Prevalence of glucose-6-phosphate dehydrogenase deficiency and alpha-thalassemia in children with sickle cell trait

Submitted: August 2, 2023
Accepted: April 17, 2024
Published: May 27, 2024
Abstract Views: 133
PDF: 103
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Authors

The present study aimed to evaluate the prevalence of alpha-thalassemia and glucose-6-phosphate dehydrogenase (G6PD) deficiency in sickle cell trait (SCT) patients to determine its effect on red blood cells (RBC) parameters. This cross-sectional study was conducted on 102 blood samples obtained from children and teenagers with SCT aged between 5 and 18 years old who were referred to Shahid Beqaei Hematology and Oncology Hospital and Abuzar Children's Hospital in Ahvaz city (Iran) from October 2021 to November 2022. About 5 mL of blood was collected via venipuncture from each patient and used to run G6PD, complete blood count, and hemoglobin (Hb) electrophoresis tests. The data were analyzed using SPSS version 22, and the significance level in all tests was considered less than 0.05. Results showed that the prevalence of heterozygous and homozygous alpha-thalassemia and iron deficiency anemia (IDA) in the examined sample was 18.63%, 18.63%, and 10.78%, respectively. Also, 13.72% of patients suffered from G6PD deficiency. The results imply that G6PD deficiency may increase the severity of anemia in SCT patients. Therefore, it is necessary to screen all SCT patients for G6PD deficiency to ensure that their condition is not exacerbated during unexpected events such as diseases or stress.

Dimensions

Altmetric

PlumX Metrics

Downloads

Download data is not yet available.

Citations

Piel FB, Hay SI, Gupta S, et al. Global burden of sickle cell anaemia in children under five, 2010–2050: modelling based on demographics, excess mortality, and interventions. PLoS Med 2013;10:e1001484. DOI: https://doi.org/10.1371/journal.pmed.1001484
Piccin A, Fleming P, Eakins E, et al. Sickle cell disease and dental treatment. J Ir Dent Assoc 2008;54:75-9.
Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet 2017;390:311-23. DOI: https://doi.org/10.1016/S0140-6736(17)30193-9
Ballas SK, Lieff S, Benjamin LJ, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol 2010;85:6-13. DOI: https://doi.org/10.1002/ajh.21550
Robert MK, Bonita FS, Joseph STG, Nina FS. Nelson Textbook of Pediatrics E-Book. Elsevier Health Sciences, 2015; p. 3408.
Balgir RS. Do tribal communities show an inverse relationship between sickle cell disorders and glucose-6-phosphate dehydrogenase deficiency in malaria endemic areas of Central-Eastern India? Homo 2006;57:163-76. DOI: https://doi.org/10.1016/j.jchb.2006.01.003
Bouanga JC, Mouélé R, Préhu C, et al. Glucose-6-phosphate dehydrogenase deficiency and homozygous sickle cell disease in Congo. Hum Hered 1998;48:192-7. DOI: https://doi.org/10.1159/000022801
Rees DC, Lambert C, Cooper E, et al. Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia. Blood 2009;114:742-3 DOI: https://doi.org/10.1182/blood-2009-04-216861
Antwi-Baffour S, Adjei JK, Forson PO, et al. Comorbidity of Glucose-6-Phosphate Dehydrogenase deficiency and Sickle Cell Disease exert significant effect on RBC indices. Anemia 2019:3179173. DOI: https://doi.org/10.1155/2019/3179173
Smith K, Kwiatkowski JL. Hemoglobinopathies. Nelson Textbook of Pediatrics. Third Edition ed 2020. p. 2540-58 and 2522-26.
Simpore J, Ilboudo D, Damintoti K, et al. Glucose-6-phosphate dehydrogenase deficiency and sickle cell disease in Burkina Faso. Pak J Biol Sci 2007;10:409-14. DOI: https://doi.org/10.3923/pjbs.2007.409.414
Chan TK. Glucose-6-phosphate dehydrogenase deficiency. J Pediatr 1996;1:23–30.
Bienzle U, Sodeinde O, Effiong CE, Luzzatto L. Glucose 6-phosphate dehydrogenase deficiency and sickle cell anemia: frequency and features of the association in an African community. Blood 1975;46:591-7. DOI: https://doi.org/10.1182/blood.V46.4.591.bloodjournal464591
Mehta A, Mason PJ, Vulliamy TJ. Glucose-6-phosphate dehydrogenase deficiency. Best Pract Res Clin Haematol 2000;13:21-38. DOI: https://doi.org/10.1053/beha.1999.0055
Sani A, Kumo BA, Kasim PM, Haruna MM. Transfusion-dependent anemia in a patient with sickle cell disease as well as HIV-associated tuberculosis. S Afr Med J 2017;4:52. DOI: https://doi.org/10.4103/ssajm.ssajm_8_17
Gibbs WN, Wardle J, Serjeant GR. Glucose‐6‐phosphate dehydrogenase deficiency and homozygous sickle cell disease in Jamaica. Br J Haematol 1980;45:73-80. DOI: https://doi.org/10.1111/j.1365-2141.1980.tb03812.x
Inati A, Noureldine M, Amhez G, et al. Prevalence of G6PD deficiency in patients with sickle cell disease and impact on disease severity in Lebanon. Conference: Haematologica 2015;100:293-4.
Firempong CK, Nsiah K, Yeboah FA. Some clinical and haematological effects of G6PD deficiency among individuals with sickle cell disorders in Kumasi, Ghana. J Med Res 2016;2:81-5. DOI: https://doi.org/10.31254/jmr.2016.2309
Zohoun A, Baglo-Agbodande T, Adje T, et al. Prevalence of association of Glucose-6-Phosphate Dehydrogenase deficiency and Sickle Cell Disease at the National Teaching Hospital of Cotonou in Benin. Open J Blood Dis 2023;13:93-101. DOI: https://doi.org/10.4236/ojbd.2023.133012
Kambale-Kombi P, Marini Djang’eing’a R, Alworong’a Opara JP, et al. Does glucose-6-phosphate dehydrogenase deficiency worsen the clinical features of sickle cell disease? A multi-hospital-based cross-sectional study. Hematology 2022;27:590-5. DOI: https://doi.org/10.1080/16078454.2022.2074715

How to Cite

Hafez-Quran, H., Keikhaei, B., Yousefi, H., Saki-Malehi, A., Saki, N., & Rezaei-Koukhdan, Z. (2024). Prevalence of glucose-6-phosphate dehydrogenase deficiency and alpha-thalassemia in children with sickle cell trait. Journal of Biological Research - Bollettino Della Società Italiana Di Biologia Sperimentale, 97(1). https://doi.org/10.4081/jbr.2024.11631