Cerebrovascular Reserve measUrements In Sickle cell diseasE, a MRI-based study

Completed

Conditions: hereditary blood disease
sickle cell disease
10018911

Registration Number: NL-OMON46956

Lead Sponsor: Academisch Medisch Centrum

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 60

Inclusion Criteria

- sickle cell disease (HbSS or HbS0)
- 18 years of age or older
- informed consent

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 < 10 mL/min)
* allergy to sulfonamide
* breastfeeding
* use of phenytoin, procain, acetylsacylic acid (*Ascal/aspirine*)
* risk of hypokalemia (use of diuretics, primary hyperaldosteronism)
* Addison*s Disease
* severe asthma or emphysema

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>cerebral blood flow (CBF) measured by ASL; velocity in the MCA measured by 3D<br /><br>TOF MRA; volume and count of semi-automatically delineated cerebral vessels on<br /><br>3D TOF MRA; volume and count of SCI on 3D fluid attenuated inverse recovery<br /><br>(FLAIR) and T2-weighted MRI scans; wall shear stress measurements</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>count and volume of SCI on previous scans, if available; hematological whole<br /><br>blood parameters including Hb, reticulocytes, free Hb, HbF, Hct, leukocytes,<br /><br>neutraphil count, platelet count, creatinine, total/direct bilirubine, LDH,<br /><br>ferritine; hematological inflammatory parameters including hsCRP, pentraxine-3,<br /><br>AGEs (pentosidine and N*-(carboxy-methyl) lysine, CML), ADMA, von Willebrand<br /><br>factor antigen, sP-selectine, sE-selectine, VCAM-1, ICAM-1, VEGF and<br /><br>nucleosomes; heartbeat frequency; complication frequency.</p><br>