Just a disease.
In the case of hereditary glucose -6- phosphate dehydrogenase (G-6-PD) deficiency, acute intravascular hemolysis occurs suddenly after eating fresh broad beans. G-6-PD can protect normal red blood cells from oxidative damage. Fresh broad bean is a strong oxidant. When G-6-PD is deficient, red blood cells will be destroyed and cause disease. The disease is inherited by sex-linked incompleteness. The G-6-PD gene is on the X chromosome, and most of the patients are male, with the ratio of male to female being about 7∶ 1. After eating raw broad beans for several hours to several days (1~ 3 days), they suddenly have fever, dizziness, irritability and nausea, and their urine is soy sauce-like or wine-colored. Generally, they can recover after 2 ~ 6 days of attack. It can be diagnosed by medical history and methemoglobin reduction test (the reduction rate is greater than 75%), especially by fluorescence spot test. Once diagnosed, you should immediately stop eating raw broad beans, blood transfusion, infusion and alkaline urine to prevent acute renal failure.
toxicology
People with genetic defects in erythrocyte glucose -6- phosphate dehydrogenase (G6PD) will suffer from acute hemolytic anemia-silkworm disease after eating fresh broad bean or contacting with broad bean pollen. Pathogenic mechanism is not very clear, coke.
Some sensitive red blood cells with known genetic defects are caused by acute intravascular hemolysis when they encounter some factor in broad beans.
clinical picture
At the early stage, there were aversion to cold, slight fever, dizziness, listlessness, lack of appetite and abdominal pain, followed by jaundice, anemia and hemoglobinuria, and the urine was soy sauce-colored. After that, the body temperature increased and the burnout was aggravated, which lasted for about 3 days. At the same time as hemolytic anemia, vomiting, diarrhea, abdominal pain, hepatomegaly and abnormal liver function occur, and about 50% patients have splenomegaly. Coma, convulsion and acute renal failure can be seen in severe cases, and they often die within1~ 2 days if they are not given first aid.
diagnose
1. Have a history of eating green broad beans or inhaling broad bean pollen.
2. Clinical features ① The incubation period ranges from several hours to 48 hours. ② Poisoning symptoms: aversion to cold, slight fever, dizziness, listlessness, lack of appetite and abdominal pain in the early stage, followed by jaundice, anemia and hemoglobinuria, and the urine was soy sauce-colored. After that, the body temperature increased and the tiredness increased, which lasted for about 3 days. ③ Experimental examination: methemoglobin reduction test (MHb) normal reduction rate >; 75% (colorimetric method), the reduction rate of MHb in patients with silkworm disease is 31%~ 74% (heterozygote inheritor), and the reduction rate is <: 30% (homozygous type); Blood containing denatured globin bodies (Hernz bodies) can be more than 5% (normal 0 ~ 0.28%).
Emergency treatment
1. Artificial vomiting.
2. Give1:5000 potassium permanganate solution for gastric lavage.
3. 25% magnesium sulfate for oral catharsis.
4. Give a large dose of glucocorticoid.
5. Change blood or input blood when necessary.
6. Alkalize urine, properly give intravenous rehydration and use diuretics to actively prevent and treat hemolytic uremic syndrome.
7. symptomatic treatment.