Congenital hemolytic anemia refers to hemolytic anemia caused by the incompatibility of blood types between the mother and the baby. The incidence rate in Guangdong is relatively high in the country, at 5 to 6 percent. Patients generally have pale complexion, difficulty breathing, fatigue, etc., and even nausea and vomiting in severe cases. So, is congenital hemolytic anemia hereditary? Now I will introduce it to you, hoping it can be helpful. Hemolytic anemia refers to anemia caused by accelerated red blood cell destruction that exceeds the compensatory capacity of the bone marrow's hematopoietic function. Congenital hemolytic anemia, also known as thalassemia, is a group of hereditary diseases. The pathogenesis is that the globin chains that synthesize hemoglobin are reduced or missing, resulting in abnormal hemoglobin structure. Red blood cells containing abnormal hemoglobin have reduced deformability and shortened lifespan. They can be destroyed prematurely by the liver, spleen, etc. of the human body, leading to anemia and even developmental abnormalities. This disease is medically known as hemolytic anemia. Hereditary polycythemia vera is mainly caused by the destruction of spherocytes by the spleen, so surgical removal of the spleen is the fundamental method of treatment. A few days after splenectomy, jaundice subsides and hemoglobin increases; the life span of red blood cells is prolonged, but cannot completely return to normal; the morphology and number of microspherical red blood cells in peripheral blood do not change. Depending on the location of red blood cell destruction, it is divided into two types: intravascular hemolysis and extravascular hemolysis. Intravascular hemolysis is generally acute hemolysis, but can also manifest as a chronic hemolytic process. It is more common in paroxysmal nocturnal hemoglobinuria, G-6PD deficiency, cold antibody-type autoimmune hemolytic anemia, and hemolytic anemia caused by drugs, physical and chemical factors, infection and other factors. Extravascular hemolysis is generally a chronic hemolytic process and is more common in hereditary spherocytosis, hemoglobinopathy, and warm antibody autoimmune hemolytic anemia. The clinical manifestations of hemolytic anemia are related to the urgency, degree and location of hemolysis. 1. Acute hemolysis has an abrupt onset and may be accompanied by sudden chills, high fever, pale complexion, back pain, shortness of breath, fatigue, irritability, and gastrointestinal symptoms such as nausea, vomiting, and abdominal pain. This is due to the massive destruction of red blood cells and the toxic effects of their decomposition products on the body. When the concentration of free hemoglobin in plasma exceeds 130 mg%, it is excreted in the urine, resulting in hemoglobinuria. The urine is colored like strong black tea or soy sauce, and jaundice may appear after 12 hours. The hemolytic products damage the renal tubular cells, causing necrosis and deposition of hemoglobin in the renal tubules, as well as peripheral circulatory weakness, which can lead to acute renal failure. Due to anemia and hypoxia, severe cases may suffer from apathy or coma, shock and heart failure. 2. Chronic hemolysis has a slow onset. In addition to the common symptoms and signs of general anemia such as fatigue, pallor, shortness of breath, and dizziness, there may be varying degrees of jaundice. Splenomegaly and liver enlargement are common. Gallstones are a common complication, and obstructive jaundice may occur. Ulcers form on the skin of the ankles of the lower limbs and are difficult to heal, which is common in patients with sickle cell anemia. The disease is hereditary and affects both genders; patients appear in every generation, not just in alternate generations. It is a dominant disease on the autosomes, so the chance of getting the disease is very high. It is recommended that patients have a baby after surgery, which can help reduce the possibility of fetal inheritance and is safer. Regular prenatal checkups are required. |
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