G6PD Deficiency information

Knowing『G6PD Deficiency』

Midify Date: 10th March 2018

The name of "bean disease" is "glucose hexaphosphate dehydrogenase deficiency", which means that the body lacks an enzyme that helps stabilize red blood cells, and the ability to protect red blood cells is reduced. This enzyme is called "glucose hexaphosphate dehydrogenase." Is "Glucose-6-Phosphate Dehydrogenase", it is also referred to as "G6PD deficiency".

"Faba bean disease" is a metabolic genetic disease that is congenital and does not infect other people. It belongs to sex-linked recessive inheritance. Due to the mutation in the single gene of the X chromosome, G6DP cannot function normally to maintain the NADPH (Nicotinamide adenine dinucleotide phosphate) content in the blood, and sufficient NADPH content can protect the red blood cells from the destruction of the oxidation reaction due to the lack of normal G6DP. In the case of certain oxides, red blood cells are vulnerable to destruction.

Therefore, when patients with faba bean disease experience external stimuli such as broad beans, stinky boluses or serious infections, red blood cells can be destroyed and dissolved, causing acute hemolysis and impaired health, especially for newborn babies. Cause permanent damage, because acute hemolysis can cause a large amount of hemoglobin to be released from the red blood cells, causing excessive hepatic load and excessive bilirubin to form jaundice. Although there is currently no medical method to cure "bean disease," patients should avoid contact with substances that can cause hemolysis for the rest of their lives. Health conditions are the same as ordinary people.

"Faba bean disease" is common in southern China and it is also the most common genetic disease in Hong Kong. Since 1984, all newborn babies born in Hong Kong will be provided with a screening service for "faba bean disease," which is provided free of charge by the Medical Genetic Services Genetic Screening Unit of the Department of Health. It will be possible to know whether or not the baby is ill by taking a cord blood test. According to data from the Department of Health from 1984 to 2016, the total discovery rate of faba bean disease for newborn babies born in Hong Kong was 2.58% for male infants and 0.22% for female infants.

Because of the gender of the baby, it depends on the combination of two sex chromosomes (X or Y): XX is a female and XY is a male. One of the combinations is from the mother and the other from the father. Although faba bean disease is caused by single-gene mutations on the X chromosome, normal X chromosomes do not cause disease. Therefore, there are more male patients (XY) than female patients (XX).

 

source:

Hong Kong Department of Health Medical Genetic Services Genetic Screening Unit

Data from the Department of Health is provided by "Datalink": https://data.gov.hk/tc/

Glucose-6-Phosphate Dehydrogenase Deficiency: www.pathophys.org/g6pd/

"G6PD Deficiency" acute hemolytic symptoms and its causes

Modify Date: 20th March 2018

"Hard bean disease" with acute haemolytic symptoms

  • It is pale, and it emits brown urine (like dark Puerh tea).

  • Newborn baby: Astragalus (yellow skin and white spots), appear to be inactive, and excessively thirsty.

  • Children or adults: Feeling tired, having a rapid heartbeat and shortness of breath.

**If you have any of these symptoms, seek medical advice immediately**

 

Causes the acute hemolytic origin of faba bean disease

  • Bacterial or viral infections (eg upper respiratory tract infections)

  • Eating broad beans and their finished products

  • Contact smell ball (made from naphthalene)

  • Taking certain Western medicines, including certain anti-fever medicines, certain antibiotics, certain antimalarial medicines (furans/sulfa drugs)

  • Use of certain traditional Chinese medicines, including Chinese wolfberry, honeysuckle, flower bloom, bezoar, pearl powder and finished products of traditional Chinese medicine

 

Source:

  1. Hong Kong Department of Health Medical Genetic Services Genetic Screening Unit

  2. Glucose-6-Phosphate Dehydrogenase Deficiency: www.pathophys.org/g6pd/

"Faba bean" contraband

Modify Date: 22 March 2018

If the disease of broad bean disease is eaten/contacted with “contraband products”, it will cause pathological reaction, but depending on individual patients with different degrees of G6PD deficiency, there will be different degrees of response. The response of infants and children is also more sensitive than that of adults. Because the former has a small amount of blood, the intake of infants and young children for the same amount of “contraband” intake is relatively large. Therefore, if the hemolytic reaction is initiated, it is relatively dangerous

 

 

Broad bean: In contrast, eating fresh faba bean is more serious than hemolysis caused by processed faba bean, but there are a lot of faba bean products hidden in the market, so we need to pay attention.

  • Broad bean crisp, mixed beans

  • Some brands of bean paste, XO sauce

  • Some brand fans, instant noodles seasoning powder

 

medicine:

  • Aspirin, certain antipyretics, certain antibiotics, certain antimalarials

  • Vitamin C (high dose), vitamin K

 

Chinese MEdicine:Lianhua, honeysuckle, bloom, bezoar, pearl powder and its traditional Chinese medicines

  • Bao Ying Dan, Monkey Zao San, Watermelon Frost, Yin Qiao San, Qi Feng San

  • Wuhuacha, battered wine

 

Menthol/Menthol, Camphor, Citronella Oil:Mainly refers to synthetic products, natural mint, natural eucalyptus, natural citronella, etc. have no effect.

  • Menthol, camphor: Most external oils, safe cream, antipyretic, analgesic/analgesic

  • Citronella Oil: Mostly used for insect repellent products

  • Naphthalenes, Camphors: Odor pills, most pest control tablets, aerosol insecticides

 

Pigment:

  • Blue Potion, Purple Potion

  • Haining Grass: Indian Painted Henna, Hair Coloring Agent

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