ArticleBlood advances2018
Biological impact of α genes, β haplotypes, and G6PD activity in sickle cell anemia at baseline and with hydroxyurea.
Article in Blood advances, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Genetic Variation and Sickle Cell Disease Severity: A Systematic Review and Meta-Analysis.JAMA network open · 2023Pooled it
- Trial
- Hydroxyurea (hydroxycarbamide) use in adults with haemoglobin SC disease: A real-world study in Quebec.British journal of haematology · 2026Article
- ß-Globin gene cluster haplotypes in Moroccan sickle cell disease patients: diversity pilot study.African health sciences · 2026Article
- Molecular Testing in Sickle Cell Disease: From Newborn Screening to Transfusion Care.International journal of laboratory hematology · 2026Review
- The pleiotropic effects of α-thalassemia on HbSS and HbSC sickle cell disease: Reduced erythrocyte cation co-transport activity, serum erythropoietin, and transfusion burden, do not translate into increased survival.American journal of hematology · 2022Article
- Incidence, kinetics, and risk factors for intra- and extracranial cerebral arteriopathies in a newborn sickle cell disease cohort early assessed by transcranial and cervical color Doppler ultrasound.Frontiers in neurology · 2022Article
- Genotypic Diversity among Angolan Children with Sickle Cell Anemia.International journal of environmental research and public health · 2021Article
- Evolutionary history of sickle-cell mutation: implications for global genetic medicine.Human molecular genetics · 2021Review
- Influence of UGT1A1 promoter polymorphism, α-thalassemia and βAnnals of hematology · 2021Article
- Alpha thalassemia, but not βAnnals of hematology · 2021Article
- High fetal hemoglobin level is associated with increased risk of cerebral vasculopathy in children with sickle cell disease in Mayotte.BMC pediatrics · 2020Article
- Comprehensive analysis of mitochondrial and nuclear DNA variations in patients affected by hemoglobinopathies: A pilot study.PloS one · 2020Article
- Article
- Optimizing Hydroxyurea Treatment for Sickle Cell Disease Patients: The Pharmacokinetic Approach.Journal of clinical medicine · 2019Article
- Why, Who, When, and How? Rationale for Considering Allogeneic Stem Cell Transplantation in Children with Sickle Cell Disease.Journal of clinical medicine · 2019Review
- The carrier state for sickle cell disease is not completely harmless.Haematologica · 2019Review
Corrections and comments
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8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Sickle cell anemia (SCA), albeit monogenic, has heterogeneous phenotypic expression, mainly related to the level of hemoglobin F (HbF). No large cohort studies have ever compared biological parameters in patients with major β-globin haplotypes; ie, Senegal (SEN), Benin (BEN), and Bantu/Central African Republic (CAR). The aim of this study was to evaluate the biological impact of α genes, β haplotypes, and glucose-6-phosphate dehydrogenase (G6PD) activity at baseline and with hydroxyurea (HU). Homozygous HbS patients from the Créteil pediatric cohort with available α-gene and β-haplotype data were included (n = 580; 301 females and 279 males) in this retrospective study. Homozygous β-haplotype patients represented 74% of cases (37.4% CAR/CAR, 24.3% BEN/BEN, and 12.1% SEN/SEN). HU was given to 168 cohort SCA children. Hematological parameters were recorded when HbF was maximal, and changes (
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