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Hemolytic Anemia in G6PD Patients Essay Example

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Hemolytic Anemia in G6PD Patients

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Hemolytic Anemia in G6PD Patients. However, assessment of the normal and variant enzymes under pretended physiologic conditions, with the effects of various intermediate metabolites and co-enzymes in red cells being taken into consideration, reveal that the G6PDs from hemolytic variant subjects are strongly inhibited by a physiologic concentration of NADPH because of their high Michaelis constant for NADP or low inhibition constant for NADPH, and they are more sensitive to inhibition by ATP. These variant enzymes cannot produce enough NADPH in red cells to uphold an adequate concentration of concentrated and reduced glutathione. The nonhemolytic variant enzymes are far less susceptible to the inhibition by NADPH because of their low Michaelis constant for NADP and high inhibition constant for NADPH.

The physiologic action of these nonhemolytic variant enzymes is expected to be more than 30 percent of the activity of the normal G6PD, and this activity is adequate to maintain the red cells unhemolyzed.But as there was a history of fava beans ingestion, a more appropriate diagnosis was Glucose 6 Phosphate Dehydrogenase Deficiency as in these cases some oxidative stress like fava beans ingestion can trigger hemolytic episodeDirect demonstration of G6PD levels were done which show decreased level of the enzymes and the tests to demonstrate this deficiency were done about 3months after the episode of acute hemolysis as during the episode false negative results can lead to misdiagnosis as reticulocytes being rich in the enzyme can give false interpretation.The boy was immediately taken to the hospital where he was given blood cell transfusion to treat the severe anemia and initially injectable steroids were given to decrease the inflammatory response and then the boy was discharged on oral steroid for five days.The plasmodium utilizes NADPH from the pentose phosphate pathway for its metabolism but it is quite deficient in G6PD deficient individuals who slow down the development of the parasite in G6PD deficient patients but after some time the plasmodium starts to produce its own enzyme so adapting to the conditions of decreased Piet, G6PDD g6pddeficiency. Detailed. Hemolytic Anemia in G6PD Patients.

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References

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1) Marijn, Schuurman., Dick van, Waardenburg., Joost Da, Costa., Hendrik, Niemarkt., & Piet, Leroy. (2008). Pediatrics. European Journal of Pediatrics, 779- 782. Retrieved May 4, 2010, from http://www.springerlink.com/content/n2277x5732j31465/

2) Wikipedia: The Free Encyclopedia: Glucose-6-phosphate dehydrogenase deficiency. Retrieved May 4, 2010, from Wikipedia site: http://en.wikipedia.org/wiki/Glucose-6-phosphate_dehydrogenase_deficiency#Signs_and_symptoms

3) Family Practice notebook.com: G6PD Deficiency. Retrieved May 4, 2010, from http://www.fpnotebook.com/Hemeonc/Hemolysis/G6pdDfcncy.htm

4) Mark, A. Belsey. (1973). The Epidemiology of Favism. Retrieved May 4, 2010, from http://whqlibdoc.who.int/bulletin/1973/Vol48/Vol48-No1/bulletin_1973_48%281%29_1-13.pdf

5) Medical Biochemistry Page. Introduction. Retrieved May 5, 2010, from http://themedicalbiochemistrypage.org/pentose-phosphate-pathway.html

6) Case Report. Acute massive haemolysis in children with glucose-6-phosphate dehydrogenase deficiency. Retrieved May 5, 2010, from http://www.hkmj.org/article_pdfs/hkm0604p149.pdf

7) G6PDD g6pddeficiency.org. Detailed Description of Classic G6PD Deficiency Hemolysis in the Red Blood Cells. Retrieved May 5, 2010, from http://g6pddeficiency.org/index.php?cmd=researchviewarticle&id=27

8) Botanical. Broad Bean (Broad Bean Toxicity). Retrieved May 5, 2010, from http://www.botanical-online.com/alcaloidesfavaangles.htm

9) Food and G6PD Deficiency. Retrieved May 5, 2010, from http://www.vigifavisme.com/en/aliments.html

10) Human Adaptability. Glucose-6-Phosphate Dehydrogenase (G6PD) and Malaria. Retrived May 6, 2010, from http://www.as.ua.edu/ant/bindon/ant475/g6pd/g6pd.htm

11) Medical Biochemistry Page. Introduction. Retrived May 6, 2010, from http://themedicalbiochemistrypage.org/pentose-phosphate-pathway.html

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