Complication, Dyslipidemia, Sickle Cell Disease
Conditions
Keywords
Sickle cell disease, lipids, vasoocclusive crisis, priapism, pulmonary arterial hypertension
Brief summary
This study aims to describe and/or searches for, in cohorts of adult sickle cell anemia (SCA) and SC sickle cell patients living in the French West Indies and followed by SCD Reference and Competence Centers: 1-lipids profiles and associations at steady state with occurrence of sickle cell disease (SCD) complications, 2-lipids profile evolution during and after prospective acute complications (vasoocclusive crises (VOC) and priapism), 3-lipids profile variation (inter /intra individuals) during 4 prospective years, 4- Genetic primary modulators of SCD complications, 5- insulin resistance (HOMA), free fatty acids and glycerol dosages, 6- lipids enzymes, lipidome and functionality of HDL in sub-groups of SCD population.
Detailed description
* Cohorts of sickle cell disease patients including sickle cell anemia (SCA) and SC sickle cell patients living in Guadeloupe and Martinique and followed by the Sickle cell disease (SCD) Reference and Competence Centers of French West Indies. * Lipid profile includes total cholesterol, HDL-cholesterol, non-HDL-cholesterol, LDL-cholesterol and triglycerides, apolipoprotein A-I and B. Medical histories and prospective collection of SCD complications include retinopathy, deafness, tinnitus, osteonecrosis, leg ulcers, strokes, acute chest syndrome, VOC, priapism, pulmonary arterial hypertension (PAH) and PAH sd (echocardiography diagnosed when tricuspid regurgitant jet velocity ≥2.5 m/sec), kidney disease: chronic renal insufficiency and/or nephropathy. * Objective 4: to describe genetic primary modulators of SCD complications: fetal hemoglobin, alpha-thalassemia, haplotypes of beta S gene. * Objective 5 will be performed in the entire cohort at inclusion and during prospective complications (VOC, priapism). * Objective 6 will be performed in a sub-group of 90 individuals (n=15 with VOC and n= 15 without VOC, n=15 with priapism and n=15 without priapism, n= 15 with pulmonary arterial hypertension syndrome (PAH Sd) and n=15 without PAH Sd), as well as in a subgroup of n = 15 patients prospectively experiencing VOC and n = 15 patients prospectively experiencing priapism. A collection of plasma is performed to fulfill objective 6, as well as a collection of blood cells for later researches.
Interventions
OTHERHDL2
to perform additional blood samples during acute phase of complications (realized between Day 1 and Day 3) in SCD patients hospitalized for vasoocclusive crisis or priapism.
Sponsors
Direction Générale de l'Offre de Soins
Centre Hospitalier Universitaire de la Guadeloupe
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
BASIC_SCIENCE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Aged from 18 years and over * Be affected with Sickle cell anemia or SC sickle cell * Living in French Caribbean Islands of Guadeloupe or Martinique and followed by physicians issued from a French West Indies Sickle Cell Reference or Competence Center * At steady state in the last month (without acute complication) * To have given a written consent after information on the study.
Exclusion criteria
* Other hemoglobinopathies than sickle cell disease * Pregnancy or lactation * Patient under judicial protection or without freedom * Patient not affiliated with a social security system * Patient hospitalized for transfusion or bleeding in the last 3 months
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| / Lipids profiles at steady state, in sickle cell anemia and SC sickle cell adult patients, classified according to occurrence of complications. | 6 years | Cohorts of sickle cell disease patients include sickle cell anemia (SCA) and SC sickle cell patients living in Guadeloupe and Martinique and followed by the Sickle cell disease (SCD) Reference and Competence Centers of French West Indies. Lipid profile includes total cholesterol, HDL-cholesterol, non-HDL-cholesterol, LDL-cholesterol and triglycerides, apolipoproteins A-I and B. Collection of medical histories and of prospective SCD complications include retinopathy, deafness, tinnitus, osteonecrosis, leg ulcers, strokes, acute chest syndrome, VOC, priapism, pulmonary arterial hypertension (PAH) and PAH sd (echocardiography diagnosed when tricuspid regurgitant jet velocity ≥2.5 m/sec), kidney disease: chronic renal insufficiency and/or nephropathy |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels. | 6 years | total cholesterol |
| Description of genetic primary modulators of SCD complications. | 6 years | Fetal hemoglobin, |
| Dosages of Insulin resistance (HOMA), | 6 years | Plasmatic insulinemia and glycemia (HOMA) will be performed in the entire cohort at inclusion and during prospective complications (VOC, priapism); lipids dosages |
| Kinetic study of lipids profile during hospitalized vasoocclusive crisis (VOC, with or without ACS) and Priapism, at return to steady state at first annual check-up, and one year after this last measurement | 6 years | Past and prospective collection of previously listed SCD complications |
| plasmatic glycerol. | 6 years | Kinetic study of plasmatic at inclusion and during prospective complications (VOC, priapism); |
| Dosages of lipids enzymes, lipidome and functionality of HDL at steady state | 6 years | The dosages of CETP (Cholesteryl Ester Transfer Protein) enzymes activities |
| free fatty acids | 6 years | kinetic study of free fatty acids at inclusion and during prospective complications (VOC, priapism); |
Countries
Guadeloupe
Outcome results
None listed