Sulfhaemoglobinemia
Introduction
Introduction to sulphide hemoglobinemia Sulfemoglobinemia (sulfhemoglobinemia) is caused by sulfmethemoglobin (SHB) in the blood of patients. basic knowledge The proportion of illness: 0.005% Susceptible people: no special people Mode of infection: non-infectious Complications: coronary heart disease atherosclerosis
Cause
Cause of sulphi hemoglobinemia
Cause:
Any drug that produces methemoglobin can also produce sulphur hemoglobin, including:
Nitrogen-containing compounds, such as potassium nitrate, sodium nitrite or nitroglycerin; aromatic amino compounds, such as sulfonamides, aniline derivatives or phenacetin, some patients do not receive any drugs to get the disease, may be associated with chronic constipation Or diarrhea-related, some patients have increased glutathione in red blood cells, the reason is unknown, once the sulphi hemoglobin is produced, it can not be restored to hemoglobin in vivo or in vitro. The red blood cells containing sulphur hemoglobin have normal life, but some people think that life expectancy is shortened. The size, thickness and amount of hemoglobin in the patient's red blood cells are in the normal range.
Pathogenesis :
The pathogenesis of this disease has not yet been clarified. In the test tube, the hemoglobin solution can generate hemoglobin by hydrogen sulfide gas, the color changes from red to dark, and the animal is fed with sulfur-containing feed, which can also produce hemoglobin, intestinal-induced carbunopathy. The patient may also have hemoglobinemia at the same time. Some people think that this is related to the absorption of hydrogen sulfide by the diseased intestinal tract. In some patients with a history of this disease, the glutathione in red blood cells is increased. It suggests that cysteine in the body may also be one of the sources of sulfur. Therefore, some people think that hydrogen sulfide may originate from the patient's red blood cells, not necessarily from the intestine, taking various oxidant drugs that can cause toxic methemoglobinemia or Chemicals can also cause sulphur hemoglobinemia, which is most commonly caused by aromatic amino compounds such as acetanilide, dapsone, metoclopramide and phenacetin, but in patients taking these drugs, vulcanization occurs. Hemoglobinemia is rare, and the same drug induces hemoglobin and methemoglobin, respectively. It is not clear that some patients do not even have a history of exposure to the above or special drugs, so individual patients may be congenital, and environmental pollution may also cause hemoglobin.
In 1983, a survey of a small town inhabited by industrial pollution in Brazil found that blood methemoglobin and sulphi hemoglobin increased in comparison with other residents. Hemoglobin has a strong absorption band near the wavelength of 620 nm. The addition of potassium cyanide does not cause discoloration. The exact position of the sulfur atom in the hemoglobin molecule has not been determined. It is generally considered that the carbon double bond of one pyrrole ring in the heme procarbazine is opened and a sulfur is added. Once the atom, sulphur hemoglobin is formed, it will not fall off, and it will lose the function of oxygen. The sulphide hemoglobin lacks oxygen carrying capacity and shifts the oxygen separation curve to the left. The effect of sulphur hemoglobin on the life of red blood cells is not clear. Some patients Red blood cell life measured by isotope is still normal, but many patients can have hemolysis.
Prevention
Sulfated hemoglobinemia prevention
By implementing a strategically balanced disease management and prevention program, hemoglobin abnormalities can be effectively reduced. Sickle cell disease is incurable; however, the following measures can be taken to control the symptoms:
Large intake of liquid
Healthy diet
Add folic acid
painkillers
Antibiotics
Thalassemia requires regular blood transfusions to maintain healthy hemoglobin and sustain life. Due to multiple blood transfusions, human organs are overloaded with iron and require secondary treatment to control this condition. Thalassemia can be cured by successful bone marrow transplantation, but this procedure is expensive and in most cases impossible.
The most cost-effective strategy to reduce the abnormal burden of hemoglobin is to supplement disease management by implementing prevention programs. A low-cost and reliable blood test identifies couples who are likely to have children affected. This genetic screening is especially suitable for pre-marital or pre-pregnancy, making it possible for couples to discuss their child's health. Subsequent genetic counseling describes to the characteristic gene carriers the risks that may be passed on to their children and the treatment that may be required if they are suffering from abnormal hemoglobin. Prenatal screening for genetic diseases involves specific ethical, legal, and social issues that require due consideration.
WHO's activities to prevent and control hemoglobin abnormalities
The governing body of the World Health Organization passed two resolutions on hemoglobin abnormalities. Resolution on sickle cell disease adopted by the Fifty-ninth World Health Assembly in May 2006 and resolution on thalassemia adopted by the WHO Executive Committee at its 118th session, calling on affected countries and WHO Organize the Secretariat to strengthen its response to these diseases.
Specifically, WHO will:
Raise awareness of the global burden of these abnormal diseases in the international community;
Promote fair access to health care services;
Provide technical support to countries in the prevention and management of these diseases;
Promote and support research to improve the quality of life of affected people.
