Heart Arteries and Sickle Cell Disease / Coeur Artères DREpanocytose (CADRE)

Cardiologie et Développement

Status: Unknown

Conditions: Sickle Cell Anemia; Sickle Cell Disease

Study type: Observational

Funder types: Other

Identifiers

NCT03114137

002

Details and patient eligibility

About

The CADRE study is a multinational observational cohort of patients with sickle-cell disease (SCD) in five west and central sub-Saharan African countries. The aim of this project is to describe the incidence and assess the predictive factors of SCD-related micro- and macro-vascular complications in sub-Saharan Africa.

Full description

Sickle cell disease (SCD), one of the lost common genetic diseases worldwide, is caused by a mutation in the β globin gene. Most patients with this disease are homozygous for the βS allele (SS), whereas others have inherited a βS allele with another mutation in the β globin gene. In addition to repeated acute ischemic insults due to the red blood cells sickling in the microcirculation, a chronic vasculopathy leads to organ injuries, such as kidney disease, stroke, pulmonary hypertension, retinopathy, bone infarcts, and leg ulcers.

CADRE is a multinational prospective observational study undertaken in five countries in sub-Saharan Africa. Patients with SCD will be recruited through outpatients' clinics in public, university and private hospitals and research centers in five countries. The CADRE protocol was approved by the relevant national ethics committee in each of the participating countries.

Primary endpoint is to measure the prevalence and the incidence of the main vascular complications in the main types of SCD: glomerulopathy, nephropathy, cardiopathy, pulmonary hypertension, retinopathy, strokes, osteonecrosis and leg ulcers.

Secondary endpoints are:

• to define the clinical and biological predictors of SCD vasculopathy in Africa
• to search for genetic risk factors for the SCD-related cardiovascular complications, in particular alpha thalassemia, persistence of foetal hemoglobin and other candidate genetic polymorphisms
• to search for functional risk factors (pulse wave velocity, capillary vasodilatation, blood visosity) for the SCD-related cardiovascular complications
• to search for new biological determinant of SCD-related cardiovascular complications, in particular alternative markers of hemolysis (microparticules, free heme) and inflammation (cytokines, leucocytes phenotyping, NET (neutrophile extracellular traps))

Enrollment: 4,500 estimated patients

Sex: All

Ages: 5+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

• age: five-year-old or more
• signature of informed consent Patients : major sickle cell syndrome confirmed by hemoglobin phenotyping: SS, SC, SBeta+ or Sbeta0 Controls : healthy parents or siblings of the patients, hospital staff or their children, matched on age+/- 3 years and country (1 control for 4 patients)

Exclusion criteria

unstable clinical status such as:

• vaso-occlusive crisis in the previous 15 days
• fever or infectious disease in the previous 15 days
• transfusion in the previous 2 months

Trial design

4,500 participants in 2 patient groups

sickle cell patients

Description:

* age: five-year-old or more * major sickle cell syndrome confirmed by hemoglobin phenotype: SS, SC, SBeta+ or Sbeta0 * steady state defined as the absence of vaso-occlusive crisis for the previous 15 days, absence of fever or infectious disease for the previous 8 days and absence of transfusion for the previous 2 months

control patients

Description:

* volunteer parents or siblings of sickle cell patients * hospital staff or their children matched on country and age +/- 3 ans with the patients