Glutamine Therapy for Hemolysis-Associated Pulmonary Hypertension
Pulmonary HypertensionSickle Cell Disease1 moreThe 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 will increase arginine bioavailability and subsequently alter sickle red cell endothelial interaction that can be identified using endo-PAT technology through nitric oxide (NO) generation, leading to changes in biological markers, and clinical outcome. Specifically our second hypothesis is that oral glutamine will decrease biomarkers of hemolysis and adhesion molecules, and improve the imbalanced arginine-to-ornithine ratio that occurs in hemolytic anemias, leading to improved arginine bioavailability and clinical endpoints of endothelial dysfunction and PH in patients with SCD and Thal.
Parvovirus B19 Infection in Hereditary Hemolytic Anemias Patients
Parvovirus B19 InfectionAlthough many studies investigated the prevalence and manifestations of HPV-B19 infection in patients with sickle cell anemia (SCA), thalassemia, and hereditary spherocytosis (HS) separately, there is limited information about the extent to which HPV-B19 infection leads to severe complications and chronic infection.
Phase II Study of High-Dose Cyclophosphamide in Patients With Severe Autoimmune Hematologic Disease...
AnemiaHemolytic5 moreOBJECTIVES: I. Determine the response rate and 1-year event-free survival in patients with severe autoimmune hematologic disease treated with high-dose cyclophosphamide.
Lavare Cycle in Patients Receiving HeartWare Left Ventricular Assist Device
ThromboembolismHemolysis1 moreLeft ventricular assist device (LVAD) patients remain at risk for pump thrombus and thromboembolic events through multiple mechanisms. The HeartWare® Ventricular Assist System (HVAD®, HeartWare Inc., Framingham, MA, USA) includes a novel speed modulation feature called Lavare™ cycle. The Lavare™ Cycle is aimed to promote washing of left ventricle to decrease blood stasis and subsequent risk of thrombus formation, ingestion and/or expulsion. No prior study has prospectively evaluated the impact of Lavare™ cycle on patient outcomes in a randomized fashion. We intend to assess effects of Lavare™ Cycle among patients receiving HVAD LVAD in this randomized controlled pilot project.
ALXN1830 in Patients With Warm Autoimmune Hemolytic Anemia
Warm Autoimmune Hemolytic AnemiaThe main objective of the study is to evaluate the safety and efficacy of ALXN1830 compared to placebo in adult participants with warm autoimmune hemolytic anemia (WAIHA).
Carbon Monoxide Monitor for the Measurement of End-Tidal Carbon Monoxide Levels in Children With...
Sickle Cell AnemiaPeople who have Sickle Cell Anemia (HbSS) produce red blood cells with shorter lifespans. These red blood cells breakdown faster, and this is called hemolysis. When red blood cells breakdown, a tiny amount of Carbon Monoxide (CO) is released into the blood and is eliminated in exhaled breath. This research study will use a device called CoSense™, which will measure Carbon Monoxide (CO) levels in breath. The purpose of the study is to see how well the device measures the CO levels that an individual breathes out.
The Plasma Large-Volume Exchange RCT
PurpuraThrombotic Thrombocytopenic1 moreThrombotic thrombocytopenia purpura / hemolytic uremic syndrome (TTP/HUS) is a rare, life-threatening disorder. TTP/HUS causes multiple blood clots to form, which prevents blood from reaching the brain and kidneys. TTP/HUS affects 3-5 people per million per year. Anyone can develop TTP/HUS, but it is most common among 30-40 year olds, and women are twice as likely as men to acquire the condition. TTP/HUS sometimes develops as a result of medication use, pregnancy or cancer; however, for the majority of patients (80%) the cause of TTP/HUS is unknown. In 1991, researchers discovered that plasma exchange was superior to plasma infusion in treating idiopathic TTP/HUS. During plasma exchange the patient's blood plasma is removed and replaced with healthy blood plasma. Without plasma exchange, the survival rate for TTP/HUS is extremely low, with fewer than 5% of patients surviving. Treating TTP/HUS with plasma exchange improved the survival rate to 80%. Although this represents a dramatic improvement, researchers are still searching for methods to improve survival. No major advances in treating TTP/HUS have occurred in the past 20 years. Recent research suggests that high-dose plasma exchange may improve the survival of TTP/HUS patients. The investigators will conduct a randomized controlled trial to test whether treating TTP/HUS patients with high-dose versus standard-dose plasma exchange improves the treatment response. The investigators will recruit 150 patients with TTP/HUS from 9 centres across Canada over three years. The investigators will evaluate whether high-dose plasma exchange improves the treatment response, survival, and whether it reduces the number and volume of plasma exchange procedures and duration of hospital stay.
A Study of an Investigational Drug, Cemdisiran (ALN-CC5), in Patients With Atypical Hemolytic Uremic...
Atypical Hemolytic Uremic SyndromeThe purpose of this study is to evaluate the safety, tolerability and pharmacokinetics of Cemdisiran in patients with aHUS.
Effect of Triflow Breathing Training
Hemodialysis ComplicationHaemolyses and Related ConditionsThis study aims to explore whether an 8-week Triflow breath training program reduced the anxiety levels and improved the heart rate variability and quality of life of patients receiving hemodialysis. To investigate the effect of the triflow respiration training on the improvement of heart rate variability, anxiety, and the quality of life in hemodialysis patients.
Use of Inhaled Nitric Oxide to Prevent Pulmonary Hypertension Associated to Stored Blood Transfusion...
HypertensionPulmonary1 moreThe objective of this study is to assess if prolonged storage time of a packed red blood cell unit may cause pulmonary vasoconstriction after transfusion, in a susceptible population such as cardiac surgery patients. The investigators will also evaluate the potential reversal effect of Inhaled Nitric Oxide on pulmonary vasoconstriction induced by stored blood transfusions.