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The objective of the study is to refine our knowledge on the physiopathology of the symptoms and the complications for the patients affected by a drepanocytic syndrome.
The establishment of risk factors and indicators of severity will allow to target better the patients requiring an adequate strategy in order to prevent the installation of some complications or to limit their worsening.
Some additional tubes will be taken during the usual control of blood test of the drepanocytic patient. A sample of urine will be also asked. Tubes, after pre-treatment, will be sent to Erasme hospital.
A series ob biological but also genetic parameters, both at asymptomatic patients and those in aigüe phase of the disease, can be measured either immediately or a little time after the prelevement.
In this way, we can study numerous domains linked to the physiopathology of the drepanocytose (hémolyse, vaso-occlusion, rheology, factors modulators of the clinical expression). The surplus of the collection could be used for other researchs. It's in this context that we also wish to constitute a biobank of serum, plasma and urine for these drepanocytic patients by surplus of taken material.
The study is realized within the framework of an academic collaboration between institutions. The bank of takings will be located in the reference center of the pathologies of the Red Blood Cell (laboratory of medical chemistry of the erasme hospital).
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| sickle cell syndrome | Inclusions of sickle cell patients aged over 17 years followed regularly in the participating centers. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| sickle cell syndrome | Genetic | Academic Study prospective multicenter observational factors responsible for nephropathy in patients with sickle cell disease followed by Belgium and the Nord-Pas -De- Calais Region and creating a biobank of blood and urine. In the population of patients with SCD followed in all participating centres. Know the prevalence of nephropathy and the relationship between it with their some of their genotypic mutations and clinical phenotype promoting mutated hemoglobin polymerization. Determine the behaviour of dense cells in the basal state and in a hypeosmolaire environment Determine the place of the erythrocyte microparticles as a biomarker of sickle cell nephropathy Studying genes known as risk factor for proteinuria Create a BioBank of samples of sickle cell patients in clinically stable condition for other research purposes. |
| Measure | Description | Time Frame |
|---|---|---|
| Urinary Albumin | Nephropathy Prevalence | each year |
| Measure | Description | Time Frame |
|---|---|---|
| Erythrocyte Microparticles | Sickle cell Nephropathy biomarker | each year |
| Eythrocyte Deformability and Erythrocyte Agregation | Sickle Cell Nephropathy Biomarker |
| Measure | Description | Time Frame |
|---|---|---|
| Urine, Plasma and Serum aliquotes in a biobank | For additional projects | Each year |
Inclusion Criteria:
Exclusion Criteria:
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We will characterize the population of sickle cell patients 17 years and older , followed by Belgium and the Nord-Pas -De- Calais, and in the study through the signing of an inform consent.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Béatrice BG Gulbis, Phd MD | Contact | +32 02 555 34 27 | 3427 | Chimie@erasme.ulb.ac.be |
| Jonathan JB Brauner, Md | Contact | +32 02 555 34 27 | 3427 | Jonathan.Brauner@erasme.ulb.ac.be |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Erasme Hospital | Recruiting | Brussels | 1070 | Belgium |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D007674 | Kidney Diseases |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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| ID | Term |
|---|---|
| D006451 | Hemoglobin, Sickle |
| ID | Term |
|---|---|
| D006455 | Hemoglobins, Abnormal |
| D006454 | Hemoglobins |
| D001798 | Blood Proteins |
| D011506 | Proteins |
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Blood and Urine Samples
|
| each year |
| Hp, ApoL1 and HO-1 gene | Sickle Cell Nephropathy risk factor | first year of inclusion |
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D014570 | Urologic Diseases |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
| D052801 | Male Urogenital Diseases |
| D000602 |
| Amino Acids, Peptides, and Proteins |
| D005914 | Globins |
| D006420 | Hemeproteins |