Cerebrovascular reserve and white matter disease in patients with chronic anemia

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 140

Inclusion Criteria

Patient group:
Sickle cell disease,
Thalassemia major, thalassemia intermedia, and HbH disease
18 years of age or older
Informed consent

Control Group:
Either AS or AA haemogobin
18 years of age or older
Informed consent

Exclusion Criteria

Patient group:
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 comprimized measeurements, such as cerebral palsy, brain tumour, meningitis, overt infrarct
Brain surgery performed in the last 3 months

ACZ contraindications
Severe liver, heart of renal dysfunction (clearance <10 mL/min)
Allgergy to sulphonamide
Pregnant or breastfeeding
Use of phenytoin, procaïne or acetylsacylic acid
Risk of hypokalaemia
Addison's disease
Severe asthma or emphysema

Control Group:
Any known chronic illness that may compromise subject safety or data integrity.
Vascular risk factors
Hypercholesterolemia
Contraindications for MRI
Contraindications for ACZ
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: Interventional

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
relationship between CVR, CB, and vascular/inflammatory markers<br>Co-localization of white matter damage and regions of low CVR<br>Global and regional rsponse of CVR to simple trnasfusions, exchange transfusions and hydroxyurea.

Secondary Outcome Measures

Name	Time	Method
Relationship between neurcognitive performance and white matter damage<br>Sex differences in baseline CVR and response to interventions<br>Relationship between haemoglobin genotype and CVR<br>