Abstract
Aim
Glucose-6-Phosphate dehydrogenase (G6PD) deficiency is the most common X-linked defect of enzyme pathways in human. The aim of this study was to inspect the efficacy of G6PD deficient red cell transfusion to G6PD patients, who were admitted for hemolysis after Broad bean ingestion, and to find out whether prolong management program and retransfusion are needed as severe hemolysis would take place.
Methods
This was a cross-sectional study, which was performed on patients admitted to hospital due to hemolytic anemia after broad bean ingestion in Jahrom and Shiraz medical school hospitals on February and March 2007. 2ml samples of transfusion blood bags, were sent to the laboratory in order to investigate the G6PD enzyme sufficiency. Hemoglobin level was checked six hours after blood transfusion. We compared the amount of hemoglobin level elevation after transfusion in patients received G6PD sufficient and G6PD deficient red cell using independent sample t-test.
Result
In 114 transfused bags, 14 bags (12.3%) were G6PD deficient. We divided patients into two groups. Group A received G6PD sufficient red cell and group B were transfused with G6PD deficient red cell. Hemoglobin level was significantly raised after transfusion of red cell in each group. The amount of hemoglobin elevation was more in group A in comparison to group B.
Conclusion
Based on our study results, proper hemoglobin level would be achieved using non G6PD deficient red cell for transfusion to G6PD deficient patients.
Keywords: Glucose-6-Phosphate dehydrogenase enzyme, G6PD deficiency, Hemolysis, Blood transfusion
Full Text
The Full Text of this article is available as a PDF (498.5 KB).
References
- 1.Bender A.D., Kethleen M.B., Daryl K.G., Frederick W.K., Peter J.K., Peter A.M. Harper’s Illustrated Biochemistry. 26th edition. New York: Mcgraw-Hill; 2003. pp. 612–614. [Google Scholar]
- 2.Bonilla J.F., Quim L., Sanchez M.C., Chuaire L. Glucose-6-phosphate dehydrogenase (G6PD). Response of the human erythrocyte and another cells to the decrease in their activity. Colombia Medica. 2007;38:76–83. [Google Scholar]
- 3.Luzzatto L. Nathan and Oskie’s hematology of infancy and childhoods. 5th edition. Philadelphia: W B Saunders; 1998. Glucose-6-phosphate dehydrogenase deficiency and hemolytic anemia; pp. 704–725. [Google Scholar]
- 4.Mehta A., Mason P.J., Vulliamy J.J. Glucose-6-phosphate dehydrogenase Deficiency. Baillieres Best pract Res clin Haemathol. 2000;13:21–38. doi: 10.1053/beha.1999.0055. [DOI] [PubMed] [Google Scholar]
- 5.Behrman R., Kliegman R., Jenson H. Nelson Text Book of Pediatric. 17th edition. Philadelphia: Sannders; 2004. pp. 1636–1638. [Google Scholar]
- 6.Scriver C.R., Beaudet A.L., Sly W.S., Valle D. The metabolic and molecular bases of inherited disease. 7th edition. New York: Mc Grow-Hill, Inc; 1995. pp. 3367–3398. [Google Scholar]
- 7.Filolsa S., Giacometti N., Wangwei C., Mattia D., Pagnini D., Alfinito F., et al. Somatic cell selection is a major determinant of the blood cell phenotype in heterozygotes for glucose-6-phosphate dehydrogenase mutations causing severe enzyme deficiency. Am J Hum Genet. 1996;59:887–895. [PMC free article] [PubMed] [Google Scholar]
- 8.Pishva N., Amoozegar H. Hyperbilirubinemia following Exchange transfusion with G-6-PD deficient donor blood. Iran J Med Sci. 2001;26:384–387. [Google Scholar]
- 9.Shalev O., Manny N., Sharon R. Post transfusional hemolysis in recipients of glucose-6-phpsphate dehydrogenase-deficient erythrocytes. Vox Sang. 1993;64:94–98. doi: 10.1111/j.1423-0410.1993.tb02525.x. [DOI] [PubMed] [Google Scholar]
- 10.Missiou T., Sagaraki S. Screening and management of potentially treatable Genetic Metabolic Disorder. Pediat. 1991;119:293–299. doi: 10.1016/S0022-3476(05)80747-4. [DOI] [Google Scholar]
- 11.Gaskin R.S., Estwick D., Peddi R. G6PD deficiency: Its role in the high prevalence of hypertension and diabetes mellitus. Ethn Dis. 2001;11:749–754. [PubMed] [Google Scholar]
- 12.Karimi M., Montemuros F.M., Danielli M.G., Farjadian S., Afrasiabi A., Fiorelli G., et al. Molecular characterization of glucose-6-phosphate dehydrogenase deficiency in the Fars Province of Iran. Haematologica. 2003;88:346–347. [PubMed] [Google Scholar]
- 13.Doxiadis S.A., Fessas P.H., Valaes T. Erythrocyte enzyme deficiency in unexplained kernicterus. Lancet. 1960;2:44. doi: 10.1016/S0140-6736(60)92688-X. [DOI] [Google Scholar]
- 14.Missiou-Tsagaraki S. Screening for glucose-6-phosphate dehydrogenase deficiency as a preventive measure: prevalence among 1,286,000 Greek newborn infants. J Pediatr. 1991;119:293–299. doi: 10.1016/S0022-3476(05)80747-4. [DOI] [PubMed] [Google Scholar]
- 15.Wolf B.H., Weaning R.S., Schutgens R.B., Noorden C.J., Vogels I.M.C., Nagelkerke N.J.D. Detection of glucose-6-phosphate dehydrogenase deficiency in erythrocytes: A spectrophotometric assay and a fluorescent spot test compared with a cytochemical method. Clin Chim Acta. 1987;168:129–136. doi: 10.1016/0009-8981(87)90281-6. [DOI] [PubMed] [Google Scholar]
- 16.Missiou-Tsagaraki S. Screening for glucose-6-phosphate dehydrogenase deficiency as a preventive measure: prevalence among 1,286,000 Greek newborn infants. J Pediatr. 1991;119:293–299. doi: 10.1016/S0022-3476(05)80747-4. [DOI] [PubMed] [Google Scholar]
- 17.Who Working Group Word Map of G-6-PD deficiency Bull WHO 1989676012633878 [Google Scholar]
