Glucose-6-phosphate dehydrogenase deficiency
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.D. [2]
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Glucose-6-phosphate dehydrogenase deficiency from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
Case Studies
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.
Primary Prevention
- The most important measure is prevention - avoidance of the drugs and foods that cause hemolysis.
- Vaccination against some common pathogens (e.g. hepatitis A) may prevent infection-induced attacks.
References
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