hereditary blood disease sickle cell disease
Conditions
Interventions
None listed
Sponsors
Academisch Medisch Centrum
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: - sickle cell disease (HbSS or HbS0) - 18 years of age or older - informed consent
Exclusion criteria
Exclusion criteria: * inability of the patient to provide informed consent or legally incompetent/incapacitated to do so * contraindications for MRI, such as pregnancy, claustrophobia or the presence of metal in the body * sickle cell crisis at the moment of participation * history of cerebral pathology that compromises measurements, such as cerebral palsy, brain tumor, meningitis, overt infarct * brain surgery performed in the last 3 months * severe liver, heart or renal dysfunction (clearance
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| cerebral blood flow (CBF) measured by ASL; velocity in the MCA measured by 3D TOF MRA; volume and count of semi-automatically delineated cerebral vessels on 3D TOF MRA; volume and count of SCI on 3D fluid attenuated inverse recovery (FLAIR) and T2-weighted MRI scans; wall shear stress measurements | — |
Secondary
| Measure | Time frame |
|---|---|
| count and volume of SCI on previous scans, if available; hematological whole blood parameters including Hb, reticulocytes, free Hb, HbF, Hct, leukocytes, neutraphil count, platelet count, creatinine, total/direct bilirubine, LDH, ferritine; hematological inflammatory parameters including hsCRP, pentraxine-3, AGEs (pentosidine and N*-(carboxy-methyl) lysine, CML), ADMA, von Willebrand factor antigen, sP-selectine, sE-selectine, VCAM-1, ICAM-1, VEGF and nucleosomes; heartbeat frequency; complication frequency. | — |
Countries
Netherlands
Outcome results
None listed