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Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD Deficiency)

An inherited disorder in which a person doesn't have enough of an enzyme called G6PD that helps red blood cells work the way they should. Also called G6PD deficiency.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About G6PD Deficiency

In glucose-6-phosphate dehydrogenase deficiency, the red blood cells break down when the body is exposed to infection, severe stress, or certain drugs, chemicals, or foods. This may lead to a condition called hemolytic anemia. This disorder is most common in African-American men and in men of Middle Eastern or Mediterranean descent. NIH - National Cancer Institute

What works? Research summarized

Evidence reviews

Should blood donors be routinely screened for glucose-6-phosphate dehydrogenase deficiency? A systematic review of clinical studies focusing on patients transfused with glucose-6-phosphate dehydrogenase-deficient red cells

The risk factors associated with the use of glucose-6-phosphate dehydrogenase (G6PD)-deficient blood in transfusion have not yet been well established. Therefore, the aim of this review was to evaluate whether whole blood from healthy G6PD-deficient donors is safe to use for transfusion. The study undertook a systematic review of English articles indexed in COCHRANE, MEDLINE, EMBASE, and CINHAL, with no date restriction up to March 2013, as well as those included in articles' reference lists and those included in Google Scholar. Inclusion criteria required that studies be randomized controlled trials, case controls, case reports, or prospective clinical series. Data were extracted following the Preferred Reporting Items for Systematic Reviews using a previously piloted form, which included fields for study design, population under study, sample size, study results, limitations, conclusions, and recommendations. The initial search identified 663 potentially relevant articles, of which only 13 studies met the inclusion criteria. The reported effects of G6PD-deficient transfused blood on neonates and children appear to be more deleterious than effects reported on adult patients. In most cases, the rise of total serum bilirubin was abnormal in infants transfused with G6PD-deficient blood from 6 hours up to 60 hours after transfusion. All studies on neonates and children, except one, recommended a routine screening for G6PD deficiency for this at-risk subpopulation because their immature hepatic function potentially makes them less able to handle any excess bilirubin load. It is difficult to make firm clinical conclusions and recommendations given the equivocal results, the lack of standardized evaluation methods to categorize red blood cell units as G6PD deficient (some of which are questionable), and the limited methodological quality and low quality of evidence. Notwithstanding these limitations, based on our review of the available literature, there is little to suggest that G6PD-deficient individuals should be excluded from donating red blood cells, although transfusions of such blood may potentially have negative impacts on premature neonates or patients who need repeated transfusions, and thus, for this group, screening for G6PD deficiency may be appropriate.

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Terms to know

Enzymes
Protein made by the body that brings about a chemical reaction - for example, the enzymes produced by the gut to aid digestion.
Erythrocytes (Red Blood Cells)
A cell that carries oxygen to all parts of the body.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Glucose (Dextrose)
A simple sugar the body manufactures from carbohydrates in the diet. Glucose is the body's main source of energy.
Phosphate
A form of phosphoric acid, which contains phosphorus. In the body, phosphates are found in the bones and teeth. Phosphates may be used to treat a high level of calcium in the blood. Adding or removing phosphate chemical groups may affect the way proteins act in the body.

More about Glucose-6-Phosphate Dehydrogenase Deficiency

Photo of an adult man

Also called: Deficiency of G-6PD, Deficiency of glucose-6-phosphate dehydrogenase, G6PD

See Also: Hemolytic Anemia

Other terms to know: See all 5
Enzymes, Erythrocytes (Red Blood Cells), Genetic

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