Cerebrovascular reserve and white matter disease in patients with chronic anemia. (IMPROvE)

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 140

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 (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 < 10 mL/min)
• Allergy to sulphonamide
• Pregnant or breastfeeding
• Use of phenytoin, procaine or acetylsacylic acid (*Ascal/aspirin*)
• Risk of hypokalaemia (use of diuretics, primary hyperaldosteronism)
• Addison*s Disease
• Severe asthma or emphysema ;Exclusion criteria (controls)
• Any known chronic illness that may compromise subject safety or data integrity. These include but are not limited to rheumatologic disorders, malignancy, severe asthma, chronic hepatic or renal insufficiency, and haemoglobinopathy or other chronic anaemia
• Vascular risk factors
• Hypercholesterolemia
• Contraindications for MRI, such as claustrophobia or the presence of metal in the body
• Contraindications for ACZ (listed in the exclusion criteria of patients)
• Developmental delay, stroke, seizure disorder, or neurological conditions other than simple migraine
• Inability to cooperate with MRI examinations
• Diabetes
• Uncontrolled hypertension or history of hypertension

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>CBF measured by ASL; number and volume of silent cerebral infarcts (SCIs) on 3D<br /><br>fluid attenuated inverse recovery (FLAIR) and T2-weighted MRI scans; brain<br /><br>volume by T1 anatomical MPRAGE; CVR measured by ASL using ACZ.</p><br>

Secondary Outcome Measures