Learn About Glucose-6-Phosphate Dehydrogenase Deficiency

What is the definition of Glucose-6-Phosphate Dehydrogenase Deficiency?

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a condition in which red blood cells break down when the body is exposed to certain drugs or the stress of infection. It is hereditary, which means it is passed down in families.

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What are the alternative names for Glucose-6-Phosphate Dehydrogenase Deficiency?

G6PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiency

What are the causes of Glucose-6-Phosphate Dehydrogenase Deficiency?

G6PD deficiency occurs when a person is missing or does not have enough of an enzyme called glucose-6-phosphate dehydrogenase. This enzyme helps red blood cells work properly.

Too little G6PD leads to the destruction of red blood cells. This process is called hemolysis. When this process is actively occurring, it is called a hemolytic episode. The episodes are most often brief. This is because the body continues to produce new red blood cells, which have normal activity.

Red blood cell destruction can be triggered by infections, certain foods (such as fava beans), and certain medicines, including:

  • Antimalarial medicines such as quinine
  • Aspirin (high doses)
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Quinidine
  • Sulfa drugs
  • Antibiotics such as quinolones, nitrofurantoin

Other chemicals, such as those in mothballs, can also trigger an episode.

In the United States, G6PD deficiency is more common among blacks than whites. Men are more likely to have this disorder than women.

You are more likely to develop this condition if you:

  • Are African American
  • Are of Middle Eastern decent, particularly Kurdish or Sephardic Jewish
  • Are male
  • Have a family history of the deficiency

A form of this disorder is common in whites of Mediterranean descent. This form is also associated with acute episodes of hemolysis. Episodes are longer and more severe than in the other types of the disorder.

What are the symptoms of Glucose-6-Phosphate Dehydrogenase Deficiency?

People with this condition do not display any signs of the disease until their red blood cells are exposed to certain chemicals in food or medicine.

Symptoms are more common in men and may include:

  • Dark urine
  • Fever
  • Pain in abdomen
  • Enlarged spleen and liver
  • Fatigue
  • Pallor
  • Rapid heart rate
  • Shortness of breath
  • Yellow skin color (jaundice)
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What are the current treatments for Glucose-6-Phosphate Dehydrogenase Deficiency?

Treatment may involve:

  • Medicines to treat an infection, if present
  • Stopping any drugs that are causing red blood cell destruction
  • Transfusions, in some cases
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What is the outlook (prognosis) for Glucose-6-Phosphate Dehydrogenase Deficiency?

In most cases, hemolytic episodes go away on their own.

What are the possible complications of Glucose-6-Phosphate Dehydrogenase Deficiency?

In rare case, kidney failure or death may occur following a severe hemolytic event.

When should I contact a medical professional for Glucose-6-Phosphate Dehydrogenase Deficiency?

Contact your health care provider if you have symptoms of this condition.

Contact your provider if you have been diagnosed with G6PD deficiency and symptoms do not disappear after treatment.

How do I prevent Glucose-6-Phosphate Dehydrogenase Deficiency?

People with G6PD deficiency must strictly avoid things that can trigger an episode. Talk to your provider about your medicines.

Genetic counseling or testing may be available to those who have a family history of the condition.

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What are the latest Glucose-6-Phosphate Dehydrogenase Deficiency Clinical Trials?
Evaluation of the STANDARD G6PD Rapid Test for Assaying the Enzymatic Activity of G6PD in French Guiana

Summary: In French Guiana, malaria is endemic and two species predominate: P. falciparum and P. vivax. The treatments against Plasmodium vivax malaria are: nivaquine for 3 days against circulating blood parasites and primaquine for 14 days against parasites dormant in the liver. Primaquine can cause iatrogenic hemolytic anemias in patients with favism, i.e. G6PD deficiency. This anemia can be severe enough...

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The Association Between Oxidative Stress and Carbohydrate Metabolism Disorders in G6PD Deficient Individuals

Summary: The purpose of this study is to investigate the effects of alpha-lipoic acid supplementation on redox status, physiological and biochemical parameters in diabetic individuals with G6PD deficiency, after acute exercise.

What are the Latest Advances for Glucose-6-Phosphate Dehydrogenase Deficiency?
CT-058 Outcome of Chemotherapy in G6PD-Deficient Patients.
Tafenoquine for the treatment of Plasmodium vivax malaria.
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Neuroprotective effect of dapsone in patients with aneurysmal subarachnoid hemorrhage: a prospective, randomized, double-blind, placebo-controlled clinical trial.
Who are the sources who wrote this article ?

Published Date: January 25, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Gregg XT, Prchal JT. Red blood cell enzymopathies. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 44.

Lissauer T, Carroll W. Haematological disorders. In: Lissauer T, Carroll W, eds. Illustrated Textbook of Paediatrics. 5th ed. Philadelphia, PA: Elsevier; 2018:chap 23.

Michel M. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 151.