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G6PD Deficiency
Aka: G6PD Deficiency, G6PD, Favism
- See Also
- Hemolytic Anemia
- Epidemiology
- Caucasians of Mediterranean ancestry (Class II)
- Greek patients
- Jewish patients
- Sardinian patients (from island of Italy)
- Black patients or African descent (Class III)
- Asian patients
- Filipino patients
- Pathophysiology
- Glucose-6-phosphate dehydrogenase (G6PD)
- Catalyzes NADP to NADPH (pentose phosphate path)
- NADPH prevents oxidative damage to cells
- RBCs depend on G6PD for sole pathway to NADPH
- RBCs are most susceptible to insufficient G6PD
- Methemoglobin (Fe3+) may form spontaneously
- Methemoglobin is typically reduced to normal Hemoglobin (Fe2+) by cellular mechanisms
- NADPH is important in combating oxidative stress and converting Hemoglobin back to Fe2+
- However in G6PD Deficiency, this fails and Methemoglobinemia is unchecked
- Results in acute Hemolytic Anemia
- Drug-induced Hemolysis affects older cells
- Younger cells have adequate enzyme levels to survive
- G6PD mutations occur on distal long arm of C Chromosome
- X -Linked Disorder
- Causes
- See Medications in G6PD Deficiency
- Onset within 72 hours of intake
- Infection (most common cause)
- Salmonella
- Eschirichia coli
- Beta-hemolytic Streptococcus
- Rickettsia
- Viral Hepatitis
- Influenza A
- Fava beans (Italian Broad Beans)
- Bell Beans
- Broad Beans
- English Dwarf Beans
- Haba Beans
- Horse Beans
- Pigeon Beans
- Silkworm Beans
- Tic Beans
- Symptoms and Signs
- Usually asymptomatic
- See Hemolytic Anemia for acute episodes
- Differential Diagnosis
- See Drug-Induced Hemolytic Anemia
- See Hemolytic Anemia Causes
- Labs
- See Hemolytic Anemia
- See G6PD Deficiency Detection
- Complications
- Increased risk of Sepsis
- Prevention
- Avoid precipitating factors
- See Medications in G6PD Deficiency
- Keep Immunizations up-to-date
- Treat Anemia following Hemolytic Anemia episode
- Iron Supplementation
- Folic Acid supplementation
- Measures that are not recommended (no benefit)
- Splenectomy
- Vitamin E
- Selenium
- References
- Golan in Goldman (2000) Cecil Medicine, p. 873-75
- Beutler (1994) Blood 84:3613-36 [PubMed]
- Frank (2005) Am Fam Physician 72:1277-82 [PubMed]
- Phillips (2018) Am Fam Physician 98(6): 354-61 [PubMed]