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.
* 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.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
BASIC_SCIENCE
Masking
NONE
Enrollment
116
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.
Unité Transversale de la Drépanocytose
Pointe-à-Pitre, Guadeloupe
/ Lipids profiles at steady state, in sickle cell anemia and SC sickle cell adult patients, classified according to occurrence of complications.
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
Time frame: 6 years
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
Past and prospective collection of previously listed SCD complications
Time frame: 6 years
Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels.
total cholesterol
Time frame: 6 years
Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels.
HDL-cholesterol
Time frame: 6 years
Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels.
non HDL-cholesterol
Time frame: 6 years
Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels.
LDL-cholesterol
Time frame: 6 years
Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels.
triglycerides
Time frame: 6 years
Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels.
Apolipoproteins A-I and B
Time frame: 6 years
Description of genetic primary modulators of SCD complications.
Fetal hemoglobin,
Time frame: 6 years
Description of genetic primary modulators of SCD complications.
alpha-thalassemia,
Time frame: 6 years
Description of genetic primary modulators of SCD complications.
haplotypes of beta S gene
Time frame: 6 years
Dosages of Insulin resistance (HOMA),
Plasmatic insulinemia and glycemia (HOMA) will be performed in the entire cohort at inclusion and during prospective complications (VOC, priapism); lipids dosages
Time frame: 6 years
free fatty acids
kinetic study of free fatty acids at inclusion and during prospective complications (VOC, priapism);
Time frame: 6 years
plasmatic glycerol.
Kinetic study of plasmatic at inclusion and during prospective complications (VOC, priapism);
Time frame: 6 years
Dosages of lipids enzymes, lipidome and functionality of HDL at steady state
The dosages of CETP (Cholesteryl Ester Transfer Protein) enzymes activities
Time frame: 6 years
Dosages of lipids enzymes, lipidome and functionality of HDL at steady state
The dosages of L-CAT (Lécithine Cholestérol Acyl Transférase) enzymes activities
Time frame: 6 years
Dosages of lipids enzymes, lipidome and functionality of HDL at steady state
The dosages of HDL lipidome,
Time frame: 6 years
Dosages of lipids enzymes, lipidome and functionality of HDL at steady state
The dosages of HDL functionality,
Time frame: 6 years
Dosages of lipids enzymes, lipidome and functionality of HDL at steady state
The dosages of free fatty acid
Time frame: 6 years
Dosages of lipids enzymes, lipidome and functionality of HDL at steady state
The dosages of glycerol
Time frame: 6 years
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