina, or have undergone

2013 Clinical Trials

363. Reduced Intensity Conditioning in Patients Aged ≤35 With Non-Malignant Disorders Undergoing UCBT, BMT, or PBSCT

Inflammatory Conditions Crohn's Disease/Inflammatory Bowel Disease Exclusion: Allogeneic hematopoietic stem cell transplant within the previous 6 months. Any active malignancy or MDS. Severe acquired aplastic anemia. Uncontrolled bacterial, viral or fungal infection (currently taking medication and with progression of clinical symptoms). Pregnancy or nursing mother. Poorly controlled pulmonary hypertension. Any condition that precludes serial follow-up. Contacts and Locations Go to Information from (...) mannosidosis Gaucher Disease Other inheritable metabolic diseases where hematopoietic stem cell transplantation may be beneficial. Hereditary anemias Thalassemia major Sickle cell disease (SCD) - patients with sickle disease must have one or more of the following: Overt or silent stroke Pain crises ≥ 2 episodes per year for past year One or more episodes of acute chest syndrome Osteonecrosis involving ≥ 1 joints Priapism Diamond Blackfan Anemia (DBA) Other congenital transfusion dependent anemias

2013 Clinical Trials

364. A Phase 1, Open-Label Study of Intravenous Sildenafil in Patients With Cirrhosis

, myocardial infarctus, life-threatening arrhythmia or stroke within 6 months before enrollment History of retinitis pigmentosa Anatomical deformation of the penis (Peyronie's disease, angulation, cavernosal fibrosis) History of sickle cell anemia, multiple myeloma or leukemia Renal failure treated with dialysis Cognitive impairment based on IRB "evaluation to sign consent form" Transjugular intrahepatic porto-systemic shunt placement Previous kidney or liver transplantation Contacts and Locations Go (...) Eligible for Study: All Accepts Healthy Volunteers: No Criteria Subjects will be eligible for the study if they meet the following inclusion criteria: Cirrhosis. Diagnosis of cirrhosis will be based on either liver biopsy OR clinical characteristics (e.g. history of ascites or encephalopathy or esophageal varices or gastric varices or splenomegaly or spider angioma or any clinical sign of portal hypertension/cirrhosis), laboratory (e.g. history of thrombocytopenia or history of APRI (Reference: Wai CT

2013 Clinical Trials

365. The Effects of Adding TCM-700C on the Standard Combination Treatment for HCV Genotype 1 Patients(Phase III)

than cornea or hair transplant or skin graft); Severe concurrent medical disease such as severe hypertension, significant coronary heart disease, poorly controlled diabetes mellitus (glycated hemoglobin A1c [HbA1c] >8.5%), not adequately controlled thyroid dysfunction, chronic obstructive pulmonary disease, severe infections (bacterial, viral, fungal, including acute tuberculosis), or hemoglobinopathies (thalassemia major or sickle cell anemia); Autoimmune hepatitis or other autoimmune conditions (...) <1500 cells/mm3; Hemoglobin <12 g/dL for women and <13 g/dL for men; Creatinine >1.5 mg/dL; Alanine aminotransferase (ALT) and/or aspartate aminotransferase (AST) >10 x upper limit of normal (ULN); Total serum bilirubin >1.5 x ULN; Subjects without cirrhosis and AFP >50 ng/mL must have an ultrasound between the screening and baseline visit with no findings suspicious for HCC. Medical conditions which are contraindications for PegIFNα 2a or RBV therapy: Psychiatric disorders; Organ transplant (other

2013 Clinical Trials

366. New Treatment Response in People With and Without Cirrhosis From Chronic Hepatitis C

) History of hemoglobinopathies (e.g., thalassemia major or sickle cell anemia), diagnoses associated with an increased baseline risk for anemia (e.g., spherocytosis), hemolytic anemia, or diseases in which anemia would be medically problematic, or hemophilia; c) Pre-existing ophthalmologic disorders considered clinically significant on eye exam, including retinal, examination. Note: all subjects with a history of diabetes or hypertension must have a documented eye exam within 12 months prior (...) , sarcoidois, etc; f) History of cardiomyopathy, coronary artery disease (including angina), interventional procedure for coronary artery disease (including angioplasty, stent procedure, or cardiac bypass surgery), ventricular arrhythmia, congestive heart failure, pulmonary hypertension, cardiomyopathy, or other clinically significant cardiac disease; g) Historical or current ECG findings indicative of cardiovascular instability, including but not limited to evidence of significant myocardial ischemia

