A 4-year-old boy with a sallow complexion and low energy came to the emergency room with the help of his father. According to the father, yesterday the child was still alive and kicking, but today he started to have bouts of fever, shivering all over, and his urine turned brown, like soy sauce. The child does not want to eat or drink, nor does he want to walk on the ground. The doctor asked if the child had eaten broad beans in recent days, and the father nodded repeatedly. While ordering them to take a blood test, the doctor told the child’s father that the child’s symptoms should be anemia caused by favism disease, which needs to be confirmed by laboratory tests, and blood transfusion is likely to be needed.
Unexpectedly, the child’s problem was so serious. The father was shocked: What kind of disease is favism? Why eating broad beans can cause such a serious disease? Can children not eat broad beans in the future?
What is Favism
Favism is one of the common clinical types of erythrocyte glucose-6-phosphate dehydrogenase deficiency. Erythrocyte glucose-6-phosphate dehydrogenase deficiency, referred to as G-6-PD deficiency, is a genetic disease. In children with G-6-PD deficiency, the red blood cells in the blood will rupture after exposure to certain triggers, causing a series of symptoms.
Boys are more affected than girls. According to statistics, there are about 400 million G-6-PD deficiency patients in the world. In my country, the disease is mainly found in the Yangtze River Basin and the provinces south of it. The incidence rate is relatively high in Yunnan, Hainan, Guangdong, Guangxi, Sichuan, Guizhou and other places. The incidence rate is 4% to 15%, and even as high as 40% in some areas. , while the northern provinces are relatively rare.
Symptoms of “Baby Bean”
Let’s call these children who have anemia after eating broad beans “broad bean babies”. The red blood cells in these “broad bean babies” are congenitally deficient in G-6-PD, which leads to hemolysis after the children eat broad beans, and even after the mother eats broad beans and then breastfeeds the child. Hemolysis is the spontaneous rupture of red blood cells in human blood, causing symptoms such as anemia, jaundice, fever, chills, and chills in children. After the red blood cells are destroyed, a large amount of hemoglobin will be produced and excreted in the urine, forming soy sauce-colored “hemoglobinuria”.
Children with broad bean disease often develop the disease during the season when broad beans are ripe. It can occur at any age, and it is common in children under 10 years old. Children generally develop symptoms within hours to days (mostly within 1 to 2 days) after eating broad beans or their products. The shorter the incubation period from eating broad beans and their products to onset, the more severe the symptoms in children. Children with mild hemolysis may not have obvious jaundice and urine color changes. In severe cases, hemolysis may occur rapidly and obvious symptoms may appear. At the same time, the decomposition products of a large amount of red blood cells can also cause discomfort such as fever and chills in the child. In severe cases, the condition of the child develops rapidly, and may even cause confusion, convulsions, shock, and acute renal failure.
How to Diagnose and Treat
Diagnosis of favism needs to be combined with the patient’s clinical manifestations, and at the same time go to the hospital for red blood cell G-6-PD activity determination. Generally speaking, the G-6-PD activity of the child is reduced, and the G-6-PD/6-PGD ratio measurement and G-6-PD gene detection can also be checked.
The treatment of favism is mainly to control hemolysis. Once the child develops hemolysis due to the disease at home, parents should try to keep the child away from the cause and give him enough water at the same time. Hospital treatment is usually infusion to correct electrolyte imbalance and keep the urine alkaline to prevent hemoglobin from depositing in the kidneys and causing kidney damage. Children with severe anemia are transfused with normal G-6-PD red blood cells. Newborns can be treated with blue light to reduce jaundice, and blood transfusion and blood exchange can be performed if necessary.
Can broad bean disease be prevented?
First of all, it is necessary to know whether the child is a child with G-6-PD deficiency. If the child is known to have genetic defects, try to avoid exposure to triggers, such as eating broad beans.
my country has already carried out the screening of the four newborn diseases, that is, 72 hours after the baby is born, after 6 to 8 normal feedings, the baby’s heel blood is collected for the screening of the four genetic diseases. These four genetic disorders include G-6-PD deficiency. Family members of newborn babies must cooperate with obstetrical medical staff to complete the screening of the four neonatal diseases in time to achieve early detection. In areas with a high incidence of G-6-PD deficiency in my country, parents should also take their children to participate in the general survey of G-6-PD deficiency.
If the baby has G-6-PD deficiency, parents should avoid eating broad beans and their products (fresh broad beans, dried broad beans, processed broad beans, etc.) in future feeding. Mothers of “broad bean babies” should also fast for broad bean foods and a series of drugs with oxidative effects during breastfeeding, and strengthen the prevention of various infections.
Parents should take the initiative to inform the doctor and teacher of the child’s condition when the baby seeks medical treatment, enters the nursery, or enters the kindergarten, and avoids exposure to factors that induce hemolysis in advance.
Learn about G-6-PD Deficiency
Favism is one of the common clinical types of G-6-PD deficiency. In addition to favism, children with G-6-PD deficiency may present with the following types of clinical manifestations.
1. Taking drugs with oxidative properties may induce symptoms similar to favism, such as some antipyretic and analgesic drugs (aspirin, etc.), antimalarial drugs (quinine, etc.), sulfa drugs, nitrofuran drugs, Dosage vitamin K etc.
2. Jaundice is a common symptom in newborns, and part of pathological jaundice is caused by G-6-PD deficiency.
3. When bacterial or viral infection occurs, hemolysis may be induced.
4. Congenital non-spherical hemolytic anemia, that is, chronic hemolysis occurs without incentives, manifested as anemia, jaundice, and enlarged spleen. When infected or taking oxidative drugs, acute hemolysis will be induced.