(1) Fava bean disease, known as glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, is the most common hereditary enzyme deficiency disease. Fava bean disease, due to hereditary erythrocyte glucose-6-phosphate dehydrogenase deficiency, is an acute hemolytic disease caused by rupture of erythrocytes after exposure to fava bean pollen, ingestion of fava beans and their products, or administration of highly oxidizing drugs.

(2) Characteristics of fava bean disease: rapid onset, rapid progression, more serious; prevalent in late spring and early summer (April-July) when fava beans are on the market; prevalent in the Yangtze River Basin as well as provinces south of the Yangtze River Basin, such as Guangdong Province; prevalent in males, "preferring males to females", and more prevalent in children under the age of 9;

(3) Fava bean disease common symptoms: hemolytic reaction, anemia, chills, pallor, headache and fever, etc.; gastrointestinal symptoms, such as loss of appetite, vomiting, vomiting, abdominal pain, and even hepatosplenomegaly, etc.; urinary symptoms, the urine is a thick tea or soy sauce, and in severe cases, renal failure;

(4) Drugs that should be avoided in daily life: 1) Prohibited drugs: sulfonamides: sulfadiazine pyrimethamine, etc.; nitrofurans: furazolidone (dysentery), furotoxin, furacilin, etc.; anti-malarials: primaquine; other drugs: large doses of vitamin C and vitamin K, phenylhydrazine, naphthalene-containing camphor, toluidine blue, thiazolone, etc.; Chinese herbal medicine ingredients: berberine (Berberine), peppermint, Chuan Huanglian, Pearl Powder, Senna, Niu Huang, Bear's Bile, Bao Ying Dan, etc. (2) caution in the use of drugs, such as antipyretic and analgesic drugs: acetaminophen, aminophen, aspirin, finasteride, indomethacin (anti-inflammatory pain), etc.; anti-malarials: quinine, chloroquine, ethylamine pyrimethamine, etc.; sulfone drugs: aminophosgene sulfone, Procodone; sulfonamides: sulfadiazine, cotrimoxazole, sulfisoxazole, sulfadimethoxine, etc.; Other drugs, such as cetirizine, phenelzine, levodopa, probenecid, colchicine, phenelzine, glibenclamide, paracetamol, phenytoin sodium, and so on;

(5) sericea disease patients should pay attention to: 1) should be prohibited from eating broad beans and their products; 2) avoid in the broad bean flowering, fruiting and harvesting season to go to the broad bean field; 3) breastfeeding women should be prohibited from eating broad beans, so as to avoid the affected breastfeeding children due to sucking the milk and the onset of the disease; 4) medical use of medication, you should be their own sericea disease patients to inform the identity of the doctor or pharmacist; 5) to comply with the doctor's instructions or consult the pharmacist to use the medication or the patient in the drug before Should read the instructions carefully before using the medicine; 6) Avoid using camphor pills containing naphthalene to expel worms; 7) When the patient is injured, do not use disinfectant containing gentian violet, such as violet drops; 8) Whenever dizziness, fatigue, fever, nausea and vomiting, abdominal pain and other symptoms occur after contacting or eating fava beans, fava bean products or oxidizing medicines, should immediately seek medical attention to clarify whether it is an attack of fava bean disease and be given the appropriate treatment.

The authoritative interpretation of Pharmaceutical Affairs, unauthorized reproduction, plagiarism will be punished.

I'm pediatrician Dr. Nan Fish and I'm happy to answer your questions.

1, this disease, as the name suggests, related to fava beans, is a kind of eating fava beans caused by acute hemolytic anemia, 1-7 years old babies, mainly because of the lack of glucose-6-phosphate dehydrogenase (G-6-PD), will destroy the stability of the body's red blood cells. (Seems like you suddenly can't face the delicious fava beans with a straight face).

2, after getting serotonin disease, the baby is usually the same as normal children, there is no discomfort and symptoms, and there is no abnormal change in appearance. Once the onset of the disease, there will be jaundice, anemia, pallor, urine is soy sauce color and other symptoms, and even cause multiple organ failure to death, is a very sinister disease.

