Thalassemia pulmonary hypertension thalassemia pulmonary hypertension inhaled nitrite
Conditions
Interventions
Sodium nitrite solution administered by inhaled nebulization to thalassemia patients,Sildenafil administered orally for 6 weeks
Experimental Drug,Experimental Drug
Nebulized sodium nitrite in thalassemia patients,Sildenafil
Sponsors
Mahidol University
Faculty of Science,
Mahidol University
Eligibility
Sex/Gender
All
Age
18 Years to 60 Years
Inclusion criteria
Inclusion criteria: beta-thalassemia/HbE Elevated TRV (> 2.5 m/s) 18 years or older Hb concentration at least 7 g/dl MetHb not over 5% Normal systemic blood pressure No other underlying diseases No smoking, alcohol and drug abuse Willing to join the study Willing to provide written inform consent
Exclusion criteria
Exclusion criteria: Age 160/100 mmHg) Has a history of portal hypertension or chronic liver disease History of orthostatic hypotension or at the time of screening G6PD deficiency or any contraindication to receiving methylene blue Is currently pregnant or breastfeeding or intends to become pregnant Has severe or chronic other diseases (heart failure, myocardial infraction, COPD, DM, thyroid, renal insufficiency, and malignancy) Known or suspected hypersensitivity or allergic to sodium nitrite or sodium nitrate Want to drop out of the study Reluctant to provide written inform consent
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change in pulmonary arterial pressure During and 0-30 min after nebulization Right heart catherization,Change in pulmonary arterial pressure During and 0-30 min after nebulization Echocardiography | — |
Secondary
| Measure | Time frame |
|---|---|
| Change in platelet activity from times zero, at time 5, 30 and 60 min after nebulization Flow cytometry,Change in systolic blood pressure, heart rate, cardiac output from time zero, at times 5, 30 and 60 min after nebulization Echocardiography and blood pressure measurement,Change in methemoglobin in venous blood from time zero and at times 120 min after nebulization Spectrophotometry,Change in concentration of nitric oxide derivatives in blood from time zero, at times 5, 10, 25, 40, 60, 90, and 120 min after nebulization Chemiluminescence,Renal clearance of nitrite from time zero to 120 min after nebulization Urinary nitrite by chemiluminescence,Change in exhaled NO (FENO) from time zero, at times 5, 30, 45, and 60 min after nebulization Chemiluminescence,Phosphorylated vasodilator-stimulated phosphoprotein in platelets 0, 5, 10, 15 and 60 min after nebulization Western blot,NADPH-oxidase in platelets 0, 5, 10, 15 and 60 min after nebulization Flow cytometry,Hemoglobin-platelet binding 0, 5, 10, 15 and 60 min after nebulization Flow cytometry | — |
Countries
Thailand
Contacts
Public ContactNathawut Sibmooh
Faculty of Science, Mahidol University
Outcome results
None listed