Favizm Sonucu Gelişen Akut Böbrek Yetmezliği: Olgu Sunumu Ve Literatür Derlemesi,

Öz

Anahtar Kelimeler

• -

Favizm Sonucu Gelişen Akut Böbrek Yetmezliği: Olgu Sunumu Ve Literatür Derlemesi,

Öz

Glukoz 6 Fosfat Dehidrogenaz (G6PD) eksikliği, en sık görülen kalıtsal hastalıklardan biridir ve X’e bağlı kalıtımsal geçiş gösterir. G6PD enzimi bütün dokularda bulunur. Pentoz Fosfat Yolunun (PFY) ilk basamağını katalize eden bu enzimin eksikliğinde; infeksiyon, bazı ilaçların kullanımı veya bakla yenmesi sonrasında neonatal sarılık veya akut hemolitik anemi gelişebilir. Özellikle mitokondriyal yapıları olmayan eritrositler için PFY’u NADPH üretimi için tek kaynaktır. G6PD eksikliği olan eritrositlerde NADP’den NADPH’e dönüşüm normal düzeyde olmadığı için oksidatif hasara yatkınlık meydana gelir ve hemoliz oluşur. Oksidatif hasara uğramış olan eritrositlerde, hemolize artmış duyarlılığın nedeni tam bilinmemektedir. G6PD eksikliğine bağlı akut böbrek yetmezliği gelişebilecek olan bir komplikasyondur. Makalemizde fava yeme öyküsü sonrasında hemoliz ve akut böbrek yetmezliği gelişen; takibinde G6PD enzim eksikliği saptadığımız olgumuzu sunduk

Anahtar Kelimeler

• G6PD enzim eksikliği, favizm, hemoliz, akut böbrek yetmezliği

Kaynakça

1. Carson PE, Flanagan CL, Ickes CE, Alving AS. Enzymatic defciency in primaquine-sensitive erythro-cytes. Science 1956; 124: 484-485.
2. Gaetani GD, Parker JC, Kirkman HN. Intracellular restraint: a new basis for the limitation inresponse to oxidative stress in human erythrocytes containing lowactivity variants of glucose-6-phosphate dehydrogenase. Proceedings of the National Academy of Sciences of the USA 1974; 71:3584-3587.
3. Beutler E. The genetics of glucose-6-phosphate dehydrogenase defciency. Seminars in Hematology 1990;27:137164.
4. De FA, Benatti U, Guida L. Favism: disordered erythrocyte calcium homeostasis. Blood 1985; 66:294-297.
5. Turrini F, Naitana A, Mannuzzu L, et al. Increased red cell calcium, decreased calcium adenosinetriphosphatase, and altered membrane proteins during fava bean hemolysis in glucose – 6 – phosphatedehydrogenase - defcient (Mediterranean variant) individuals. Blood 1985; 66: 302305.
6. Tsai KJ, Shih LY, Hung IJ, et al. Enhanced vesiculation exacerbates complement-dependent hemolysis inglucose6-phosphate dehydrogenase defcient red blood cels. Life Sciences 1996; 59: 867-876.
7. Fischer TM, Meloni T, Pescarmona GP, Arese P. Membrane cross bonding in red cells in favic crisis: amissing link in the mechanism of extravascular haemolysis. British Journal of Haematology 1985; 59:159-169.
8. Jain SK. Glutathione and glucose-6-phosphate dehydrogenase defciency can increase proteinglycosylation. Free Radical Biology and Medicine 1998; 24: 197-201.

9. Bienzle U, Ayeni O, Lucas AO, Luzzatto L. Glucose-6phosphate dehydrogenase and malaria: greaterresistance of females heterozygous for enzyme defciency and of males with non-decient variant. Lancet 1972; 107-110.
10. Cappadoro M, Giribaldi G, O'Brien E, et al. Early phagocytosis of glucose-6-phosphate dehydrogenase (G6PD) - defcient erythrocytes parasitized by Plasmodium falciparum may explain malaria protection inG6PD defciency. Blood 1998; 92: 2527-2534.
11. Lewis SM, Sanders KL. Screening for G6PD by sigma kits [letter]. Clinical and Laboratory Haematology 1989; 11: 76-78.
12. Cocco P, Todde P, Fornera S, et al. Mortality in a cohort of men expressing the glucose-6-phosphatedehydrogenase defciency. Blood 1998; 91: 706-709.
13. Russo G, Mollica F, Pavone L, Schiliro G. Hemolytic crises of favism in Sicilian females heterozygousfor G-6PD defciency. Pediatrics 1972; 49: 854-859.
14. Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet. 2008;371: 64-74.
15. Galiano S, Gaetani GF, Barabino A, et al. Favism in the African type of glucose-6-phosphate dehydro-genase defciency. British Medical Journal 1990; 300:232- 236.
16. Bienzle UE, Luzzatto L. Erythrocyte glucose 6-phosphate dehydrogenase defciency (G6PDtype A¡) and neonatal jaundice. Acta Paediatrica 1976; 65: 701-703.
17. Shannon K, Buchanan GR. Severe hemolytic anemia in black children with glucose-6-phosphatedehydrogenase defciency. Pediatrics 1982; 70: 364-369.
18. Angle CR. Glucose-6-phosphate dehydrogenase defciency and acute renal failure. Lancet 1972; 134.
19. Gellady AM, Greenwood RD. G-6-PD hemolytic anemia complicating diabetic ketoacidosis. Journal of Pediatrics 1972; 80: 1037-1038.
20. Lee DH, Warkentin TE, Neame PB, Ali M. Acute hemolytic-anemia precipitated by myocardial-infarction and pericardial tamponade in g6pd defciency. American Journal of Hematology 1996; 51:174-175.
21. Kimmick G, Owen J. Rhabdomyolysis and hemolysis associated with sickle-cell trait and glucose-6-phosphatedehydrogenase defciency. Southern Medical Journal 1996; 89: 1097-1098.
22. Mordmuller B, Turrini F, Long H, et al. Neutrophils and monocytes from subjects with theMediterranean G6PD variant: e€ect of Plasmodium falciparum hemozoin on G6PD activity, oxidativeburst and cytokine production. European Cytokine Network 1998; 9: 239-245.
23. Schwartz JP, Cooperberg AA, Rosenberg A. Plateletfunction studies in patients with glucose-6-phosphate dehydrogenase defciency. British Journal of Haematology 1974; 27: 273-280.
24. Vives CJ, Feliu E, Pujades MA, et al. Severe-glucose-6phosphate dehydrogenase (G6PD) defiency associated with chronic hemolytic anemia, granulocyte dysfunction, and increased susceptibility toinfections: description of a new molecular variant (G6PD Barcelona). Blood 1982; 59: 428-434.
25. Balinsky D, Gomperts E, Cayanis E, et al. Glucose-6phosphate dehydrogenase Johannesburg: a newvariant with reduced activity in a patient with congenital nonspherocytic haemolytic anaemia. British Journal of Haematology 1973; 25: 385-392.
26. Newman TB, Maisels MJ. Evaluation and treatment of jaundice in the term newborn: a kinder, gentleapproach. Pediatrics 1992; 89: 809-818.