Broad bean disease
Introduction
Introduction to broad bean disease Commonly known as faba bean disease, hereditary glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common type of hereditary enzyme deficiency disease, commonly known as faba bean disease. About 200 million people worldwide suffer from this disease. China is one of the high-incidence areas of this disease. It is characterized by a distribution of high and low north. The prevalence rate is 0.2-44.8%. The cause of G6PD deficiency is due to the mutation of G6PD gene, which leads to the decrease of the activity of the enzyme. Red blood cells cannot resist oxidative damage. And suffered damage, causing hemolytic anemia. basic knowledge The proportion of sickness: 0.01%--0.02% (more common in children, the incidence rate is about 0.01%-0.02%, with genetic predisposition, mostly occurs in the season of broad bean maturity) Susceptible people: no specific people Mode of infection: non-infectious Complications: hemolytic anemia
Cause
Cause of broad bean disease
Erythrocyte glucose-6-phosphate dehydrogenase (G6PD) has a genetic defect. After eating green fresh beans or exposure to broad bean pollen, acute hemolytic anemia, faba bean disease, occurs, and the pathogenesis is not yet clear. Sensitive red blood cells known to have genetic defects cannot provide sufficient NADPH due to defects in G6PD to maintain the reducing (antioxidation) of reduced glutathione (GSH), which is induced after encountering certain factors of broad bean species. The erythrocyte membrane is oxidized to produce a hemolysis reaction.
Prevention
Broad bean disease prevention
Experts say that as long as you don't eat a lot of broad beans, you can avoid faba disease. However, patients with hereditary red blood cell deficiency, suffering from hemorrhoids bleeding, indigestion, chronic colitis, uremia and other patients should pay attention to, should not eat broad beans.
Complication
Broad bean disease complications Complications hemolytic anemia jaundice
The clinical manifestations of this disease vary in severity. Most patients, especially female heterozygotes, usually do not develop symptoms and have no symptoms. Some patients may present with symptoms of chronic hemolytic anemia. Often due to consumption of broad beans, taking or exposure to certain drugs, infections and other induced hemoglobinuria, jaundice, anemia and other acute hemolysis reactions. Severe acute hemolytic anemia induced by G6PD deficiency is caused by excessive destruction of red blood cells. If not treated in time, it may cause liver, kidney, or heart failure, or even death.
Symptom
Broad bean disease symptoms common symptoms abdominal pain fatigue nausea cold proteinuria diarrhea low fever black urine
The clinical manifestations of G6PD deficiency are roughly the same as those of general hemolytic anemia. It is divided into clinical types such as neonatal jaundice, faba bean disease, drug-induced hemolysis, infectious hemolysis, and non-spherical cell hemolytic anemia. The clinical manifestations of this disease vary in severity. Most patients, especially female heterozygotes, usually do not develop symptoms and have no symptoms. Some patients may present with symptoms of chronic hemolytic anemia. Often due to consumption of broad beans, taking or exposure to certain drugs, infections and other induced hemoglobinuria, jaundice, anemia and other acute hemolysis reactions. Severe acute hemolytic anemia induced by G6PD deficiency is caused by excessive destruction of red blood cells. If not treated in time, it may cause liver, kidney, or heart failure, or even death.
Examine
Inspection of broad bean disease
(1) Blood picture:
1 Hemoglobin drops sharply. The weight is reduced to less than 10 grams / liter.
2 The red blood cells are reduced to a minimum of 0.5×1012/liter.
3 Reticulocytes were significantly increased by >0.20.
4 Peripheral blood smears showed nucleated red blood cells.
5 leukocytes rise, up to (10 ~ 20) × 109 / liter, and even a leukemia-like reaction;
6 platelet count is normal or increased.
(2) Bone marrow:
1 Both the erythroid cell line and the granulocyte cell line are obviously proliferated, and the younger the granulocyte cell line, the more obvious the proliferation.
2 The red blood cell line is mainly composed of middle and late erythroid cells.
(3) urine test:
1 Urine is soy sauce, rich brown, red wine, washed water, yellow, etc.
2 The positive rate of occult blood test can reach 60% to 70%.
3 urine test showed protein, red blood cells and cast, urinary bilirubin and urinary bilirubin were positive.
4 serum free hemoglobin increased. Binding globin is reduced.
5 Determination of glucose-6-phosphate dehydrogenase activity was reduced.
Diagnosis
Diagnosis and identification of broad bean disease
The disease is inherited by sexual association. The G-6-PD gene is on the X chromosome. Most of the patients are male. The ratio of male to female is about 7:1. It takes several hours to several days (1 to 3 days) after eating raw beans. Sudden fever, dizziness, irritability, nausea, urine is soy sauce-like or wine-colored, usually recover after 2 to 6 days, but if the person is not rescued in time, it will endanger life due to circulatory failure. It can be diagnosed by medical history and methemoglobin reduction test (reduction rate greater than 75%), especially by fluorescence point test.
1. Have a history of eating green broad beans or inhaling broad bean pollen.
2. Clinical features:
1 incubation period of several hours to 48 hours.
2 Poisoning performance: early aversion to cold, fever, dizziness, fatigue, loss of appetite, abdominal pain, followed by jaundice, anemia, hemoglobinuria, urine is soy sauce color, after which the body temperature rises, fatigue and fatigue increase, sustainable 3 Around day.
3 experimental examination: methemoglobin reduction test (MHb) normal human reduction rate > 75% (colorimetric method), MHb reduction rate of faba bean disease patients 31% ~ 74% (heterozygous inheritance), reduction rate <30% (homozygous Type); the blood-containing denatured globin body (Hertz body) can be higher than 5% (normally 0 to 0.28%).
