What is glucose hexakisphosphate dehydrogenase deficiency?
Glucose hexakisphosphate dehydrogenase (G6PD) is an enzyme that helps red blood cells work properly. Some people have lower than normal levels of glucose hexaphosphate dehydrogenase in their red blood cells. This is called glucose hexaphosphate dehydrogenase deficiency, or sericosis.
Most children with glucose hexaphosphate dehydrogenase (G6PD) deficiency usually have perfectly normal health, but sometimes it can cause problems. This article explains the problems that glucose hexaphosphate dehydrogenase (G6PD) deficiency can cause and what to do if your child has these problems. Zhang Qianshen, Department of Pediatrics, Shenzhen Maternal and Child Health Hospital
Glucose hexakisphosphate dehydrogenase (G6PD) deficiency is passed from parent to child
Glucose hexakisphosphate dehydrogenase (G6PD) deficiency is a genetic disorder, which means it is passed from one or both parents to their children and affects more boys than girls.
About 4 million people worldwide have glucose hexaphosphate dehydrogenase (G6PD) deficiency. Anyone can have glucose hexaphosphate dehydrogenase (G6PD) deficiency, but it is more common in certain populations, such as those from Africa or areas where malaria is common, such as the Mediterranean, Caribbean and Southeast Asian regions. Patients with glucose hexaphosphate dehydrogenase (G6PD) deficiency in populations of African descent are usually milder and more severe in those from the Mediterranean and some parts of Asia.
Glucose hexakisphosphate dehydrogenase (G6PD) works to protect red blood cells
Red blood cells carry oxygen to all parts of the body. Glucose hexakisphosphate dehydrogenase (G6PD) is an enzyme that helps red blood cells function properly. It also helps the body protect red blood cells against damage by outside substances when the body is hot or when certain drugs are taken.
If a person does not have enough glucose hexakisphosphate dehydrogenase (G6PD), some of the red blood cells in their body may be destroyed when they have a fever or take certain medications. The rupture of red blood cells is called hemolysis. If this happens, a person may lack enough red blood cells and become anemic.
Problems that may be caused by people with glucose hexakisphosphate dehydrogenase (G6PD) deficiency
Problems for newborns
If a mother is a carrier of the glucose hexakisphosphate dehydrogenase (G6PD) deficiency gene, she may pass it on to one or more of her children. Some newborns develop jaundice (yellowish skin) shortly after birth, and it is more likely to occur in male infants. If left untreated, severe jaundice can be severely brain toxic to newborns. Babies are usually placed under a special light for a few days to treat the condition.
Many infants are diagnosed with glucose hexakisphosphate dehydrogenase (G6PD) deficiency because of severe jaundice that develops shortly after birth. Once the jaundice subsides, there should generally be no other problems with glucose hexaphosphate dehydrogenase (G6PD) deficiency, as long as fava beans and certain medications are avoided.
Problems in children and adults
Most people with glucose hexaphosphate dehydrogenase (G6PD) deficiency have perfectly normal health, but sometimes it can cause problems.
Some children and adults with glucose hexaphosphate dehydrogenase (G6PD) deficiency may develop hemolysis and anemia when they are exposed to one of the following triggers.
1. fever.
2. use of certain medications listed later in this article.
3. eating fava beans; this is called fava bean disease.
If your child is exposed to a trigger that causes hemolysis, in more severe cases, your child can develop one or more of the following symptoms of anemia.
1. pale skin.
2. easy fatigue.
3. rapid heartbeat.
4. shortness of breath.
5. shortness of breath.
6, jaundice (yellowing of the skin and eyes).
7. back pain.
8. darkening or teal color of the urine.
If your child has any of these symptoms, go to the nearest emergency room immediately.
Symptoms of glucose hexaphosphate dehydrogenase (G6PD) deficiency usually disappear quickly once the trigger is removed or resolved, usually in a few weeks.
Certain medications that children with G6PD should not take
If your child has glucose hexakisphosphate dehydrogenase (G6PD) deficiency, he or she should not take any of the following medications.
Acetylsalicylic acid (ASA or aspirin), vitamin C, chloramphenicol, chloroquine, aminophenazone, dimercaprol, adriamycin, miparin, methylene blue, methyl, nalidixic acid, naphthalene which is the common household mothball, furantoin, primaquine, quinine, sulfacetamide, sulfadiazine, sulfonamide (cotrimoxazole), sulfanilamide, sulfapyridine, sulfisoxazole (Pediazole), toluidine blue.
This list contains only the names of drugs that are more likely to cause hemolysis in patients with glucose hexaphosphate dehydrogenase (G6PD) deficiency. Some drugs that are not on this list can also cause problems. Tell your doctor, pharmacist, or nurse that your child has glucose hexaphosphate dehydrogenase (G6PD) deficiency when you see a doctor or before you take a certain medication, and ask them to confirm that there is no effect on G6PD before giving your child any medication, including herbal remedies.
The risk and severity of hemolysis is dose-related. If your child takes high doses of these medications, hemolysis is more likely to occur and is more severe.
Your child should avoid exposure to mothballs and some foods
Your child should not be in close contact with mothballs (naphthalene).
Your child should not eat fava beans. Some people should also avoid red wine, certain legumes such as, blueberries, soy foods, tonic water, and camphor.
important points
1, G6PD is an enzyme that helps protect red blood cells.
2, Most people with G6PD deficiency are healthy, but sometimes red blood cell destruction (hemolysis) occurs with fever or when taking certain medicines and foods and leads to anemia.
3, Children with G6PD deficiency should avoid certain medicines and foods.
4. Please tell your pharmacist, nurse, or doctor that your child has G6PD deficiency.
5. If your child has symptoms of anemia, take your child to the nearest emergency room immediately.