3. Basically, the disease is not transmitted to men but to women, and the ratio of men to women is as high as 7:1.

4. At present, sericulture can not be corrected with medication, or to prevent the best. If there is a family history of the disease, it is advisable for parents to give birth to a daughter rather than a son.

5. If a baby boy is born, the baby must not be allowed to eat fava beans or certain oxidizing drugs.

Fish Xiaonan: 80s pediatrician mom, attending physician at Children's Hospital of Zhejiang University School of Medicine.

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Fava bean disease is a condition that causes acute intravascular hemolysis in some children with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency after eating fava beans or fava bean products such as vermicelli.This condition occurs in children under the age of 10, and is more common in boys. Yesterday, Eun-ming saw one such child in the clinic. The disease usually develops within 24 to 48 hours after the child has eaten fava beans or fava bean products.Acute intravascular hemolysis, due to the massive destruction of red blood cells, the child'sElevated bilirubin values can occurPale.anemic) will also occur at the same timejaundice. The child's indirect bilirubin is elevated as seen in the graph below.

At the same time, due to the presence of intravascular hemolysis in the child, the hematocrit will be lower than usual. The chart below shows a comparison of the child's hemoglobin (hemoglobin concentration) at the onset of the disease and that at normal. In the morbid state, the child's hematocrit is only 4 grams.Normally a child has a hematocrit value of 12 grams.As the red blood cells in the child's body are destroyed in large quantities, the organs of the body will suffer from insufficient supply of oxygen and blood, and as a result, symptoms such as dizziness, anorexia, nausea, vomiting, and fatigue will occur. Progression of the disease can appearhemoglobinuriaIn children with severe hemolysis, there can beOliguria, anuria, acidosis and acute renal failureThe situation. At the onset of the child's symptoms, prompt hydration, correction of electrolyte disturbances, and alkalinization of the urine to prevent hemoglobin deposition in the renal tubules are needed. If necessary, transfuse G-6-PD normal red blood cells. Prevent the emergence of renal failure.Current research suggests that the cause of hemolysis induced by fava beans may be related to their richness in dopa, dopamine, fava bean pyrimidines, isoureidoids, and other oxidants. However, many patients with G-6-PD deficiency do not necessarily develop disease after eating fava beans, but for the health of patients with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, it is beneficial to avoid exposure to strong oxidizing substances as much as possible. Population screening is recommended in areas with a high prevalence of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency (Guangdong, Guangxi, Fujian, Sichuan, Guizhou, Yunnan, and Hainan). This screening is already performed in many maternal and child institutions. For those with definite glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, it is recommended to avoid taking drugs with strong oxidizing effects.I hope the answer is helpful to you. Good luck with your health.

Fava beans are a popular food, rich in nutrients, can be fried, cold, and can be made into a variety of small food. Every year at the turn of spring and summer is a large number of beans on the market season, many people want to eat, but many people in the eating of fava beans may cause a hereditary disease - fava bean disease.

What is Fava Bean Disease?

Fava bean disease is a disease caused by a deficiency of glucose 6-phosphate dehydrogenase (G-6-PD), which manifests itself as acute intravascular hemolysis that occurs suddenly after consumption of fresh fava beans in the presence of an inherited G-6-PD defect. Fava bean disease, an acute hemolytic anemia, occurs in individuals with genetic defects in erythrocyte G-6-PD after consumption of fresh green fava beans or exposure to fava bean pollen. It is known that genetically defective sensitive erythrocytes, due to G-6-PD defects can not provide enough reduced coenzyme II to maintain reduced glutathione reduction (antioxidant effect), in the encounter with a certain factor in the fava bean induced erythrocyte membrane oxidation, erythrocyte membrane phospholipids molecules of unsaturated fatty acids oxidized to generate lipid peroxidation, damage to the cell membrane. G-6-PD has a role in protecting normal red blood cells from oxidative damage, fresh fava beans are very strong oxidizing agents, when G-6-PD deficiency is the destruction of erythrocytes and cause disease. This disease is most common in children under 5 years of age, and male patients account for more than 90%. Fava bean disease is hereditary.In principle, it has been determined that fava beans are forbidden for people with fava bean disease.. However, it is not always the case that eating large quantities of fava beans continuously or at one time leads to the onset of fava bean disease.

What are the major clinical manifestations of Gammopathy?

