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| ID | Type | Description | Link |
|---|---|---|---|
| 2023-A02784-41 | Registry Identifier | IDRCB |
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| Name | Class |
|---|---|
| Inserm U955 | OTHER |
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Evaluation of AHSP concentration in total blood as a biomarker in adult and pediatric sickle cell patients
Research involving non-interventional humans. Selection and inclusion of patients by CHSD investigators. Collection of clinical data by investigators and CHSD URC staff.
Biochemical and hematological measurements by the CHSD medical biology laboratory for "care" samples Pseudonymization of 4 mL "research" samples by the URC then fractionation of the samples by 1 mL, and storage at -80°C within the medical biology laboratory of the CHSD.
Transport of samples at -80°C to team 1 of Dr Baudin-Creuza (Créteil). Preparation of genomic DNA from a 1 mL fraction then α and β globin genotyping by Dr Pissard.
Measurement of the AHSP concentration from the other fractions. Comparison of the AHSP concentration according to the group of subjects, and with the different parameters, then correlation analysis
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| sickle cell patients over 15 and a half years old | Experimental | sickle cell patients over 15 and a half years old with SS or Sβ0 phenotype |
|
| control patients over 15 and a half years old | Other | control patients over 15 and a half years old without hemoglobin abnormalities, recruited at the hospital |
|
| pediatric sickle cell patients (aged between 3 and 15 and a half years) | Experimental | pediatric sickle cell patients (aged between 3 and 15 and a half years) with SS or Sβ0 phenotype |
|
| pediatric control patients (aged between 3 and 15 and a half years) | Other | pediatric control patients (aged between 3 and 15 and a half years) without hemoglobin abnormalities, recruited at the hospital |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Measurement of AHSP concentration | Biological | Pseudonymization and identification of research samples EDTA tube of 4 mL for patients over 15 and a half years old, and 2 EDTA tubes of 2 mL for children aged 3 to 15 and a half years, totaling 4 mL) by Team 4 according to the procedure in force at the URC. The 4 mL research sample will then be divided into 1 mL fractions by a technician in the laboratory and stored at -80°C in the medical biology laboratory. |
| Measure | Description | Time Frame |
|---|---|---|
| AHSP concentrations measured in patients | Comparison of AHSP concentrations measured in total blood between: Those of adult sickle cell patients and those of adult control patients without sickle cell disease Those of pediatric sickle cell patients and those of pediatric control patients without sickle cell disease | 6 MONTH |
| Measure | Description | Time Frame |
|---|---|---|
| Correlation analysis | Correlation analysis between the AHSP parameter and the clinical-biological, therapeutic, and genetic parameters of adult and pediatric sickle cell patients. | 6 MONTH |
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Inclusion Criteria:
Inclusion Criteria for Sickle Cell Patients
Inclusion Criteria for Control Patients.
1 -Adults: > 15 years and 6 months /Pediatrics: ≥ 3 years and ≤ 15 years and 6 months 2-Absence of Hemoglobinopathy 3-Follow-up for one of the following conditions (adults): Evaluation of hematological disease excluding hemoglobinopathy, evaluation of prolonged fever or inflammatory syndrome, initial or episodic evaluation of an auto-inflammatory disease or systemic disease, general health deterioration
-Follow-up for one of the following conditions (pediatrics): Suspected precocious puberty, growth delay, or neurodevelopmental disorder
4-Blood sample planned as part of medical care
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Stéphanie NGO, Coordinating Investigator | Contact | stephanie.ngo@ch-stdenis.fr | 01 42 35 60 | stephanie.ngo@ch-stdenis.fr |
| Stéhanie COSSEC, Project Coordinator | Contact | stephanie.cossec@ghtpdfr.fr | 01.42.35.61.40 | stephanie.cossec@ghtpdfr.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Centre Hospitalier de Saint-Denis | Recruiting | Saint-Denis | 93200 | France |
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| Label | URL |
|---|---|
| Decreased sickle red blood cell adhesion to laminin by hydroxyurea is associated with inhibition of Lu/BCAM protein phosphorylation | View source |
| F. Clinical management of adult sickle-cell disease. Curr Opin Hematol | View source |
| 2D DIGE based proteomics study of erythrocyte cytosol in sickle cell disease: altered proteostasis and oxidative stress |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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30 sickle cell patients over 15 and a half years old with SS or Sβ0 phenotype 30 control patients over 15 and a half years old without hemoglobin abnormalities, recruited at the hospital 20 pediatric sickle cell patients (aged between 3 and 15 and a half years) with SS or Sβ0 phenotype 20 pediatric control patients (aged between 3 and 15 and a half years) without hemoglobin abnormalities, recruited at the hospital
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|
| View source |
| Alpha-hemoglobin stabilizing protein (AHSP), a kinetic scheme of the action of a human mutant, AHSPV56G | View source |
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |