Favism is a disease caused by the lack of glucose phosphate dehydrogenase in the body. It is a hereditary disease and a very serious disease. Patients will have acute attacks, leading to hemolytic anemia, often high fever, jaundice, nausea and vomiting, and fainting and shock. If rescue is not timely, it may even lead to kidney and heart failure, leading to death. What is favism? Is it serious? Favism is a disease caused by glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, characterized by sudden onset of acute intravascular hemolysis after consuming fresh broad beans. The patients are mainly distributed in provinces south of the Yangtze River, mainly in Hainan, Guangdong, Guangxi, Yunnan, Guizhou, Sichuan and other provinces. The cause of the disease is due to G6PD gene mutation, which leads to reduced enzyme activity, and red blood cells cannot resist oxidative damage and are destroyed, causing hemolytic anemia. The complications of favism are: the severity of the clinical manifestations of this disease varies. Most patients, especially female heterozygotes, do not usually suffer from the disease and have no subjective symptoms. Some patients may show symptoms of chronic hemolytic anemia. Acute hemolytic reactions such as hemoglobinuria, jaundice, and anemia are often induced by eating broad beans, taking or contacting certain drugs, or infection. Severe acute hemolytic anemia induced by G6PD deficiency can cause excessive destruction of red blood cells and, if not treated in time, can lead to liver, kidney, or heart failure, or even death. What is the pathological cause of favism ? The pathological cause of favism is that people with genetic defects in erythrocyte glucose-6-phosphate dehydrogenase (G6PD) will develop acute hemolytic anemia - favism - after eating fresh broad beans or coming into contact with broad bean pollen. The pathogenic mechanism is not yet very clear.It is known that sensitive red blood cells with genetic defects cannot provide enough NADPH to maintain the reducing property (antioxidant effect) of reduced glutathione (GSH) due to the defect of G6PD. When encountering a certain factor in broad beans, it induces oxidation of the red blood cell membrane and produces a hemolytic reaction. G-6-PD protects normal red blood cells from oxidative damage. Fresh broad beans are very strong oxidants. When G-6-PD is deficient, red blood cells will be destroyed and cause disease. What are the symptoms and signs of favism? The symptoms and signs of favism are: chills, mild fever, dizziness, fatigue, lack of appetite, abdominal pain in the early stage, followed by jaundice, anemia, hemoglobinuria, soy sauce-colored urine, after which the body temperature rises, fatigue and weakness worsen, which can last for about 3 days. At the same time as hemolytic anemia occurs, vomiting, diarrhea, and abdominal pain increase, the liver is enlarged, and liver function is abnormal. About 50% of patients have splenomegaly. Severe cases may cause coma, convulsions and acute renal failure. If emergency treatment is not timely, death often occurs within 1 to 2 days. Most cases develop 1 to 2 days after eating broad beans. Early symptoms include anorexia, fatigue, low fever, nausea, and variable abdominal pain. This is followed by yellowing of the corners of the eyes and systemic jaundice due to hemolysis, as well as soy sauce-colored urine and anemia symptoms. In severe cases, there may be urine masses, shock, heart and kidney failure. In severe cases of hypoxia, fixed deviation of both eyes may also be seen. |
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