2013 Clinical Trials

367. Pregnancy Related Causes of Deaths in Ghana: A 5-Year Retrospective Study (Full text)

%) and genital tract sepsis (2.5%). The remaining 20.5% (130) resulted from indirect obstetric causes, including: infections outside the genital tract, (9.2%), anemia (2.8%), sickle cell disease (2.7%), pulmonary embolism (1.9%) and disseminated intravascular coagulation (1.3%). The top five causes of maternal death were: haemorrhage (21.8%), abortion (20.7%), hypertensive disorders (19.4%), infections (9.1%) and ectopic gestation (8.7%).Ghana continues to have persistently high levels of preventable causes (...) statistical software (Version 19).Of 5,247 deaths among women aged 15-49, 12.1% (634) were pregnancy-related. Eighty one percent of pregnancy-related deaths (517) occurred in the community or within 24 hours of admission to a health facility and 18.5% (117) occurred in a health facility. Out of 634 pregnancy-related deaths, 79.5% (504) resulted from direct obstetric causes, including: haemorrhage (21.8%), abortion (20.8%), hypertensive disorders (19.4%), ectopic gestation (8.7%), uterine rupture (4.3

2013 Ghana Medical Journal

368. Sildenafil Versus Placebo in Chronic Heart Failure

ischemic optic-neuropathy), unexplained visual disturbance. Sickle cell anemia, multiple myeloma, leukemia or penile anatomic deformities (angulation, cavernosal fibrosis, Peyronie`s disease) that increases the risk of priapism. Hepatic failure. Drug and alcohol abuse which precludes compliance with the protocol. Inability to understand or sign the written informed consent form of the study, Contacts and Locations Go to Information from the National Library of Medicine To learn more about this study (...) Study Description Go to Brief Summary: This protocol describes a 2-arm randomised controlled pilot study assessing the tolerance, safety and efficacy of sildenafil compared to control. The hypothesis is that sildenafil will be well tolerated and efficacious in patients with chronic heart failure (NYHA class II and III) with evidence of systolic dysfunction (EF ≤40 %) and secondary pulmonary hypertension (SPAP >40mmHg). Patients that satisfy the inclusion criteria will be randomized to sildenafil

2012 Clinical Trials

369. Safety Study of Recombinant Interferon Variant(PEG-IFN-SA) to Treat HCV Disease

thyroid disease, diabetes, etc. pulmonary diseases, including invasive pulmonary disease, pneumonia, shortness of breath. Eye diseases, including retinopathy, retinopathy. chronic infectious disease history (history of tuberculosis). chronic kidney disease, serum creatinine level> 1.5 times upper limit of normal at screening, renal insufficiency, renal anemia history. anemia (including thalassemia, sickle hemoglobin, anemia), and hemophilia. peptic ulcer not controlled, colitis, pancreatitis (...) and vomiting. HCV RNA level was tested by COBAS Taqman HCV Test system of Roche. Blood cell counts were tested using an automatic cell counter such as WBC, neutrophil, PLT and HGB. Biochemical indicators were tested by automatic biochemical analysis instrument. Study Design Go to Layout table for study information Study Type : Interventional (Clinical Trial) Actual Enrollment : 80 participants Intervention Model: Single Group Assignment Masking: None (Open Label) Primary Purpose: Treatment Official Title

2012 Clinical Trials

370. Tadalafil and Nesiritide as Therapy in Pre-clinical Heart Failure

Patients with sickle cell anemia, multiple myeloma, leukemia or penile deformities placing them at risk for priapism (angulation, cavernosal fibrosis or Peyronie's disease) Contraindication to nesiritide. Patients with an allergy to iodine. Valve disease (> moderate aortic or mitral stenosis; > moderate aortic or mitral regurgitation) Hypertrophic cardiomyopathy Infiltrative or inflammatory myocardial disease (amyloid, sarcoid) Pericardial disease Have experienced a myocardial infarction or unstable (...) output but did not improve renal function. However, chronic PDEV inhibition did enhance the renal actions of exogenous BNP, specifically improving glomerular filtration rate (GFR) and renal cGMP generation. PDEV inhibitors are FDA approved for erectile dysfunction and pulmonary hypertension. Study Design Go to Layout table for study information Study Type : Interventional (Clinical Trial) Actual Enrollment : 43 participants Allocation: Randomized Intervention Model: Crossover Assignment Masking

2012 Clinical Trials

371. Study to Evaluate the Effect of RGMA001 on Patients With Non Alcoholic Fatty Liver Disease (NAFLD)

are pregnant or breast feeding. Platelet count < 90 x 103 / µL (< 90 x 109 /L) at screening. Hemoglobin (Hgb) concentration < 12 g/dL (< 120 g/L) in females or < 13 g/dL (< 30 g/L) in males at screening. Any patient with a baseline increased risk for anemia (e.g., thalassemia, sickle cell anemia, spherocytosis, history of gastrointestinal bleeding) or for whom anemia would be medically problematic. Patients with history of severe psychiatric disease, including psychosis and/or severe depression (...) of the following conditions: (1) poorly controlled hypertension, OR (2) screening or baseline blood pressure ≥ 160 mmHg for systolic OR (3) screening or baseline blood pressure ≥ 100 mmHg for diastolic blood pressure. History of bleeding disorders or anticoagulant use Type I or II diabetes with HbA1C > 8.5% at screening. History or other evidence of chronic pulmonary disease associated with functional limitation. History of severe cardiac disease (e.g. NYHA Functional Class III or IV, myocardial infarction

2012 Clinical Trials

372. Clinical Trial of Group ACYW135 Meningococcal Polysaccharide Vaccine 003

application of antibiotics or anti-virus treatment in the whole body within the past 1 week; History of fever within the past 3 days (axillary temperature ≥38.0℃); Participating in another clinical trial; History of allergy, eclampsia, epilepsy, encephalopathy and mental disease or family disease; Thrombopenia or other coagulopathy that may cause contraindication to intramuscular injection; Acute chronic disease (such as Down syndrome, diabetes, sickle cell anemia or neurologic disease, Guillain-Barre (...) 3 months and vaccination with other products within the last 2 weeks; Axillary temperature ≤37.0℃. Exclusion Criteria: Any acute disease, such as: tumor, autoimmunity disease, progressive atherosclerotic disease or diabetes with complication, chronic obstructive pulmonary disease need oxygen uptake, acute or progressive hepatopathy or nephropathy, congestive heart-failure, etc.; Allergic to vaccines or drugs (history of allergy to any vaccine in the past); History of neurologic symptom or signs

2012 Clinical Trials

373. Clinical Trial of Group ACYW135 Meningococcal Polysaccharide Vaccine 002

of allergy, eclampsia, epilepsy, encephalopathy and mental disease or family disease; Thrombopenia or other coagulopathy that may cause contraindication to intramuscular injection; Acute chronic disease (such as Down syndrome, diabetes, sickle cell anemia or neurologic disease, Guillain-Barre Syndrome); Known or suspected diseases, including: respiratory system disease, acute infection or active stage of chronic disease, SBAV infection of children or mothers, cardiovascular disease, acute hypertension (...) disease or diabetes with complication, chronic obstructive pulmonary disease need oxygen uptake, acute or progressive hepatopathy or nephropathy, congestive heart-failure, etc.; Allergic to vaccines or drugs (history of allergy to any vaccine in the past); History of neurologic symptom or signs; Known or suspected (or high risk) impaired or abnormal immune function, e.g.: receive immunosuppressant or immunopotentiator therapy, take immunoglobulin or blood product or plasma extract (except

2012 Clinical Trials

374. Clinical Trial of Group ACYW135 Meningococcal Polysaccharide Vaccine 001

the past 3 days (axillary temperature ≥38.0℃); Participating in another clinical trial; History of allergy, eclampsia, epilepsy, encephalopathy and mental disease or family disease; Thrombopenia or other coagulopathy that may cause contraindication to intramuscular injection; Acute chronic disease (such as Down syndrome, diabetes, sickle cell anemia or neurologic disease, Guillain-Barre Syndrome); Known or suspected diseases, including: respiratory system disease, acute infection or active stage (...) ℃. Exclusion Criteria: Any acute disease, such as: tumor, autoimmunity disease, progressive atherosclerotic disease or diabetes with complication, chronic obstructive pulmonary disease need oxygen uptake, acute or progressive hepatopathy or nephropathy, congestive heart-failure, etc.; Allergic to vaccines or drugs (history of allergy to any vaccine in the past); History of neurologic symptom or signs; Known or suspected (or high risk) impaired or abnormal immune function, e.g.: receive immunosuppressant

2012 Clinical Trials

375. Trial of HQK-1001 in Beta Thalassemia Intermedia in Lebanon

, an innate type of hemoglobin which is normally made but is suppressed in infancy. Fetal globin (HbF) can perform the function of the missing beta globin and reduce anemia in beta thalassemia, when it is produced in higher amounts than normal. In this trial, 10 patients with beta thalassemia intermedia in Lebanon will all receive the study drug for 6 months at a dose which has been previously shown to be safe in normal volunteers and in beta thalassemia and sickle cell patients and to stimulate fetal (...) Exclusion Criteria: Red blood cell transfusions within 3 months prior to administration of study drug QT Segment corrected (QTc)> 450 msec Use of Erythropoiesis Stimulating Agents(ESAs)within 9 days of first dose Hydroxyurea treatment within 6 months of first study drug History of significant arrythmias, syncope, or resuscitation Alanine Transaminase (ALT)> 4x upper limit of normal Serum creatinine > 1.5 mg/dl Sse of iron chelating agents within 7 days of first dose Pulmonary hypertension requiring

2012 Clinical Trials

376. Oxygen: the poison is in the dose. (PubMed)

Oxygen: the poison is in the dose. Cell-free hemoglobin (Hb) has been blamed for a spectrum of problems, including vasoconstriction pancreatitis, myocardial infarction, and pulmonary hypertension in hemolytic anemia, malaria, and sickle cell anemia, and from Hb-based oxygen carriers (HBOCs). Toxicities have been attributed to scavenging of nitric oxide (NO). However, while NO scavenging may explain many in vitro effects, and some effects in animal models and clinical trials with HBOCs, key (...) inconsistencies in the theory require alternative explanations. This review considers the hypothesis that cell-free Hb oversupplies oxygen to tissues, leading to oxygen-related toxicity, possibly through formation of reactive oxygen species and local destruction of NO. Evidence for this hypothesis comes from various sources, establishing that tissue oxygen levels are maintained over very narrow (and low) levels, even at high oxygen consumption. Tissue is normally protected from excessive oxygen by its

2013 Transfusion

377. Beta-thalassemia and Microparticles

cells and granulocytes. The ability to form microparticles is an essential part of physiological coagulation.However, MP may play an important procoagulant role in several diseases including sickle cell disease, and paroxysmal nocturnal haemoglobinuria (PNH). Several studies reported the presence of MP in TI and their potential role in the hypercoagulable state. The investigators propose in this study to investigate the presence and origin of MP in TM patients. Study Design Go to Layout table (...) , to quantify the elevation of MP as well as their procoagulant activity, to describe their production kinetic, to determine the transfusional or endogenous origin of erythrocytic MP and finally to compare their characteristics with those found in TI patients. To study, in TM and TI patients, the relationship between the number, the procoagulant activity of MP and the clinical (thromboembolic episodes,splenectomy, presence of pulmonary hypertension) biological and plasmatic data reflecting the patient's

2011 Clinical Trials

378. A Study of Tranexamic Acid (XP12B) in Women With Heavy Menstrual Bleeding

and prothrombin mutation. Thalassemia or sickle cell disease (sickle cell trait individuals are not excluded). History or presence of subarachnoid hemorrhage. Use or anticipated use of medications taken to relieve β-Hydroxy β-methylbutyric acid (HMB) including the use of vaginal [rings, creams, gels] and transdermal hormone products; use of oral estrogen-, progestin- or SERM-containing drug products, or intrauterine progestins containing drug products. Use or anticipated use of Lupron (1 or 3 month) depot (...) hypertension, macular degeneration or retinopathies. History or presence of alcoholism or drug abuse within the past year. Malignancy, or treatment for malignancy, within the previous 2 years, with the exception of basal cell carcinomas of the skin or squamous cell carcinoma of the skin. Does not read or understand English. 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

2011 Clinical Trials

379. Choosing Opioid Management for Pain and Analyzing Acute Chest Syndrome (ACS) Rates Equally

for MeSH terms Anemia, Sickle Cell Acute Chest Syndrome Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia Hematologic Diseases Hemoglobinopathies Genetic Diseases, Inborn Lung Diseases Respiratory Tract Diseases Respiration Disorders Morphine Nalbuphine Analgesics, Opioid Narcotics Central Nervous System Depressants Physiological Effects of Drugs Analgesics Sensory System Agents Peripheral Nervous System Agents (...) Posted : February 20, 2018 Sponsor: Children's Healthcare of Atlanta Collaborator: Atlanta Clinical and Translational Science Institute Information provided by (Responsible Party): Saadia Khizer, Children's Healthcare of Atlanta Study Details Study Description Go to Brief Summary: The pathophysiology of sickle cell disease (SCD) manifestations, are complex with interactions of intracellular hemoglobin, membrane and endothelial activation but the hallmark remains recurrent and painful vaso-occlusive

2011 Clinical Trials

380. Topical Sodium Nitrite for Chronic Leg Ulcers in Adult Patients With Blood Disorders

and other hemolytic disorders is unknown, mechanical obstruction by dense sickled red cell, increased venous pressure, bacterial infections, abnormal autonomic control with excessive vasoconstriction when in the dependent position, degree of anemia with decrease in oxygen carrying capacity, and in situ thrombosis, have all been proposed as potential contributing factors. Recent studies have reported increased incidence of leg ulcers in patients with pulmonary hypertension. Our group has pioneered (...) to the leg ulcer and tests performed by the study researchers. For the fourth and final week, participants will return for additional cream treatment sessions, imaging studies, blood draws, and other tests as directed by the study researchers. Study participation will end in the following week (week 5). Subjects will come for a final visit one month after the end of the study. Condition or disease Intervention/treatment Phase Sickle Cell Anemia Sickle Cell Disease Chronic Hemolytic Disorders Drug: Sodium

2011 Clinical Trials

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