In the early stage, there are malignant cold, slight fever, dizziness, tiredness and weakness, lack of appetite, abdominal pain, followed by jaundice, anemia, hemoglobinuria, and soy sauce-colored urine, and thereafter an increase in body temperature and aggravation of tiredness and weakness, which can last for about 3 days. In conjunction with the appearance of hemolytic anemia, vomiting, diarrhea and abdominal pain worsen, liver enlargement, abnormal liver function, splenomegaly in about 50% of patients. In severe cases, coma, convulsions and acute renal failure can be seen, and death often occurs within 1 to 2 days if emergency treatment is not adequate.

How do I get first aid treatment for fava bean disease?

(1) Artificially induce vomiting. (2) Gastric lavage with 1:5000 potassium permanganate solution. (3) 25% magnesium sulfate oral diarrhea. (4) Give high dose glucocorticoid. (5) Change blood or input fresh blood if necessary. (6) Alkalize the urine, give intravenous rehydration and use diuretics appropriately, and actively prevent and treat hemolytic uremic syndrome. (7) Symptomatic treatment.

What medications should not be taken by people with Gamma Disease?

(1) Chinese medicines and proprietary Chinese medicines that can induce fava bean disease: peppermint, camphor, naphthol, Sichuan lotus, powdered oxalis, waxflower, bear's gall bladder, pearl powder, baby powder, open-ended tea, seven-percent powder, infantilizer, oxalic acid detoxification pills, and so on.

(2) Western drugs that can induce fava bean disease: sulfonamides (e.g., sulfadiazine, sulfamethoxazole, sulfasalazine), aminophenyl sulfone, nitrofurans (e.g., furotoxin, furazolidone, furacillin), quinolones (e.g., pefloxacin, enrofloxacin), aspirin, phenacidine, primaquine, chloroquine, quinine, isosorbide dinitrate, nifedipine, berberine, cimetidine, and vitamin K3, Vitamin K4, Methylene blue, etc.

concluding remarks

Fava bean disease is most common in children under 5 years old, the onset of the disease is rapid, fierce, if the lack of understanding, will greatly threaten the lives of children and serious consequences, so it must be highly valued. For patients who have already occurred acute hemolysis of sericulture, should immediately stop eating fava beans and drugs that may cause hemolysis, for those who cause infection to actively treat the infection, light patients in addition to the above measures, generally do not need treatment, observation of changes in the condition or symptomatic treatment can be, the condition of the patient should be sent to the hospital for active treatment of serious patients.

Fava bean disease, in fact, the scientific name is called "glucose-6-phosphate dehydrogenase (G6PD) deficiency", is a congenital genetic disease, this kind of patients since birth, "G6PD" this kind of material is very little, for the later onset of the disease laid the foundation for the disease, but also the "internal cause" of this disease. The "internal cause" of the disease is the consumption of fava beans (or bean paste made from fava beans), which is the "external cause". In addition, there is another external cause: the consumption of oxidizing drugs. For example, primaquine, quinine, sulfonamides, antipyretics (finasteride), etc. can also cause this condition.

So why does this happen? At present, it is believed that the patient is very deficient in the substance "G6PD" from birth due to a genetic mutation, which leads to one result; the red blood cells that transport oxygen in our body are like bloated balloons, which are especially prone to "rupture" once the "external cause" is induced, the red blood cells rupture, and many substances inside the cells are released into the blood, which is called "hemolytic anemia". Once induced by "external factors", the red blood cells rupture, and many intracellular substances will be released into the blood, which is the so-called "hemolytic anemia", the patient will appear a series of symptoms, at first there is a general malaise, dizziness, fatigue, poor appetite, nausea, vomiting, followed by Yellowish color, urine color like soy sauce, serious cases will appear coma, and multi-organ failure. As for the beans and other "external causes" is how to induce, start the above said hemolytic process "the specific reasons are not clear. It is worth noting that those congenital "G6PD" deficiency patients eating fava beans onset of disease is actually only a small portion, in addition, eating fava beans may not be 100% onset of disease, the severity of the onset of disease and the amount of fava beans eaten does not have anything to do with it, which suggests that there are other unknown factors involved in the development of the process.

