Prevalence and Pathophysiology of Systemic Arterial Pressure Abnormalities in Childhood Sickle Cell Disease (DrépaPA)

It is usually found that the blood pressure of adults with sickle cell disease is lower than in non-sickle cell patients. On the other hand, three recent prospective studies in children with sickle cell disease show prevalence of hypertension diagnosed by ambulatory blood pressure measurement (ABPM) ranging from 32 to 45% but on small numbers of patients (n = 54 at most). This hypertension appears to affect kidney function and has been previously associated with the risk of hemorrhagic stroke. It is therefore important to know the prevalence of hypertension in children with sickle cell disease and to determine its mechanisms. The factors which could explain this high prevalence are the increase in arterial stiffness and the increase in systemic vascular resistance linked to the alteration of the sympathovagal balance contributing to the regulation of vascular tone. Indeed, a disturbance of this balance with an increase in vasoconstrictor sympathetic tone has already been found. Hypothesis: In a subgroup of sickle cell children there is systemic hypertension (prevalence: main objective) linked to the alteration of the sympathovagal balance already established during sickle cell disease (increase in sympathetic tone and decrease in parasympathetic tone) affecting systemic vascular resistance (secondary pathophysiological objectives).

Study Overview

• Status: Recruiting
• Conditions: Sickle Cell Disease
• Intervention / Treatment: Diagnostic test: Blood Pressure measurement

Detailed Description

Main objective (200 children): To evaluate the prevalence of elevated blood pressure (former pre-hypertension) and hypertension (including masked hypertension) in children with sickle cell disease. Secondary objectives (60 children): to evaluate the prevalence of loss of nocturnal decrease in blood pressure (dipping); to evaluate arterial stiffness (pulse wave velocity: PWV) and vascular resistance (Augmentation Index, AI) in the different groups: normal, pre-hypertension and hypertension; to evaluate the cardiac sympathovagal balance by studying heart rate variability (HRV) in the three groups: normal, pre-hypertension and hypertension; to evaluate arterial in the three groups: normal, pre-hypertension and hypertension; to evaluate whether the absence of nocturnal dipping is or is not an associated factor of abnormal arterial stiffness, systemic vascular resistance, sympathovagal balance or baroreflex by comparison of subjects with normal dipping versus abnormal dipping.

• Study Type: Observational
• Enrollment (Anticipated): 200

Contacts and Locations

• Study Contact: Name: Christophe DELCLAUX, MD, PhD; Phone Number: +331 40 03 41 90; Email: christophe.delclaux@aphp.fr
• Study Contact Backup: Name: Bérengère KOEHL, MD; Email: berengere.koehl@aphp.fr

Study Locations

• France
  • Paris, France, 75019
    • Recruiting
    • Robert Debre Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

childhood sickle cell disease

Description

Inclusion Criteria:

• child (age <18 years);
• sub-Saharan or Caribbean origin;
• major sickle cell disease (SS, SC and Sbeta-thalassemia);
• height ≥ 120 cm; absence of treated hypertension or antihypertensive treatment;
• parents informed and not opposed to participation in research

Exclusion Criteria:

-

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Cross-Sectional

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Ambulatory Blood Pressure Monitoring (ABPM)	mean, systolic and diastolic arterial pressure	2 years

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Investigators: Principal Investigator: Bérengère KOEHL, MD, Assistance Publique - Hôpitaux de Paris

Study record dates

Study Major Dates

• Study Start (Actual): June 1, 2021
• Primary Completion (Anticipated): June 1, 2023
• Study Completion (Anticipated): June 1, 2023

Study Registration Dates

• First Submitted: May 27, 2021
• First Submitted That Met QC Criteria: May 27, 2021
• First Posted (Actual): June 2, 2021

Study Record Updates

• Last Update Posted (Actual): October 18, 2021
• Last Update Submitted That Met QC Criteria: October 15, 2021
• Last Verified: May 1, 2021

More Information

Terms related to this study

• Keywords: systemic hypertension; sickle cell disease
• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell
• Other Study ID Numbers: APHP201318

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No