WHO strategy for prevention and control of chronic diseases
WHO's work on hemoglobin abnormalities is now included in the WHO Framework for Chronic Disease Prevention and Control in the Department of Chronic Diseases and Health Promotion. The Divisions strategic objectives are to raise awareness of the global chronic disease epidemic; to create a healthy environment, especially for the poor and disadvantaged populations; to slow and reverse the growth of common chronic disease risk factors such as unhealthy diets and physical inactivity; and to prevent Premature death and avoidable disability caused by major chronic diseases.
Complication
Combustion hemoglobinemia complications Complications coronary heart disease atherosclerosis
Checking glycosylated hemoglobin can predict coronary heart disease. Blood sugar is a "continuous risk factor" for cardiovascular disease. Controlling blood sugar is the key to preventing cardiovascular and cerebrovascular diseases, while glycated hemoglobin is the "gold standard" for blood glucose monitoring. Its level of change can directly affect coronary artery disease.
Glycated hemoglobin is a product of non-enzymatic glycation reaction under the action of hyperglycemia. It is an independent risk factor for coronary heart disease, which can increase the viscosity of red blood cells, reduce fluidity, reduce function, slow down oxygenation, and cause tissue deficiency. oxygen. When glycated hemoglobin is elevated, the risk of complications such as atherosclerosis increases, and if it continues to rise, it will exacerbate the development of atherosclerosis.
Patients with elevated glycosylated hemoglobin should not only be treated in time to improve vascular endothelial cell function, but also have to undergo coronary heart disease examination.
Symptom
Symptoms of sulphide hemoglobinemia common symptoms cyanosis abdominal pain diarrhea dizziness dyspnea hemolytic anemia constipation
The onset is slow, cyanosis is the main clinical manifestation, blue blemishes on the skin and face, blue-gray, no symptoms, or only blemishes, severe dizziness, headache, even irritability, fainting, about half of patients There are constipation, diarrhea, abdominal pain, these symptoms may not be directly related to sulphated hemoglobin, some patients have a history of taking drugs or exposure to toxic substances, and some patients may have mild hemolytic anemia.
Examine
Examination of sulphi hemoglobinemia
1. Appearance: The arterial blood containing sulphide hemoglobin is blue-brown and does not change color after shaking in the air. After being added to the methylene blue thermostat, the blood cannot be turned red.
2. Determination of sulphide hemoglobin: There is a strong absorption peak near 620 nm, and potassium cyanide is not lost. According to the method of multi-wavelength absorbance correction, the content of sulphi hemoglobin can be calculated.
3. In isoelectric focusing electrophoresis: There is a green zone between oxyhemoglobin and deoxyhemoglobin.
According to the condition, clinical manifestations, symptoms, signs, X-ray, B-ultrasound, electrocardiogram, biochemical examination.
Diagnosis
Diagnosis and identification of sulphi hemoglobinemia
Diagnostic criteria
Mainly relying on laboratory tests to make a diagnosis, the clinical manifestations of obvious cyanosis and blood in the air are blue-brown, do not change color after shaking, check with a spectrophotometer, a specific light absorption peak can be seen at 620nm, and cyanide is added. It does not disappear after potassium, which can be used as a reliable diagnosis method for this disease. The medical history of medication can also help diagnose.
Differential diagnosis
The identification method is to take a little anticoagulation of the patient in a small triangular bottle, for example, the blood quickly turns bright red, indicating that the blood contains more reduced hemoglobin; if the blood cannot turn red, it is certain that there are other abnormal hemoglobin in the blood. When observed by the naked eye, methemoglobin has a chocolate-like brown color, sulphi hemoglobin is blue-brown, and hemoglobin M is chocolate brown due to methemoglobin production. The blood is diluted 10 to 20 times with distilled water, and ammonium sulfide or 1% potassium cyanide is added. Drops, such as methemoglobin, change from tan to bright red; if it is too long, it turns blue-brown (methemoglobin-oxyhemoglobin-sulfated hemoglobin); if the color is not bright red, it is sulfided hemoglobin, spectroscope The absorption band of methemoglobin is observed at a wavelength of 618-630 nm, and the hemoglobin is at a wavelength of 607-620 nm; after the addition of ammonium sulfide or potassium cyanide, the absorption band of methemoglobin disappears, and the absorption band of sulphur hemoglobin does not change ( As the hemoglobin can not be restored once it is produced, the identification of hereditary methemoglobinemia and HbM is two. Different genetic pattern; methemoglobinemia autosomal recessive, autosomal dominant HbM, abnormal zone while the former when the latter no hemoglobin electrophoresis.
Sulfated hemoglobin is often misdiagnosed as methemoglobin, and clinical trials are very similar, requiring careful laboratory testing.