Because of the genetic mutation of sericulture, there is no curative treatment, or prevention, so, small sugar friendly reminder, hair over the disease, to avoid eating fava beans (including fava bean products) as well as some oxidizing drugs.

Gu Xiaohua

Suin

Apple-苹

I'm glad to have the opportunity to be able to answer your questions.

In medical terms, the scientific name for fava bean disease isGlucose-6-phosphate dehydrogenase deficiency, which is an inherited metabolic defect. Once a person suffering from fava bean disease consumes fava beans, a hemolytic reaction will occur after 24-48 hours, and in severe cases it can even lead to death. In addition to fava beans, some medications can also lead to hemolytic reaction in patients, including camphor, violet drops, quinine, sulfonamide antibiotics, blood glucose pills, aspirin, and so on. Therefore, people suffering from fava bean disease must avoid exposure to fava beans as well as these medications.

Currently, people with serovar diseases can be accurately identified through testing, and the presence of the disease can be determined after birth. Worldwide, sericosis is the most common inherited metabolic defect and is most common in males; female sericosis patients are rare. If a son has fava bean disease, his mother must be a carrier of the gene that causes the disease, but she is likely to behave normally because the disease is incompletely dominant. In China, fava bean disease is more prevalent in the southern region.

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China's centuries-old feudal society has inherited the idea of "favoring sons over daughters" and "passing on the male child to the female child", which has been inherited in G6PD deficiency. Yes, because G6PD deficiency (scientific name: glucose-6-phosphate dehydrogenase deficiency) is an X-chromosome with incomplete dominant inherited metabolic disease. It's hobbies stay on the 23rd pair of chromosomes associated with sex, the X chromosome, and are passed on to the next generation via the X chromosome.It is well known that males have X-Y paired sex chromosomes and females have X-X paired sex chromosomes. Girls: father has G6PD deficiency, mother does not The normal X gene passed from mother to girl will mask the effects of the father's diseased X chromosome, and the girl will not get serosanguineous disease as a carrier of the diseased gene. No disease will develop. Mother has G6PD deficiency, father does not. The normal X gene passed from the father to the girl will mask the effects of the mother's pathologic X chromosome and the girl will not get fava bean disease and will be a carrier of the pathologic gene. No disease will develop. Both father and mother have G6PD deficiency. Both X chromosomes are pathologic and there is no way to mask it to cause the girl to develop the disease. Boys: mother has, father does not, both parents have both boys will develop the disease. g6pd deficiency male:female = 9:1A breakdown of those patriarchal genetic diseases Hypertrophic Progressive Muscular Dystrophy Congenital Absence of Gammaglobulin Neurologic Optic Atrophy Hereditary Deafness Red-green color blindness Hemophilia Baldness

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Fava bean disease is a hereditary disease that causes hemolytic anemia in crazy people who eat fava beans.

My child also has this disease, found at birth, because this disease makes the child child was born, the jaundice index is particularly high, stayed in the incubator for a week, at that time I was worried sick.

I learned from my doctor that this disease is invisible cross-generation from mother to mother, and if I gave birth to a daughter, I wouldn't have it, and it just so happened that I gave birth to a son and was passed it on to me. I was actually surprised to hear about this disease because I had never heard of an allergy to fava beans before.

The doctor told me a lot about the precautions to be taken for this disease, and I also looked up information about it on the Internet, so I have been very careful about my child's diet and medication since he was a child, fearing that my child will encounter something that cannot be used and cause an allergy. I also read that the longer the child grows, the better the body's resistance, slowly less prone to allergic hemolysis, I do not know whether it is true or not, but I hope hope it is, but I will always monitor the child's diet and medication taboos, do not want to let the child to take risks.

Contraindications of serotonin: mainly diet and drug use, diet at home their families know, no big problem, mainly after the child goes to school, in the kindergarten must be clear with the school teacher, their children can not eat what.

Then there is the medication, the child is sick with medication, whether it is in the hospital or a small clinic or a pharmacy, remember to explain to the doctor that their child has fava bean disease, to avoid the drugs that can not be used.

Specifically what can not be used, through the doctor and online information a check will know, I will not list them here, the main thing is that parents have to be attentive, do not at times ignore the problem.

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