Incidence Of Severe Glucose-6-Phosphate Dehydrogenase(G6PD) Deficiency In Countryside Villages Of The Central City Of Manisa, Turkey

Abstract

-

Keywords

• -

Incidence Of Severe Glucose-6-Phosphate Dehydrogenase(G6PD) Deficiency In Countryside Villages Of The Central City Of Manisa, Turkey

Abstract

Aim: The primary objective of this study was to determine the incidence of severe G6PD deficiency in selected countryside villages of central city of Manisa in Turkey. Secondarily to inform and protect G6PD deficient people from acute hemolytic crisis and neonatal jaundice by delivery of the updated protective food and drug list prepared in the light of the WHO- G6PD Working Committe reports. Methods: In this study, the incidence of severe G6PD deficiency were screened by Beutler\'s Fluorescence Spot test among 1604 people living in the contryside villages of central city of Manisa in Turkey. Results: Thirty five out of 1604 tested people were found to have severe G6PD deficiency. The incidence of severe G6PD deficiency were 2.2 % in sampled population. There was a difference for the incidence between male (3.2%) and female (1.14%) as expected due to X-linked heritance. There was no significant differences in the prevalence of severe G6PD deficiency between the countryside villages connected to central city of Manisa. Conclusion: The high incidence of severe G6PD deficiency implies that this inherited metabolite disorder is an important health problem in Manisa region and it is necessary to carry out large-scale screening in the whole population since severe- full G6PD deficiency related health problems are preventable. For this reason it must be included in the pool of genetic screening tests in regional health policy. Key words: Severe G6PD deficiency, fluorescence spot test, acute hemolytic anemia, Manisa, Turkey.

Keywords

• Severe G6PD deficiency, fluorescence spot test, acute hemolytic anemia, Manisa, Turkey

References

1. Beutler E. Glucose-6-phosphate
2. dehydrogenase deficiency. N Engl J Med 1991;324:169–74
3. Scott MD Luo L, Lubin BH et al. NADPH, Modulates Oxidant Senstivity in Normal and Glucose-6-Phosphate Dehydrogenase Deficient Erythrocytes. Blood 1991; 77(9):2059-64 Status
4. Mehta A, Mason PJ, Vulliamy TJ. Glucose- 6-phosphate dehydrogenase deficiency. Bailliere’s Best Pract Res Clin Haematol 2000;13(1):21–38
5. Luzzatto L, Mehta A, Vulliamy TJ. In: C.R. Scriver, A.L. Beaudet, W.S. Sly and D. Valle, Editors, The Metabolic and Molecular Basis of Inherited Disease, McGraw-Hill, New York, 2001: 4517–53
6. Beutler E. G6PD Deficiency. Blood 1994; 84 (11): 3613-36
7. WHO Working Group. Glucose-6- phosphate dehydrogenase deficiency. Bull WHO 1989;67:601–11
8. Valaes T. Severe neonatal jaundice associated with glucose-6-phosphate dehydrogenase deficiency, pathogenesis and global epidemiology. Acta Paediatr Suppl 1994;394:58–76

9. Meloni T, Forteloni G, Meloni GF. Marked decline of favism after neonatal glucose- 6-phosphate dehydrogenase screening and health education: the northern Sardinian experience. Acta Haematol 1992;87:29
10. Annual official statistical reports of Manisa city. The Health Ministry Regional Office in Manisa 1998
11. Uzunoğlu S. The investigation of Mediterranean mutation causing to severe G6PD deficiency in Aegean region by using DNA technology. Ph.D thesis. Dept. of Medical Biology. The Institute of Health Sciences. Dokuz Eylul University 1995:14-46
12. Barbette SS, Versie DJ. Modification of Neonatal Screening Test For Erythrocyte Glucose-6-Phosphate Deficiency. Clinica Chimica Acta 1976; 71:239–44 Dehydrogenase
13. Solem E, Prizer C. Mass screening for glucose-6-phosphate deficiency:improved fluorescent spot test. Clinica Chimica Acta 1985;152:135–42
14. Demircan K. The determination of normal activity values of Glucose 6-phosphate dehydrogenase in newborns and adults in Manisa region. Phd thesis. Dept of Medical Biology.The Institute of Health Sciences. Celal Bayar University. Manisa. 1999
15. Keskin N, Ozdes I, Keskin A, Acikbas I, Bagci H. Incidence and molecular analysis of glucose-6-phosphate dehydrogenase deficiency in the province of Denizli, Turkey. Med Sci Monit 2002;8(6):453
16. Dolunay M. The prevalence of G6PD deficiency and its biochemical phenotype in the central part of Antalya province. Ms.C thesis, Dept of Biochemistry. Faculty of Medicine. Akdeniz University.