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 Table of Contents  
ORIGINAL ARTICLE
Year : 2015  |  Volume : 14  |  Issue : 2  |  Page : 83-86

Prevalence of glucose-6-phosphate dehydrogenase deficiency among sickle cell patients


Department of Medical Laboratory Science, School of Basic Medical Science, College of Medical Science, University of Benin, Nigeria

Date of Web Publication21-Nov-2015

Correspondence Address:
Osime Evarista Odaburhine
Department of Medical Laboratory Science, School of Basic Medical Science, College of Medical Science, University of Benin
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2384-5589.170160

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  Abstract 

Background: Anemia is one of the important clinical significance in sickle cell disease, and a major clinical consequence of glucose-6-phosphate dehydrogenase (G6PD) deficiency is hemolytic anemia. The aim of this study is to compare the enzyme activity in sickle cell patients (SS) and normal hemoglobin (Hb) (AA) individuals. Patients and Methods: A total of 100 hundred samples comprised of 50 sickle cell patients as test subjects and 50 Hb AA individuals were used as controls. Each of the blood sample collected was screened immediately for G6PD deficiency using the methemoglobin reduction test and Randox kit. Results obtained were subjected to statistical analysis using paired t-test. Result: Glucose-6-phosphate dehydrogenase deficiency was found to be significantly higher in test subjects (P < 0.01) when compared with the control subjects. It was also shown that G6PD levels tend to increase with increase in age though this increase was not significant (P > 0.05). There was no significant difference in G6PD levels between males and females sickle cell patients (P > 0.05). Conclusion: These results, therefore, suggests that G6PD enzyme activity is much more reduced in the sickle cell patient who may contribute to making them prone to hemolytic anemia when compared to normal individuals.

Keywords: Glucose 6 phosphate dehydrogenase, hemolysis, sickle cell disease


How to cite this article:
Odaburhine OE. Prevalence of glucose-6-phosphate dehydrogenase deficiency among sickle cell patients. Afr J Med Health Sci 2015;14:83-6

How to cite this URL:
Odaburhine OE. Prevalence of glucose-6-phosphate dehydrogenase deficiency among sickle cell patients. Afr J Med Health Sci [serial online] 2015 [cited 2020 Aug 4];14:83-6. Available from: http://www.ajmhs.org/text.asp?2015/14/2/83/170160


  Introduction Top


Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most well-known human genetic defect (enzymopathy) affecting over 400 million subjects throughout the world. [1],[2] This genetic disorder affects various geographic populations; [3] occurring both in black and white races. [4] The major clinical consequence of G6PD deficiency is hemolytic anemia. [5] This disorder is usually episodic, but the vast majority of people with G6PD deficiency have no symptoms until subjected to oxidative challenge. The term sickle cell disorder refers to the state in which the red cell undergoes sickling when it is deoxygenated. The sickle cell disease (SCD) are disorders in which sickling produces prominent clinical manifestation such as hemolytic anemia. [6] The major symptoms of hemolytic anemia are jaundice, dark urine, abdominal and back pain, lowered red blood cell count and elevated bilirubin. [7] In Africa where SCD is prevalent, sickle cell patients with G6PD deficiency suffer more as a result of combined effect on red blood cells due to excess oxidative free radicals. [8] It has been recorded that the highest incidence of G6PD deficiency is found in those with the greatest occurrence of thalassemia. [9] Thus the deficiency is very common among persons of Africa, Asian and Mediterranean descent. Individuals that suffer from severe and chronic form of deficiency in addition, may have gallstones, enlarge spleen, defective white blood cell and cataracts becoming more severe in sickle cell individuals. Among all inherited red blood cell (RBC) disorders, G6PD deficiency and sickle cell trait share the following features. Being highly frequent in many geographical areas and ethnic groups, usually asymptomatic and in stable conditions not altering hemoglobin (Hb) level, RBC count and indices.


  Justification of study Top


A lot of sickle cell patients often encounter some form of crises or the other, most of which leads to anemia. Hence this study is to evaluate the risk of enzyme activity G6PD on sickle cell patients.


  Patients and methods Top


Study Subjects

A total of fifty (50) patients were randomly selected, this comprised of 30 male and 20 female sickle cell patients whose Hb genotype have been confirmed SS as test subjects and 50 individuals whose Hb genotype have been confirmed AA as control subjects. These subjects whose ages ranged from 10 to 40 years were all students in post primary and tertiary institutions attending a sickle cell clinic in Benin City. Consent was obtained from each participant prior to blood collection.

Sample Collection

Four milliliters of venous blood was collected from each participant into 1% ethylene diamine tetraacetic acid bottle and carefully mixed. They were then transported to the laboratory in an ice pack for analysis. G6PD activity was measured using the Randox G6PDH kits, whose principle is based on the measurement of the rate of absorbance change at 340 nm due to the reduction of nicotinamide adenine dinucleotide phosphate. Determination of packed cell volume (PCV) was carried out using the Hematocrit method as described by Schalm et al. [10] Dacie and Lewis. [11] Hb concentration was determined using the cyanmethemoglobin method. [12] All results were analyzed using paired t-test, measure of significance was taken as P value (i.e., P > 0.05) was regarded as insignificant while P < 0.05 was regarded as significant and P < 0.01 highly significant.


  Results Top


There was a significant increase in G6PD levels (P < 0.01) between sickle cell patients and normal individuals while there was none when the sexes of these individuals were compared as shown in [Table 1] and [Table 2]. There was also no significant change in G6PD levels among the different age ranges. PCV and Hb levels were observed to be reduced in test subjects when compared to that of the controls as shown in [Table 3] and [Table 4], [Figure 1] and [Figure 2] respectively.
Figure 1: Comparison of packed cell volume levels between male and female

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Figure 2: Comparison of hemoglobin results between males and females

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Table 1: G6PD levels between sickle cell patients and normal subjects

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Table 2: G6PD levels between male and female test subject

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Table 3: G6PD levels between different age groups of both test and control subjects

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Table 4: PCV and Hb levels among test and control subjects

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  Discussion Top


Intracorpuscular RBC defects might affect its survival, resistance to various stresses and/or interaction with other cells like leucocytes or endothelial cells. G6PD levels were found to be significantly reduced in test subjects when compared to controls [Figure 3] this could be attributed to the rigidity and lose of red cell integrity in sickle cell patients and a depressed erythroporetic activity making them more prone to oxidative stress. A review of the work by KO [13] also showed that sickle cell patients have a significantly reduced G6PD levels, an assay of the immature red cell would naturally yield higher enzyme level. [14] The reduced levels of G6PD recorded in sickle cell anemia patients would cause red blood cells to counter balance oxidative stress, exposing them to intravascular hemolysis.
Figure 3: Comparison of Glucose-6-phosphate dehydrogenase between normal and sickle cell patient

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The incidence rate of deficiency was observed not to be significant between males and females [Figure 4] (P > 0.05). This is at variance with other studies that observed a higher occurrence in females than in males. [14] This can be attributed to the geographic location of the study population since the levels of G6PD deficiency is seen to vary from one geographic location to another. [15]
Figure 4: Comparison of Glucose-6-phosphate dehydrogenase between male and female

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The genes responsible for inheriting G6PD are located on the x-chromosomes. Consequently, enzyme deficiency finds full expression in males carrying a variant gene (hemizygotes) and in female homozygous. [16] Thus, the prevalence of GB6PD deficiency in any population is determined by the number of deficient males. [17] Females are also at risk of hemolysis and jaundice. [18] In a population, with a high prevalence rate, early detection of the enzyme deficiency by neonatal screening can prevent complications, such as neonatal jaundice that is usually severe enough to cause death or permanent neurological damage. [19] Early detection can also help to avoid the recourse to permanent support with drugs by deficient individuals.

In this study, it was observed that there was no statistical difference in G6PD levels among the various age brackets, although it was found to be higher in the age group of >30years. This difference was not significant as shown in [Figure 5]. This goes to show that this enzyme deficiency can occur in all irrespective of their ages. In a study done by Abubakar et al., they recorded the highest number of deficient subjects within the age interval of 16-22 years old. Since these subjects are of marriageable age, there is a need for the development of appropriate control strategies based on the availability of relevant and adequate information on the frequency and severity of its complication.
Figure 5: Comparison of Glucose-6-phosphate dehydrogenase across age groups

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Glucose-6-phosphate dehydrogenase deficiency has been associated with a wide variety of serious clinical manifestations including hemolytic anemia [20],[21] neonatal jaundice [22] and kernicterus [23] as well as being an important risk factor for diabetes mellitus [24] and recurrent abortion. [25] This may also contribute to the reduce pack cell volume and Hb levels in SCD patients recorded in this study (P < 0.01).

In conclusion, G6PD levels are seen to be reduced in sickle cell anemia patients with females been more deficient than males and can occur in all persons irrespective of their ages. It is recommended that early diagnosis and characterization of G6PD deficiency be done to provide important information for genetic counseling and epidemiology surveillance, thus preventing the occurrence of a hemolytic crisis in the affected population.


  Acknowledgement Top


I wish to express my heartfelt appreciation to the consultant for permission to use the patients attending the sickle cell clinic of the Central Hospital, Benin City.

 
  References Top

1.
Du WJ, Qundibin LC, Duan S. Molecular Characterization of G6PD deficiency from various ethnic groups in Southern China Medical Genomics. 1999; HGM 2002 Poster Abstract.  Back to cited text no. 1
    
2.
Luzzatto L, Mehta AB. G-6-PD Deficiency In: Scriver R, Arthur LB, Beaudet NS, David V. editors. Metabolic and Molecular Basis of Inherited Disease. 7 th ed. New York: McGraw-Hill; 1995.  Back to cited text no. 2
    
3.
Beutler E, Westwood B, Prchal JT, Vaca G, Bartsocas CS, Baronciani L. New glucose-6-phosphate dehydrogenase mutations from various ethnic groups. Blood 1992;80:255-6.  Back to cited text no. 3
    
4.
Szeinberg HH, West MC, Gallapher D, Mentzer W. The cooperative study of sickle cell diseases, effect of G6PD deficiency upon sickle cell anemia. Blood 1988;71:748-52.  Back to cited text no. 4
    
5.
Huisman TH, Carver FH, Efremov GD. A Syllabus of Human Hemoglobin Variants. Augusta, GA, USA: The Sickle Cell Anemia Foundation; 1996.  Back to cited text no. 5
    
6.
Beutler E. Hemolytic Anemia in Disorders of Red Cell Metabolism. New York: Plenum Medical Book Company; 1990. p. 23-64.  Back to cited text no. 6
    
7.
Al-Omran A, Al-Ghazal F, Gupta S, John TB. Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice in Al-Hofuf area. Ann Saudi Med 1999;19:156-8.  Back to cited text no. 7
    
8.
Fleming AF. Hematologic disorders In: Strickland GT, editor. Hunter's Tropical Medicine. Philadelphia: WB Saunders; 1991. p. 52.  Back to cited text no. 8
    
9.
Tagarelli A, Piro A, Bastone L, Tagarelli G. Identification of glucose 6-phosphate dehydrogenase deficiency in a population with a high frequency of thalassemia. FEBS Lett 2000;466:139-42.  Back to cited text no. 9
    
10.
Schalm OW, Jain NC, Caroll EJ. Veterinary Hematology. 3 rd ed. Philadelphia: Lea and Fabiger; 1995.  Back to cited text no. 10
    
11.
Dacie JV, Lewis SM. Practical Hematology. 10 th ed. England: ELBS with Churchill Livingstone; 2001. p. 37-85.  Back to cited text no. 11
    
12.
Jain NC. Schalm's Veterinary Hematology. 4 th ed. Philadelphia: Lea and Fabiger; 1986. p. 564-72.  Back to cited text no. 12
    
13.
Ko CH, Yung E, Li K, Li CL, Ng PC, Fung KP, et al. Multiplex primer extension reaction screening and oxidative challenge of glucose-6-phosphate dehydrogenase mutants in hemizygous and heterozygous subjects. Blood Cells Mol Dis 2006;37:21-6.  Back to cited text no. 13
    
14.
Abubakar A, Musa A, Lliyasu B, Adetunji J, Olaniru O, Akande T, et al. Incidence of Glucose-6-Phosphate Dehydrogenase (G-6-PD) Deficiency in Apparently Healthy Individuals in some parts of North Central Nigeria. J Med Lab Sci 2005;14:29-34.  Back to cited text no. 14
    
15.
Kahinde MO, Akinyaju OO. Glucose-6-Phosphate Dehydrogenase Deficiency: A review and proposals for its control in Nigeria. Niger Med Pract 1991;21:47-52.  Back to cited text no. 15
    
16.
Stamatoyannopoulos G, Voigtlander V, Kotsakis P, Akrivakis A. Genetic diversity of the "Mediterranean" glucose-6-phosphate dehydrogenase deficiency phenotype. J Clin Invest 1971;50:1253-61.  Back to cited text no. 16
[PUBMED]    
17.
Miller DR. Hemolytic Anemias: Metabolic disorders. In: Miller DR, Baehner RL, editors. Blood Disease of Infancy and Childhood. St. Louis; Mosby Year Book Inc.; 1995. p. 354.  Back to cited text no. 17
    
18.
McCurdy PR, Schner JH, Hansen IM. Red Cell G-6-PD variants in Romania. Rev Enr Clin Biol 1997;17:66-9.  Back to cited text no. 18
    
19.
Green LS. G6PD deficiency as protection against falciparum malaria: An epidemiologic critique of population and experimental studies. Phys Anthrop 1993;36:153-78.  Back to cited text no. 19
    
20.
Beutler E. G6PD deficiency. Blood 1994;84:3613-36.  Back to cited text no. 20
    
21.
Beutler E. G6PD: Population genetics and clinical manifestations. Blood Rev 1996;10:45-52.  Back to cited text no. 21
    
22.
Valaes T. Severe neonatal jaundice associated with glucose-6-phosphate dehydrogenase deficiency: Pathogenesis and global epidemiology. Acta Paediatr Suppl 1994;394:58-76.  Back to cited text no. 22
    
23.
Nair PA, Al Khusaiby SM. Kernicterus and G6PD deficiency - A case series from Oman. J Trop Pediatr 2003;49:74-7.  Back to cited text no. 23
    
24.
Gaskin RS, Estwick D, Peddi R. G6PD deficiency: Its role in the high prevalence of hypertension and diabetes mellitus. Ethn Dis 2001;11:749-54.  Back to cited text no. 24
    
25.
Cramer DW, Wise LA. The epidemiology of recurrent pregnancy loss. Semin Reprod Med 2000;18:331-9.  Back to cited text no. 25
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]


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