Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report

Patrick Olanrewaju Osho; Matilda Adesuwa Osagie Ojo; Evelyn Salewa Osho; Ndidi Aisha Okunnuga; Oluwatosin Idowu Oni; Olugbenga Festus Fabusiwa

doi:doi:10.11648/j.ijgg.20200803.12

Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report

Patrick Olanrewaju Osho, Matilda Adesuwa Osagie Ojo, Evelyn Salewa Osho, Ndidi Aisha Okunnuga, Oluwatosin Idowu Oni, Olugbenga Festus Fabusiwa

Published in International Journal of Genetics and Genomics (Volume 8, Issue 3)

Received: 16 July 2020 Accepted: 18 August 2020 Published: 21 September 2020

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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.

Published in	International Journal of Genetics and Genomics (Volume 8, Issue 3)
DOI	10.11648/j.ijgg.20200803.12
Page(s)	102-105
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2020. Published by Science Publishing Group

Keywords

Glucose-6-phoshate Deficiency, Co-expression, Sickle Cell Disease, Pathological Co-existence

References

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[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.
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[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.
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[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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APA Style

Patrick Olanrewaju Osho, Matilda Adesuwa Osagie Ojo, Evelyn Salewa Osho, Ndidi Aisha Okunnuga, Oluwatosin Idowu Oni, et al. (2020). Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report. International Journal of Genetics and Genomics, 8(3), 102-105. https://doi.org/10.11648/j.ijgg.20200803.12

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ACS Style

Patrick Olanrewaju Osho; Matilda Adesuwa Osagie Ojo; Evelyn Salewa Osho; Ndidi Aisha Okunnuga; Oluwatosin Idowu Oni, et al. Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report. Int. J. Genet. Genomics 2020, 8(3), 102-105. doi: 10.11648/j.ijgg.20200803.12

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AMA Style

Patrick Olanrewaju Osho, Matilda Adesuwa Osagie Ojo, Evelyn Salewa Osho, Ndidi Aisha Okunnuga, Oluwatosin Idowu Oni, et al. Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report. Int J Genet Genomics. 2020;8(3):102-105. doi: 10.11648/j.ijgg.20200803.12

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@article{10.11648/j.ijgg.20200803.12,
  author = {Patrick Olanrewaju Osho and Matilda Adesuwa Osagie Ojo and Evelyn Salewa Osho and Ndidi Aisha Okunnuga and Oluwatosin Idowu Oni and Olugbenga Festus Fabusiwa},
  title = {Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report},
  journal = {International Journal of Genetics and Genomics},
  volume = {8},
  number = {3},
  pages = {102-105},
  doi = {10.11648/j.ijgg.20200803.12},
  url = {https://doi.org/10.11648/j.ijgg.20200803.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijgg.20200803.12},
  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.},
 year = {2020}
}

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TY  - JOUR
T1  - Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report
AU  - Patrick Olanrewaju Osho
AU  - Matilda Adesuwa Osagie Ojo
AU  - Evelyn Salewa Osho
AU  - Ndidi Aisha Okunnuga
AU  - Oluwatosin Idowu Oni
AU  - Olugbenga Festus Fabusiwa
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PY  - 2020
N1  - https://doi.org/10.11648/j.ijgg.20200803.12
DO  - 10.11648/j.ijgg.20200803.12
T2  - International Journal of Genetics and Genomics
JF  - International Journal of Genetics and Genomics
JO  - International Journal of Genetics and Genomics
SP  - 102
EP  - 105
PB  - Science Publishing Group
SN  - 2376-7359
UR  - https://doi.org/10.11648/j.ijgg.20200803.12
AB  - 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.
VL  - 8
IS  - 3
ER  -

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Author Information

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

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APA Style

Patrick Olanrewaju Osho, Matilda Adesuwa Osagie Ojo, Evelyn Salewa Osho, Ndidi Aisha Okunnuga, Oluwatosin Idowu Oni, et al. (2020). Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report. International Journal of Genetics and Genomics, 8(3), 102-105. https://doi.org/10.11648/j.ijgg.20200803.12

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ACS Style

Patrick Olanrewaju Osho; Matilda Adesuwa Osagie Ojo; Evelyn Salewa Osho; Ndidi Aisha Okunnuga; Oluwatosin Idowu Oni, et al. Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report. Int. J. Genet. Genomics 2020, 8(3), 102-105. doi: 10.11648/j.ijgg.20200803.12

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AMA Style

Patrick Olanrewaju Osho, Matilda Adesuwa Osagie Ojo, Evelyn Salewa Osho, Ndidi Aisha Okunnuga, Oluwatosin Idowu Oni, et al. Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report. Int J Genet Genomics. 2020;8(3):102-105. doi: 10.11648/j.ijgg.20200803.12

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@article{10.11648/j.ijgg.20200803.12,
  author = {Patrick Olanrewaju Osho and Matilda Adesuwa Osagie Ojo and Evelyn Salewa Osho and Ndidi Aisha Okunnuga and Oluwatosin Idowu Oni and Olugbenga Festus Fabusiwa},
  title = {Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report},
  journal = {International Journal of Genetics and Genomics},
  volume = {8},
  number = {3},
  pages = {102-105},
  doi = {10.11648/j.ijgg.20200803.12},
  url = {https://doi.org/10.11648/j.ijgg.20200803.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijgg.20200803.12},
  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.},
 year = {2020}
}

Copy | Download

TY  - JOUR
T1  - Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report
AU  - Patrick Olanrewaju Osho
AU  - Matilda Adesuwa Osagie Ojo
AU  - Evelyn Salewa Osho
AU  - Ndidi Aisha Okunnuga
AU  - Oluwatosin Idowu Oni
AU  - Olugbenga Festus Fabusiwa
Y1  - 2020/09/21
PY  - 2020
N1  - https://doi.org/10.11648/j.ijgg.20200803.12
DO  - 10.11648/j.ijgg.20200803.12
T2  - International Journal of Genetics and Genomics
JF  - International Journal of Genetics and Genomics
JO  - International Journal of Genetics and Genomics
SP  - 102
EP  - 105
PB  - Science Publishing Group
SN  - 2376-7359
UR  - https://doi.org/10.11648/j.ijgg.20200803.12
AB  - 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.
VL  - 8
IS  - 3
ER  -

Copy | Download