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Sickle cell disease (SCD) is an inherited haemoglobinopathy disorder caused by mutations in HBB gene with amino-acid substitution on β globin chain. The consequence is synthesis of altered haemoglobin S (HbS) which polymerises in red blood cell (RBC) at deoxygenated state. SCD is associated with chronic haemolytic anaemia, vaso-occlusive crisis (VOC) leading to frequent hospitalisation.
The aim of the study was to to investigate whether a combination of routine laboratory biomarkers of haemolysis could be used to predict VOC development in confirmed SCD patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Sickle cell disease | Confirmed sickle cell disease withHaemoglobin profile was determined by high performance liquid chromatography (HPLC) (Variant II Biorad, California, United States), by capillary electrophoresis on Capillarys 3 Octa® (Kit hydragel hémoglobine Sebia, Lisses, France) and iso-electrofocalisation. Patients were included during injury evaluation in our tertiary centre. |
| |
| Healthy | 25 healthy controls matched on age and gender |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Erythrocytic parameters and thrombin generation assay measurement | Biological | Erythrocytic parameters and thrombin generation assay measurement |
|
| Measure | Description | Time Frame |
|---|---|---|
| Hospitalisation for Vaso-occlusive crisis within one years | Following injury consultation, evaluation of biological markers predicting vaso-occlusive crisis requiring hospitalisation in the year | 1 year |
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Inclusion Criteria:
Exclusion Criteria:
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All patients in the study were diagnosed and treated for SCD at Rouen University Hospital. Patients were included during annual visit in our tertiary centre. Patients with VOC were included less than 24hours after admission to emergency department. All patients treated with hydroxyurea have been treated for at least three years. All patients received a systematic annual visit to determine VOC development in the year. Patients were analyzed in four subgroups based on genotype and clinical status:
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Rouen university Hospital | Recruiting | Rouen | 76000 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 40862241 | Derived | Feugray G, Grall M, Gillardin B, Burdeau J, Ozanne N, Dumesnil C, Fauvel C, Billoir P. Hemoglobin and High-Density Lipoprotein as Biomarker of Left Atrial Dilatation in Sickle Cell Disease. EJHaem. 2025 Aug 22;6(4):e70135. doi: 10.1002/jha2.70135. eCollection 2025 Aug. |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D000098644 | Vaso-Occlusive Crises |
| D019851 | Thrombophilia |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |