We have determined the molecular basis and frequency of alpha-thalassaemia in Nigeria. The alpha-thalassaemia determinant in this population is caused by only one type of single alpha globin gene deletion (-alpha 3.7). Comparison of the haematological features of those patients who have sickle-cell disease with (-alpha/alpha alpha, -alpha/-alpha) or without (alpha alpha/alpha alpha) alpha-thalassaemia showed similar trends to those reported in Jamaican and U.S. patients with these interactions. However, in contrast to studies in some other African populations we have shown that the frequency of alpha-thalassaemia in Nigeria is the same (0.24) in patients with or without homozygous sickle-cell disease (AA, AS and AC genotypes).
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/j.1600-0609.1987.tb00013.x | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Systematic Review of Genetic Modifiers Associated with the Development and/or Progression of Nephropathy in Patients with Sickle Cell Disease.
Int J Mol Sci
May 2024
Haematology Department, Faculty of Basic Clinical Sciences, College of Health Sciences, Nnamdi Azikiwe University, Nnewi PMB 5025, Anambra State, Nigeria.
Sickle cell nephropathy (SCN) is a common complication of sickle cell disease (SCD) that significantly contributes to morbidity and mortality. In addition to clinical and life-style factors, genetic variants influence this risk. We performed a systematic review, searching five databases.
View Article and Find Full Text PDFClinical and laboratory factors associated with splenic dysfunction among patients with sickle cell disease in a malaria endemic region.
Trans R Soc Trop Med Hyg
December 2023
Department of International Public Health, Liverpool School of Tropical Medicine, Liverpool, L3 5QA, UK.
Background: Although loss of splenic function is the expected natural course for individuals with sickle cell disease (SCD), factors such as high HbF and coexistence of alpha thalassemia may ameliorate this process. We evaluated factors associated with two surrogate markers of spleen dysfunction, namely Howell-Jolly bodies (HJBs) and argyrophilic inclusion (AI) red cell counts, among patients with SCD.
Methods: Cross-sectional data of 182 patients with SCD (median age 11 y; 1-45 y) and 102 normal controls (median age 12 y; 1-32 y) were evaluated.
The Effect of Alpha Thalassemia, HbF and HbC on Haematological Parameters of Sickle Cell Disease Patients in Ibadan, Nigeria.
Mediterr J Hematol Infect Dis
January 2022
Department of Medicine, University of Chicago, IL, USA.
Background: Sickle cell disease is a protean disease with limited data on Nigeria's phenotypic and genetic variants. This study was conducted to provide baseline data on these variants by characterising the existing forms of sickle cell disease and correlating these with basic haematological parameters.
Methods: Adult and paediatric patients with SCD were recruited from a tertiary health centre in Nigeria.
Understanding the interplay of genetic factors with haemoglobin expression and pathological processes in sickle cell disease is important for pharmacological and gene-therapeutic interventions. In our nascent study cohort of Nigerian patients, we found that three major disease-modifying factors, HbF levels, α-thalassaemia deletion and genotype, had expected beneficial haematological effects. A key variant, while improving HbF levels (5.
View Article and Find Full Text PDFAssociation of alpha-thalassemia and Glucose-6-Phosphate Dehydrogenase deficiency with transcranial Doppler ultrasonography in Nigerian children with sickle cell anemia.
J Clin Lab Anal
June 2021
Department of Molecular Biology and Biotechnology, Nigerian Institute of Medical Research, Lagos, Nigeria.
Background: Stroke is a devastating complication of sickle cell anemia (SCA) and can be predicted through abnormally high cerebral blood flow velocity using transcranial Doppler Ultrasonography (TCD). The evidence on the role of alpha-thalassemia and glucose-6-phosphate dehydrogenase (G6PD) deficiency in the development of stroke in children with SCA is conflicting. Thus, this study investigated the association of alpha-thalassemia and G6PD(A ) variant with abnormal TCD velocities among Nigerian children with SCA.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!