Heart Arteries and Sickle Cell Disease / Coeur Artères DREpanocytose
Maneno muhimu
Kikemikali
Maelezo
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))
Tarehe
| Imethibitishwa Mwisho: | 04/30/2018 |
| Iliyowasilishwa Kwanza: | 03/29/2017 |
| Uandikishaji uliokadiriwa Uliwasilishwa: | 04/09/2017 |
| Iliyotumwa Kwanza: | 04/13/2017 |
| Sasisho la Mwisho Liliwasilishwa: | 05/23/2018 |
| Sasisho la Mwisho Lilichapishwa: | 05/24/2018 |
| Tarehe halisi ya kuanza kwa masomo: | 02/29/2012 |
| Tarehe ya Kukamilisha Msingi iliyokadiriwa: | 11/30/2020 |
| Tarehe ya Kukamilisha Utafiti: | 11/30/2022 |
Hali au ugonjwa
Awamu
Vikundi vya Arm
| Mkono | Uingiliaji / matibabu |
|---|---|
| sickle cell patients 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 volunteer parents or siblings of sickle cell patients
hospital staff or their children matched on country and age +/- 3 ans with the patients |
Vigezo vya Kustahiki
| Zama zinazostahiki Kujifunza | 5 Years Kwa 5 Years |
| Jinsia Inastahiki Kujifunza | All |
| Njia ya sampuli | Non-Probability Sample |
| Hupokea Wajitolea wa Afya | Ndio |
| Vigezo | 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 |
Matokeo
Hatua za Matokeo ya Msingi
1. Prevalence and 10 year-incidence of SCD-related vascular complications in different phenotypes of SCD: glomerulopathy [10 years]
2. Prevalence and 10 year-incidence of SCD-related vascular complications in different phenotypes of SCD: cardiopathy [10 years]
3. Prevalence and 10 year-incidence of SCD-related vascular complications in different phenotypes of SCD: pulmonary hypertension [10 years]
4. Prevalence and incidence and the 10 year-incidence of the main SCD-related vascular complications in different phenotypes of SCD: retinopathy [10 years]
5. Prevalence and 10 year-incidence of SCD-related vascular complications in different phenotypes of SCD:stroke [10 years]
6. Prevalence and 10 year-incidence of SCD-related vascular complications in different phenotypes of SCD:osteonecrosis [10 years]
7. Prevalence and 10 year-incidence of SCD-related vascular complications in different phenotypes of SCD: leg ulcers [10 years]
8. Prevalence and 10 year-incidence of SCD-related vascular complications in different phenotypes of SCD: priapism [10 years]
Hatua za Matokeo ya Sekondari
1. Potential biological risk marker measured at baseline and follow up visits: carotid-femoral pulse wave velocity [10 years]
2. Potential biological risk marker measured at baseline and follow up visits: complete blood count [10 years]
3. Potential biological risk marker measured at baseline and follow up visits: LDH level [10 years]
4. Potential biological risk marker measured at baseline and follow up visits: bilirubin level [10 years]
5. Potential biological risk marker measured at baseline and follow up visits: microparticules measure [10 years]
6. Potential biological risk marker measured at baseline and follow up visits: free heme level [10 years]
7. Potential biological risk marker measured at baseline and follow up visits: inflammatory cytokines [10 years]
8. Potential biological risk marker measured at baseline and follow up visits: neutrophil extracellular traps [10 years]
