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Definition

Glucose-6-Phosphate Dehydrogenase ( G6PD ) deficiency is the most common human enzyme deficiency in the world; it affects an estimated 400 million people. G6PD deficiency is also known as "favism," since G6PD deficient individuals are also sometimes allergic to fava beans. G6PD deficiency is an allelic abnormality which is inherited in an X-linked recessive fashion. When someone has G6PD deficiency, complications can arise; hemolytic anemia and prolonged neonatal jaundice are the two major pathologies associated with G6PD deficiency. Both of these conditions are directly related to the inability of specific cell types to regenerate reduced nicotinamide adenine dinucleotide phosphate ( NADPH ); this reaction is normally catalyzed by the G6PD enzyme. A consequence of this is that G6PD deficient individuals are resistant to the malaria causing parasite.

Symptoms and signs

In G6PD deficient individuals, anemia is usually caused by certain oxidative drugs, infections, or fava beans. When any one of these agents, or their metabolites, enters a G6PD deficient red blood cell, hemoglobin becomes denatured, thus destroying its function as the principal oxygen carrying molecule. A child with G6PD deficiency who is exposed to a medication or infection that triggers the destruction of RBCs may have no symptoms at all. In more serious cases, a child may exhibit symptoms of anemia ( also known as a hemolytic crisis ), including: paleness ( in darker-skinned children paleness is sometimes best seen in the mouth, especially on the lips or tongue ), extreme tiredness, rapid heartbeat, rapid breathing or shortness of breath, jaundice, or yellowing of the skin and eyes, particularly in newborns, an enlarged spleen, dark, and tea-colored urine. In addition to being susceptible to hemolytic anemia, G6PD deficient individuals are also predisposed to prolonged neonatal jaundice. This can be a potentially serious problem as it can cause severe neurological complications and even death.

Prevention and Precaution

The most important measure is prevention - avoidance of the drugs and foods that cause hemolysis, including :

1. Do not take medication randomly (such as certain types of antibiotics, aspirin, etc.), all medications must be prescribed by a physician.
2. Avoid consuming broad beans or their products (broad bean crisps). If the mother is breastfeeding, she herself cannot eat fava beans , otherwise the child may develop acute hemolytic anemia.
3. Mothball cannot be placed in the closet and toilet.
4. Do not use gentian violet .
5. Products containing menthol or similar substances as the main pharmacological ingredients are not recommended for children with glucose-6-phosphate dehydrogenase deficiency. Similar substances include camphor, cineole, essential oils of eucalyptus, thujone, niaouli, wild thyme, pine Oleol (terpineol), terpine (terpine), citral (citral), turpentine (turpentine) and pine needle (pine needle). These substances are widely used in medicines, cosmetics, tobacco products, alcohol, tea, pesticides, detergents, candy, chewing gum, toothpaste, mouthwash, shampoo, soap, and essential oils.
6. If you find symptoms of jaundice, anemia (pale) or tea-colored (or cola-colored) urine, please take your child to the hospital as soon as possible.
7. When seeing a doctor, you should take the initiative to tell the medical staff that your child has this disease and show a G-6-PD deficiency memo card.