The co-existence of glucose-6-phosphate dehydrogenase deficiency among individuals with hemoglobinopathies and their effects on red cellindices

Sawer Sabri; Dilveen Ahmed  Mohammed

doi:10.47419/bjbabs.v4i02.201

Authors

Sawer Sabri Department of Medical Laboratory Technology, College of Health and Medical Technologies, Duhok Polytechnic University, Duhok, Iraq
Dilveen Ahmed Mohammed Laboratory department, Azad Teaching Hospital, Directorate General of Health in Duhok

DOI:

https://doi.org/10.47419/bjbabs.v4i02.201

Keywords:

Glucose-6-phosphate dehydrogenase, Sickle Cell Disease, Thalassemia, G6PDD, Duhok, Iraq

Abstract

Glucose 6 phosphate dehydrogenase deficiency and hemoglobinopathies are common among people of Duhok city in northern Iraq. The study was carried out to determine the prevalence of G6PD deficiency among individuals with thalassemia and sickle cell disorder, Glucose 6 phosphate dehydrogenase was estimated in eighty-four (84) persons with hemoglobinopathies to observe its prevalence. A manual procedure was used to estimate the G6PD enzyme activity of fresh samples from the participants using BIOLABO SA, G6PD assay kit (Maizy, France).G6PD deficit was detected in 15(17.90%) of the case, (10.71%) of SCD, and (7.14%) of beta thalassemia syndromes with a statistically significant association between G6PD enzyme level and clinical diagnosis. No significant correlation between RBC count and RDW was seen between low and G6PD enzyme level individuals with hemoglobinopathies.The study emphasized the prevalence of G6PD deficit to be higher among individuals with hemoglobinopathy compared to the normal subject from a previous study from the same geographical region.

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References

Smith-Whitley K. Reproductive issues in sickle cell disease. Blood.2014;124(24):3538–3581. 10.1182/blood-2014-07-577619.

Al-Allawi N, Eissa AA, Jubrael JM, et al. Prevalence and molecular characterization of Glucose-6-Phosphate dehydrogenase deficient variants among the Kurdish population of Northern Iraq. BMC Blood Disord. 2010;10(6):6–6. 10.1186/1471-2326-10-6.

Lin M, Yang LY, Xie DD, et al. G6PD deficiency and hemoglobinopathies: molecular epidemiological characteristics and healthy effects on malaria endemic Bioko Island, Equatorial Guinea. PLoS one. 2015;10(4):123991–123991. 10.1371/journal.pone.0123991.

Gibbs WN, Wardle J, Serjeant GR. Glucose-6-phosphate dehydrogenase deficiency and homozygous sickle cell disease in Jamaica. Br J Haematol. 1980;45(1):24672826–24672826.

Pornprasert S, Phanthong S. Anemia in patients with coinherited thalassemia and glucose-6-phosphate dehydrogenase deficiency. Hemoglobin. 2013;37(6):536–579. 10.3109/03630269.2013.819558.

Ha AN. Thalassaemia and glucose-6-phosphate dehydrogenase deficiency in sickle cell disorder patients in Taiz. Yemen East Mediterr Health J. 2011;17(5):21796953– 21796953.

Gautam N, Gaire B, Manandhar T, et al. Glucose 6 phosphate dehydrogenase deficiency and hemoglobinopathy in South Western Region Nepal: a boon or burden. BMC Res Notes. 2019;12(1):734–734. 10.1186/s13104-019-4762-6.

Beutler E. Glucose-6-phosphate dehydrogenase deficiency: a historical perspective. Blood. 2008;111(1):16–24. 10.1182/blood-2007-04-077412.

Au SW, Gover S, Lam VM, et al. Human glucose-6-phosphate dehydrogenase: the crystal structure reveals a structural NADP+ molecule and provides insights into enzyme deficiency. Structure. 2000;8(3):293–303. 10.1016/s0969-2126(00)00104-0.

Garcia J, Han D, Sancheti H, Yap LP, Kaplowitz N, Cadenas E. Regulation of mitochondrial glutathione redox status and protein glutathionylation by respiratory substrates. J Biol Chem. 2010;285(51):39646–54. 10.1074/jbc.M110.164160.

Taylor SM, Cerami C, Fairhurst RM. Hemoglobinopathies: slicing the Gordian knot of Plasmodium falciparum malaria pathogenesis. PLoS pathog. 2013;9(5):1003327–1003327. 10.1371/journal.ppat.1003327.

Taher A, Vichinsky E, Musallam K, et al.; 2014.

Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet. 2008;371(9606):64–74. 10.1016/S0140-6736(08)60073-2.

Taylor SM, Fairhurst RM. Malaria parasites and red cell variants: when a house is not a home. Curr Opin Hematol. 2014;21(3):193–200.

1097/MOH.0000000000000039.

Allawi A, N, Dousky A, A. Frequency of haemoglobinopathies at premarital health screening in Dohuk, Iraq: implications for a regional prevention programme. East Mediterr Health J. 2010;16(4):20795420–20795420. https://iris.who.int/handle/10665/117880.

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;p. 3179173–3179173. 10.1155/2019/3179173.

Nkhoma ET, Poole C, Vannappagari V, et al. The global prevalence of glucose-6- phosphate dehydrogenase deficiency: a systematic review and meta-analysis. Blood Cells Mol Dis. 2009;42(3):267–78. 10.1016/j.bcmd.2008.12.005.

Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;11(9757):61029–61029. 10.1016/S0140-6736(10)61029-X.

Mason PJ, Bautista JM, Gilsanz F. G6PD deficiency: the genotype-phenotype association. Blood Rev. 2007;21(5):267–83. 10.1016/j.blre.2007.05.002.

Liu TZ, Lin TF, Hung IJ, et al. Enhanced susceptibility of erythrocytes deficient in glucose-6-phosphate dehydrogenase to alloxan/glutathione-induced decrease in red cell deformability. Life Sci. 1994;55(3):55–60. 10.1016/0024-3205(94)00888-4.

Kittisares K, Palasuwan D, Noulsri E, Palasuwan A. Thalassemia trait and G6PD deficiency in Thai blood donors; 2019. https://doi.org/10.1016/j.transci.2019.03.009.