sickle cell disease (anemia), hypoxia-reperfusion injury, endothelial damage, oxidative stress, NO-availability, inflammation, sikkelcelziekte, chronische ontsteking, endotheelschade.
Conditions
Interventions
N-acetylcysteine 1200 mg or 2400 mg a day.
Sponsors
This project will be carried out by the CURAMA programme, which is a collaborative effort between the Department of Vascular Medicine, Academic Medical Center (Amsterdam, the Netherlands), the Department of Internal Medicine Slotervaart Hospital (Amsterdam, the Netherlands), the Department of Internal Medicine, Sint Elisabeth Hospital (Curaçao, Netherlands Antilles), Red Cross Bloodbank Foundation Curaçao (Curaçao, Netherlands Antilles), the Laboratory of Clinical Thrombosis and Hemostasis in th
Eligibility
Inclusion criteria
Inclusion criteria: 1. High performance liquid chromatography confirmed diagnosis of HbSS, HbSC or HbSâ genotype . 2. Aged 18-65 years 3. Written informed consent
Exclusion criteria
Exclusion criteria: 1. Bloodtransfusion in the preceding four months. 2. Pregnancy or the desire to get pregnant in the following 7 months. 3. Concommitant use of hydroxyurea, vitamin K antagonists or other oral anticoagulants, or contraindications for NAC. 4. Impaired renal function of more than 60% (as assessed by the Kockroft-Gauld equation) 5. Known gatsric or duodenal ulcer 6. Concomittant use of anti-hypertensives, sildefanil or nitrates.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary end-points are the effects of NAC on the laboratory markers (hemoglobin, red blood cell counts, reticulocyte counts, leukocyte counts and differentiation, platelet counts, erythrocyte sedimentation rate, a blood smear will be analyzed microscopically for the number of ISC per field, as well as the number of Heinz bodies, intra-erythrocytic GSH and GSSG levels, NO availability, SRBC phosphatidylserine (PS) exposure, annexin V, creatinine, BUN, electrolytes, transaminase levels, albumin levels, LDH, indirect bilirubin levels, free hemoglobin levels, high sensitive CRP, sVCAM-1, ET-1, IL-8, pro-thrombin fragments (F1.2), D-dimer levels, protein S (free and total) and C activity, vWF-Ag activity). | — |
Secondary
| Measure | Time frame |
|---|---|
| Tolerability of study medication (in this phase admittedly in a non-controlled fashion) at every visit by history taking and by scoring of a NAC for SCD check-list. | — |
Contacts
Public ContactB.J. Biemond
Academic Medical Center (AMC), Department of Clinical Chemistry, P.O. Box 22660
Outcome results
None listed