Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme deficiency worldwide. It is particularly prevalent in Africa, the Middle East, and Southeast Asia. G6PD deficiency is an X-linked genetic disorder, meaning it is more likely to affect males than females. Females must inherit two defective copies of the gene to be affected, whereas a male with one defective gene will definitely have G6PD deficiency.
Favism Disease and Its Symptoms
A specific manifestation of G6PD deficiency is known as favism. Favism disease occurs after the consumption of certain foods, particularly fava beans, which can trigger a severe reaction in individuals with G6PD deficiency. Favism symptoms include:
- Sudden rise in body temperature
- Yellowing of the skin and mucous membranes (jaundice)
- Dark yellow-orange urine
- Pallor, fatigue, and general physical deterioration
- Rapid, heavy breathing
- Weak, rapid pulse
How is G6PD Deficiency and Favism Diagnosed?
A simple blood test measures the amount of G6PD enzyme in red blood cells. Decreased levels indicate reduced G6PD activity. Favism disease is identified through clinical symptoms and confirmed with laboratory tests. G6PD screening is essential for newborns and is included in Medlabs’ Neonatal Screening Test.
Favism Treatment and Management of G6PD Deficiency
If you notice any symptoms associated with G6PD deficiency or favism, contact your physician immediately. Severe hemolytic crises, which are common in favism, are typically treated with blood transfusions. The best therapy is to avoid prohibited drugs and certain foods.
Favism Foods to Avoid and Medications to Avoid
1- Antibiotics:
- Sulfanilamide
- Sulfapyridine
- Sulfadimidine
- Sulfacetamide
- Glucosulfone sodium
- Nitrofurantion
- Furazolidone
- Nitrofurazone
- Dapsone
- Sulfoxone
- Sufisoxazole
2. Antimalarials:
- Primaquine
- Pamaquine
- Chloroquine
3. Anthelmintics:
- B-Naphthol
- Stibophen
- Nitidazole
4. Miscellaneous:
- Probenecid
- Thiazide Diuretics
- Phenothiazine
- Chloramphnicol
- Orinase
- Dimercaptol
- Methylene blue
- Naphthalene (mothballs)
- Vitamin K
5. Analgesics (Containing aspirin or phenacetin):
- Aspirin
- Bufferin
- Anacin
- Exedrin
- Empirin
- APC Tablets
- Darvon Compound
- Coricidin
6. Food:
- Favism Foods to Avoid: Fava beans
Complications of G6PD Deficiency and Favism:
While symptoms can be observed, complications are often confirmed through laboratory tests. Common complications include:
-
Anemia:
Anemia can result from the ingestion of certain oxidative medications, fava beans, or infections like hepatitis, typhoid, and pneumonia, leading to the destruction of red blood cells.
-
Jaundice:
Jaundice occurs when red blood cells break down, causing yellowing of the skin, the whites of the eyes, and mucous membranes. This happens due to the deposition of bile salts in tissues, a direct result of reduced enzyme activity in the liver.