Lipid Balance in Adult Sickle Cell Patients

Study Overview
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Brief Summary
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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
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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.

Official Title
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Study of Lipid Balance in Adult Sickle Cell SS or SC Patients at Steady State and According to Clinical Phenotypes and During Acute Complications Acronym : HDL2

Conditions
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Sickle Cell Disease, Dyslipidemia, Complication

Intervention / Treatment
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* Other: HDL2


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Basic Science
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Intervention/Treatment |
| --- |
|Other: HDL2|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.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| / 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 | 6 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 | 6 years | 
| Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels. | total cholesterol | 6 years | 
| Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels. | HDL-cholesterol | 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 | 6 years | 
| Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels. | LDL-cholesterol | 6 years | 
| Study of variation of lipid profile, at steady state, during a 4 years period study intra and inter individual levels. | triglycerides | 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 | 6 years | 
| Description of genetic primary modulators of SCD complications. | Fetal hemoglobin, | 6 years | 
| Description of genetic primary modulators of SCD complications. | alpha-thalassemia, | 6 years | 
| Description of genetic primary modulators of SCD complications. | haplotypes of beta S gene | 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 | 6 years | 
| free fatty acids | kinetic study of free fatty acids at inclusion and during prospective complications (VOC, priapism); | 6 years | 
| plasmatic glycerol. | Kinetic study of plasmatic at inclusion and during prospective complications (VOC, priapism); | 6 years | 
| Dosages of lipids enzymes, lipidome and functionality of HDL at steady state | The dosages of CETP (Cholesteryl Ester Transfer Protein) enzymes activities | 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 | 6 years | 
| Dosages of lipids enzymes, lipidome and functionality of HDL at steady state | The dosages of HDL lipidome, | 6 years | 
| Dosages of lipids enzymes, lipidome and functionality of HDL at steady state | The dosages of HDL functionality, | 6 years | 
| Dosages of lipids enzymes, lipidome and functionality of HDL at steady state | The dosages of free fatty acid | 6 years | 
| Dosages of lipids enzymes, lipidome and functionality of HDL at steady state | The dosages of glycerol | 6 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Sickle cell disease, lipids, vasoocclusive crisis, priapism, pulmonary arterial hypertension