Sickle Cell Nephropathy

　　Sickle cell nephropathy is caused by genetic inheritance of abnormal beta globin chain subunit genes of hemoglobin. Patients receive two identical abnormal hemoglobin genes from both parents, known as homozygotes, in which red blood cells lack normal hemoglobin (HbA) and are replaced by abnormal hemoglobin (HbS), known as sickle cell anemia or sickle cell disease. Patients receive one abnormal hemoglobin gene from one parent, known as heterozygotes, in which red blood cells contain both normal and abnormal hemoglobin, but the normal ones account for more than 50%, known as sickle cell trait. Both reasons can lead to the occurrence of sickle cell nephropathy.

　　The two most common complications and disabling diseases of sickle cell nephropathy are osteonecrosis and osteomyelitis. The main complications of renal damage in patients are acute and chronic renal failure. Factors such as hypoxia, fatigue, infection, dehydration, fever, cold exposure, or acidosis can trigger sickling of red blood cells. Some patients experience more severe sickling and massive hemolysis at night, during infection, and when they catch a cold, which may even induce acute crises.

　　According to the different types, the clinical manifestations of this disease are as follows:

　　1, Homozygous sickle cell disease type

　　This type has varying degrees of hemolytic anemia, mild jaundice, and vascular occlusion crisis as its prominent feature. It often causes severe pain in the trunk and limbs, including visceral, bone, joint, and muscle pain, especially with掌骨、附骨and finger (toe) bone infarction being more common. Infection, dehydration, hypoxia, and acidosis are its triggers.

　　2, Heterozygous sickle cell disease type

　　This type is usually asymptomatic and does not cause hemolytic anemia, so it is also called sickle cell tendency. It only appears under severe hypoxia conditions with microcirculatory disorders, mainly occurring in the straight blood vessels of the renal medulla because there is a significant osmotic pressure there, which makes red blood cells prone to dehydration. In addition, the physiological characteristics of low oxygen tension and low pH value lead to sickling of red blood cells, stasis of renal medulla blood flow, and even vascular occlusion. In severe cases, renal papillary necrosis may occur.

　　Although there is no specific treatment method for this disease at present, it is very beneficial to prevent acute crises and improve the prognosis by guiding patients to avoid causes of sickle cell transformation, strengthen self-care, pay attention to warmth, avoid fatigue, and adopt integrated therapy of traditional Chinese and Western medicine to treat the disease, which will make the treatment more effective.

　　5

　　What laboratory tests are needed for sickle cell nephritis

　　This disease is caused by abnormal hemoglobin, and its diagnosis generally requires the following examinations:

　　2. Urine examination

　　Urine examination may show hematuria, proteinuria, hyposthenuria; this disease can occur in both pure and heterozygous types, and hematuria can be gross hematuria, but mainly microscopic hematuria, which may be caused by microscopic necrosis of renal medulla and renal papilla, or glomerular hematuria. Sickle cell disease can occur typical nephrotic syndrome, at this time, there may be laboratory changes of nephrotic syndrome.

　　Patients with this disease should eat light and easy-to-digest foods, avoid seafood, beef, mutton, spicy and irritating foods, alcohol, and all kinds of irritants, especially for patients with Yin deficiency. At the same time, patients should pay attention to eating more fresh vegetables and moderate amounts of fruit, drinking water appropriately, to help the disease recover faster.

　　The treatment principles of this disease include the treatment of sickle cell disease and the treatment of kidney damage. The treatment of sickle cell disease is mainly based on the treatment of sickle cell anemia, the relief of sickle cell anemia, the reduction of blood viscosity, and symptomatic treatment, while avoiding factors that exacerbate sickle cell anemia; kidney damage is mainly treated with symptomatic treatment.

　　Drugs may reverse sickle cell anemia but cannot correct its genetic defects. Sickle cell drugs can relieve sickle cell anemia, reduce blood viscosity, thereby improving microcirculation, increasing blood perfusion, improving tissue oxygen supply, reducing kidney damage, and alleviating clinical symptoms. Hemoglobinopathies generally do not require treatment, as the solubility of heterozygotes ranges from 35% to 68%, and the degree of hemolysis is low. The ideal sickle cell drug has not yet been seen, and the commonly used drugs currently include zinc sulfate, dihydroergocristine (Hydergine, dihydroergocryptine), 30% urea sucrose solution, methyl diacetyl dimine, carbamyl phosphate, 5-azacytidine (5-Azacytidine), Nootropyl (contains piracetam, aniracetam), and others.