Glucose-6-phosphate dehydrogenase deficiency medical therapy: Difference between revisions
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'''Glucose-6-phosphate dehydrogenase (G6PD) deficiency''' is an [[Sex-linked|X-linked recessive]] [[hereditary disease]] featuring abnormally low levels of the [[G6PD]] enzyme, which plays an important role in [[red blood cell]] function. Individuals with the disease may exhibit non-immune [[hemolytic anemia]] in response to a number of causes. It is closely linked to '''[[favism]]''', a disorder characterized by a hemolytic reaction to consumption of [[Vicia faba|broad bean]]s, with a name derived from the [[Italian language|Italian]] name of the broad bean (''fava''). Sometimes the name, [[favism]], is alternatively used to refer to the enzyme | '''Glucose-6-phosphate dehydrogenase (G6PD) deficiency''' is an [[Sex-linked|X-linked recessive]] [[hereditary disease]] featuring abnormally low levels of the [[G6PD]] enzyme, which plays an important role in [[red blood cell]] function. Individuals with the disease may exhibit non-immune [[hemolytic anemia]] in response to a number of causes. It is closely linked to '''[[favism]]''', a disorder characterized by a hemolytic reaction to consumption of [[Vicia faba|broad bean]]s, with a name derived from the [[Italian language|Italian]] name of the broad bean (''fava''). Sometimes the name, [[favism]], is alternatively used to refer to the enzyme | ||
deficiency as a whole. | deficiency as a whole. | ||
==Treatment== | |||
* In the acute phase of hemolysis, [[blood transfusion]]s might be necessary. Blood transfusion is an important symptomatic measure, as the transfused red cells are generally not G6PD deficient. | |||
* [[Dialysis]] in [[acute renal failure]] | |||
* Some patients benefit from removal of the [[spleen]] ([[splenectomy]]), as this is an important site of [[red blood cell]] destruction. * [[Folic acid]] should be used in any disorder featuring a high [[red blood cell]] turnover. | |||
* Although [[vitamin E]] and [[selenium]] have antioxidant properties, their use does not decrease the severity of [[G6PD deficiency]]. | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
Revision as of 18:14, 21 September 2012
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Glucose-6-phosphate dehydrogenase deficiency Microchapters |
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Differentiating Glucose-6-phosphate dehydrogenase deficiency from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Glucose-6-phosphate dehydrogenase deficiency medical therapy On the Web |
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CDC on Glucose-6-phosphate dehydrogenase deficiency medical therapy |
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Glucose-6-phosphate dehydrogenase deficiency medical therapy in the news |
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Directions to Hospitals Treating Glucose-6-phosphate dehydrogenase deficiency |
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Risk calculators and risk factors for Glucose-6-phosphate dehydrogenase deficiency medical therapy |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.D. [2]
Overview
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked recessive hereditary disease featuring abnormally low levels of the G6PD enzyme, which plays an important role in red blood cell function. Individuals with the disease may exhibit non-immune hemolytic anemia in response to a number of causes. It is closely linked to favism, a disorder characterized by a hemolytic reaction to consumption of broad beans, with a name derived from the Italian name of the broad bean (fava). Sometimes the name, favism, is alternatively used to refer to the enzyme deficiency as a whole.
Treatment
- In the acute phase of hemolysis, blood transfusions might be necessary. Blood transfusion is an important symptomatic measure, as the transfused red cells are generally not G6PD deficient.
- Dialysis in acute renal failure
- Some patients benefit from removal of the spleen (splenectomy), as this is an important site of red blood cell destruction. * Folic acid should be used in any disorder featuring a high red blood cell turnover.
- Although vitamin E and selenium have antioxidant properties, their use does not decrease the severity of G6PD deficiency.