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G6PD deficiency may have a subtle effect on the severity of hemolysis and also worsen the degree of anaemia in SCD when the two disorders coexist. Therefore, selective decision should be taken in patients in whom the two conditions coexist in the choice of drug and in the treatment of infections.The prevalence of the G-6-PD deficiency is high in SCD patients, but does not differ from that observed among non-SCD subjects .However, the G-6-PD deficiency appears to worsen the clinical features of SCD, there were more hospitalizations, major vaso-occlusive crises among G-6-PD deficient sickle cell patients.
Sickle cell disease (SCD) is not frequent in Egypt except in the Oases where the carrier rate varies from 9 to 22%. It is a hereditary blood disorder characterized by the production of abnormal hemoglobin molecules that cause red blood cells to take on a crescent or sickle shape. This haemoglobin called haemoglobin S, which causes red blood cells to become stiff and sticky, leading to various health complications as recurrent pain, fatigue, anaemia, and increased infection susceptibility.prevalence of G6PD deficiency is 4.3% with a male:female ratio of 3.2:1. Enzyme activity was significantly higher in males than females. It is located on X chromosome which leads to a lower level of reduced glutathione, an antioxidant, in red blood cells (RBCs). Most of the time, those who are affected have no symptoms. However, they should avoid specific triggers that may promote oxidative stress such as fava beans, that may fragilize RBCs and cause hemolysis. G6PD deficiency may have a subtle effect on the severity of hemolysis and also worsen the degree of anaemia in SCD when the two disorders coexist. Therefore, selective decision should be taken in patients in whom the two conditions coexist in the choice of drug and in the treatment of infections. The prevalence of the G-6-PD deficiency is high in SCD patients, but does not differ from that observed among non-SCD subjects .However, the G-6-PD deficiency appears to worsen the clinical features of SCD, there were more hospitalizations, major vaso-occlusive crises among G-6-PD deficient sickle cell patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Newborns who are delivered in NEW VALLEY GOVERNORATE | Screening of coexistence between sickle cell anaemia and G6PD deficiency |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| G6pd enzyme sickling test | Diagnostic Test | Measuring the enzyme level of G6PD and presence of Hbs |
|
| Measure | Description | Time Frame |
|---|---|---|
| prevelance of coexistence between sickle cell anaemia and G6PD deficiency in NEW VALLEY GOVERNORATE | Evaluate the prevalence of coexistence between sickle cell anaemia and G6PD deficiency in NEW VALLEY GOVERNORATE to create data base for endemic hereditary disease | One year |
| Measure | Description | Time Frame |
|---|---|---|
| Early diagnosis to decrease incidanc of complications | Early diagnosis of coexistence between sickle cell anaemia and G6PD deficiency to tell specific treatment and maintain haemoglobin level high to prevent more complications | One year |
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Inclusion Criteria:
- All new born with good general health
Exclusion Criteria:
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The study will be conducted on all newborn delivered in pediatric department in NEW VALLEY UNIVERSITY from jan2025 to Jan 2026
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Fatma Hussein Mahmoud, Assistant lecturer | Contact | 01062172982 | +2 | fh449615@gmail.com |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D005955 | Glucosephosphate Dehydrogenase Deficiency |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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EDTA samples and serum
| G6PD enzyme and sickling test | Diagnostic Test | Measuring enzyme level of G6PD and level of HbS |
|
| G6PD enzyme and HPLC | Diagnostic Test | Measuring G6PD enzyme level in neonates and measuring level of HbS |
|
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D002239 | Carbohydrate Metabolism, Inborn Errors |
| D008661 | Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |