Structural and functional changes in arteries are increasingly being recognized as significant features of sickle cell disease. This study aims to determine whether there are differences in arterial function parameters between children with sickle cell disease with normal and abnormal transcranial Doppler velocity. After informed consent is obtained, participants will have vascular, Transcranial Doppler, haematological and biochemical parameters measured. Researchers will compare children with sickle cell disease who have normal Transcranial Doppler velocity and no history of stroke with children with those who have an abnormal Transcranial Doppler velocity with or without a history of stroke to see if there are significant differences in arterial function parameters.
Pediatric participants with sickle cell anaemia (HbSS, HbSβ0 thalassemia, HbSD, HbSOArab)
Age: Between 4-16 years of age, at the time of enrolment
Has had at least one complete TCD study whether or not on hydroxyurea treatment or in a clinical trial.
Parent or guardian willing and able to provide informed consent and child gives assent
Patients in whom a TCD study cannot be completed
Patients who have had an Erythrocyte transfusion in the past two months
Patients who are acutely ill or have had an acute infection in the past two weeks
Pediatric participants with sickle cell anemia (HbSS, HbSβ0 thalassemia, HbSD, HbSOArab) Age 4-16 years with or without a previous stroke with at least one previous transcranial Doppler study.
Other
Cross-sectional
Not listed
Mean difference in aortic pulse wave velocity in mmHg between patients with an abnormal transcranial Doppler Velocity compared to those with a normal Transcranial Doppler velocity without stroke
The primary outcome of aortic pulse wave velocity in mmHg, will be measured in all patients with an Arteriograph 24 (TensioMed, Budapest, Hungary). Before the assessment, the patient will be allowed to rest for 5 -10 minutes. An appropriate cuff size will be used for each patient. The test will be carried out in a calm, temperature-controlled room.
Mean difference in aortic augmentation index, in % between patients with an abnormal transcranial Doppler Velocity compared to those with a normal Transcranial Doppler velocity without stroke.
The primary outcome of aortic augmentation index, in % will be measured in all patients with an Arteriograph 24 (TensioMed, Budapest, Hungary). Before the assessment, the patient will be allowed to rest for 5 -10 minutes. An appropriate cuff size will be used for each patient. The test will be carried out in a calm, temperature-controlled room.
Mean difference in brachial augmentation index, in %, between patients with an abnormal transcranial Doppler Velocity compared to those with a normal Transcranial Doppler velocity without stroke
Brachial augmentation index, in %, will be measured in all patients with an Arteriograph 24 (TensioMed, Budapest, Hungary). Before the assessment, the patient will be allowed to rest for 5 -10 minutes. An appropriate cuff size will be used for each patient. The test will be carried out in a calm, temperature-controlled room.
Mean difference in aortic systolic blood pressure in mmHg between patients with an abnormal transcranial Doppler Velocity compared to those with a normal Transcranial Doppler velocity without stroke
Aortic systolic blood pressure in mmHg, will be measured in all patients with an Arteriograph 24 (TensioMed, Budapest, Hungary). Before the assessment, the patient will be allowed to rest for 5 -10 minutes. An appropriate cuff size will be used for each patient. The test will be carried out in a calm, temperature-controlled room.
Mean difference in aortic pulse pressure, in mmHg between patients with an abnormal transcranial Doppler Velocity compared to those with a normal Transcranial Doppler velocity without stroke
Aortic pulse pressure in mmHg, will be measured in all patients with an Arteriograph 24 (TensioMed, Budapest, Hungary). Before the assessment, the patient will be allowed to rest for 5 -10 minutes. An appropriate cuff size will be used for each patient. The test will be carried out in a calm, temperature-controlled room.
Mean difference in brachial systolic pressure in mmHg, between patients with an abnormal transcranial Doppler Velocity compared to those with a normal Transcranial Doppler velocity without stroke
Brachial systolic pressure in mmHg will be measured in all patients with an Arteriograph 24 (TensioMed, Budapest, Hungary). Before the assessment, the patient will be allowed to rest for 5 -10 minutes. An appropriate cuff size will be used for each patient. The test will be carried out in a calm, temperature-controlled room.
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