Volume 8, Issue 3, September 2020, Page: 102-105
Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report
Patrick Olanrewaju Osho, Department of Haematology & Immunology, Faculty of Basic Clinical Sciences, University of Medical Sciences, Ondo-City, Nigeria
Matilda Adesuwa Osagie Ojo, Department of Haematology & Immunology, Faculty of Basic Clinical Sciences, University of Medical Sciences, Ondo-City, Nigeria
Evelyn Salewa Osho, Department of Radiology, Faculty of Basic Clinical Sciences, University of Medical Sciences, Ondo-City, Nigeria
Ndidi Aisha Okunnuga, Oncology Unit, Department of Radiology, Faculty of Basic Clinical Sciences, University of Medical Sciences, Ondo-City, Nigeria
Oluwatosin Idowu Oni, Department of Haematology/Virology, University of Medical Sciences Teaching Hospital, Akure, Nigeria
Olugbenga Festus Fabusiwa, Department of Medical Services, Ondo State primary Health Care Development Agency, Oke-Eda, Akure
Received: Jul. 16, 2020;       Accepted: Aug. 18, 2020;       Published: Sep. 21, 2020
DOI: 10.11648/j.ijgg.20200803.12      View  56      Downloads  36
Abstract
Sickle cell anaemia and Glucose-6-phosphate dehydrogenase deficiency have anaemia as major clinical consequence. The two disorders are rarely co-expressed in a patient. However, the pathological co-existence of both disorders tends to worsen and aggravate the clinical presentation in affected individuals. We report a diagnosis of the co-inheritance of these two disorders in a 43 year old man who was diagnosed with SCA at childhood. He was managed in a secondary health care facility since child hood. He has had multiple blood transfusions on account of repeated episodes of haemolytic anaemia which was solely attributed to SCA. He was referenced our center on account of recurrent severe heamolytic anaemia with a PCV of 9%. In the preceding 2 months to detecting his G6PD deficient status, he was having monthly blood transfusions on account of severe anaemia. Following the detection of his G6PD deficiency status and appropriate intervention with glutathione and selenium supplements and counseling to avoid exposure to oxidizing agents, he had a respite in the frequency of acute episodes of haemolysis necessitating blood transfusion, as well as an improvement in his steady state PCV of 16%. This case report underscores the importance of routine screening for G6PD status in patients with SCA so as to institute appropriate measures to reduce the worsening of incidence of acute episodes of haemolysis and the need for recurrent blood transfusions on account of this.
Keywords
Glucose-6-phoshate Deficiency, Co-expression, Sickle Cell Disease, Pathological Co-existence
To cite this article
Patrick Olanrewaju Osho, Matilda Adesuwa Osagie Ojo, Evelyn Salewa Osho, Ndidi Aisha Okunnuga, Oluwatosin Idowu Oni, Olugbenga Festus Fabusiwa, Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report, International Journal of Genetics and Genomics. Vol. 8, No. 3, 2020, pp. 102-105. doi: 10.11648/j.ijgg.20200803.12
Copyright
Copyright © 2020 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Reference
[1]
Antwi-Baffour, S., Adjei, J. K., Forson, P. O., Akakpo, S. Kyeremeh, R. and Seidu, M. A. (2019). Comorbidity of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease Exert Significant Effect on RBC Indices Hindawi Anemia. 2019: 1-9 https://doi.org/10.1155/2019/3179173.
[2]
L. van den Broek, E. Heylen, and M. van den Akker, “Glucose-6-phosphate dehydrogenase deficiency: not exclusively in males,” Clinical Case Reports, vol. 4, no. 12, pp. 1135–1137, 2016. View at: Publisher Site | Google Scholar.
[3]
Cappellini, M. D. and Fiorelli G. 2008. Glucose‐6‐phosphate dehydrogenase deficiency. Lancet 371: 64–74. [PubMed] [Google Scholar].
[4]
Ronquist, G., and Theodorsson E.. 2007. Inherited, non‐spherocytic haemolysis due to deficiency of glucose‐6‐phosphate dehydrogenase. Scand. J. Clin. Lab. Invest. 67: 105–111. [PubMed] [Google Scholar].
[5]
Manju T (2017) Glucose 6 phosphatase dehydrogenase (G6PD) and neurodegenerative disorders: Mapping diagnostic and therapeutic opportunities. Genes Dis. 4 (4): 196–203.
[6]
Prchal JT, Gregg XT. Red Cell Enzymes. Hematology/ASH Education Book. Available at http://asheducationbook.hematologylibrary.org/content/2005/1/19.full?sid=5fb34610-c164-4deb-9e85-0922086efaea. Accessed: June 29, 2020.
[7]
Ogunkanbi, S. O, Adedoyin, O. T., Biliaminu, S. A. and Ernest S. K. (2019) Prevalence of Glucose-6- Phosphate Dehydrogenase Deficiency among Steady-State Sickle Cell Disease Children at University of Ilorin Teaching Hospital, Ilorin. Open Journal of Pediatrics, 9, 29-38.
[8]
Umar M. Sickle cell anaemia: Prevention and control. 2010 http://www.gamji.com/article5000/NEW5314.HTM. Accessed July 28th 2020.
[9]
Jidda M L, Ibrahim KK, Aiki G, Ngaski AA, Blessing J, et al. (2017) The Prevalence of Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency in Students of Sultan Abdurrahaman School of Health Technology Gwadabawa, Sokoto, North-Western Nigeria. International Blood Research & Reviews. 7 (4): 1-6.
[10]
Firempong CK, Nsiah K, Yeboah FA. (2016). Some clinical and haematological effects of G6PD deficiency among individuals with sickle cell disorders in Kumasi, Ghana. The Journal of Medical Research. 2 (3): 81-85.
[11]
Fasola, F. A., Fowodu, F. O., Shokunbi, W. A. and Kotila, T. R. (2019). The effect of the coinheritance of glucose-6-phosphate dehydrogenase deficiency on the severity of sickle cell disease. Niger Postgrad Med J; 26: 118-22.
[12]
Kalliopi G, Ioannis GF, Dimitrios D, Konstantinos P, Panagiotis T, et al. (2019) Exercise in Glucose-6-Phosphate Dehydrogenase Deficiency: Harmful or Harmless? A Narrative Review Oxidative medicine and cellular longevity 2019 (6): 1-7.
[13]
Yang Y, Sauve A A. (2016) NAD (+) Metabolism: Bioenergetics, Signaling and Manipulation for Therapy. 1864 (12): 1787-1800.
[14]
Belisario A R, Rodrigues S R, Elvin T N, Velloso R, Maria S C, et al. (2016) Glucose -6-Phosphate dehyrogenase Deficiency in Brazillian Children With Sickle Cell Anaemia is not Associated With Clinical Ischaemic Stroke or High-Risk Transcranial Doppler. Padiatr Blood Cancer. 63: 1046-1049.
[15]
Benkerrou M., Alberti C., Couque N., Haouari Z., Ba A. et al. (2013). Impact of glucose-6-phosphate dehydrogenase deficiency on sickle cell anaemia expression in infancy and early childhood: A prospective study. Br J Haematol. 163: 646-54.
[16]
Samuel Antwi-Baffour, Jonathan Kofi Adjei, Peter Owadee Forson, Stephen Akakpo, Ransford Kyeremeh and Mahmood Abdulai Seidu (2019). Comorbidity of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease Exert Significant Effect on RBC Indices. Hindawi Anemia. https://doi.org/10.1155/2019/317917.
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