Sickle Cell Disease - Thalassemia
Conditions
Interventions
None listed
Sponsors
Academisch Medisch Centrum
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: Inclusion criteria (patient groups) • Sickle cell disease • Thalassemia major, thalassemia intermedia, and HbH disease • 18 Years of age or older • Informed consent;Inclusion criteria control group • Either AS or AA haemoglobin, • 18 Years of age or older • Informed consent
Exclusion criteria
Exclusion criteria: Exclusion criteria (patient groups) • Hospitalization in the past month for any reason • Inability of the patient to provide informed consent • Contraindications for MRI, such as claustrophobia or the presence of metal in the body • Sickle cell crisis at the moment of participation up to one month prior to participation • History of cerebral pathology that compromises measurements, such as cerebral palsy, brain tumour, meningitis, overt infarct • Brain surgery performed in the last 3 months ;ACZ contraindications • Severe liver, heart or renal dysfunction (clearance
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| CBF measured by ASL; number and volume of silent cerebral infarcts (SCIs) on 3D fluid attenuated inverse recovery (FLAIR) and T2-weighted MRI scans; brain volume by T1 anatomical MPRAGE; CVR measured by ASL using ACZ. | — |
Countries
Netherlands
Outcome results
None listed