Arterial Function Parameters and Transcranial Doppler Velocity in Paediatric Patients With Sickle Cell Disease

The Relationship of Transcranial Doppler Velocity With Other Vascular Measurements in Children With Sickle Cell Anaemia

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.

Structural and functional changes in arteries are increasingly being recognized as significant features of sickle cell disease and arterial function in sickle cell anaemia (SCA) is gaining importance.

The goal of this observational study is to compare arterial parameters in children with sickle cell disease who have normal or abnormal Transcranial Doppler velocity. The main question it aims to answer is: whether there is a significant difference in arterial Function parameters measured by aortic pulse wave velocity, augmentation index, brachial and central blood pressure in Jamaican children with Sickle Cell anaemia who attend the Sickle Cell Unit in Kingston Jamaica who are reported to have normal or an abnormal Transcranial Doppler velocity and whether the probability of having an abnormal Transcranial Doppler velocity or higher arterial function parameters is increased by specific biophysical markers.

Participants who are identified will be informed about the study and potential risks. All patients giving written informed consent will then undergo arterial function (arteriograph,TensioMed® Arteriograph24™,Budapest, H-1181 Hungary), Transcranial Doppler, haematological and biochemical measurements.

Researchers will compare children with sickle cell disease who have normal Transcranial Doppler velocity and no history of stroke with children with this illness who have an abnormal Transcranial Doppler velocity to see if there are significant differences in regional arterial function.

Inclusion Criteria:

1. Pediatric participants with sickle cell anaemia (HbSS, HbSβ0 thalassemia, HbSD, HbSOArab)
2. Age: Between 4-16 years of age, at the time of enrolment
3. Has had at least one complete TCD study whether or not on hydroxyurea treatment or in a clinical trial.
4. Parent or guardian willing and able to provide informed consent and child gives assent
5. Ability to comply with study-related evaluations.

Exclusion Criteria:

• Participants who meet any of the following criteria are disqualified from enrollment in the study:

  1. Patients in whom a TCD study cannot be completed
  2. Patients who have had an Erythrocyte transfusion in the past two months
  3. Patients who are acutely ill or have had an acute infection in the past two weeks