Bila Sistanna: Causes, Symptoms and Treatment

Cystinuria, under the protection of the cystinin, is a genetic disease that distinguishes a defect in the process of transferring amino acids, namely: Cysteine, lysine, arginine and ornithin by the cultural prophet in college. A similar disorder also occurs in the small intestine, so the amino acids filtered in the total glassurus are not absorbed into the blood and are excreted with urine. Cysteine ​​as low solubility 300 milligrams/ L equals 1.25 milloline/ L for the disease is in the urine corridors. The source of a disease is 1% – 2% of adults, and 6% – 8% of children with kidney batter and urine corridors from Cystin. These stones lead to blockages and pollution in the kidneys and urine system, and it can cause difficult damage to the actions of the kidneys, and no bowel disease associated with cystine has yet to be described. Symptoms of cystinin are often symptoms of cystin, the youth stage, rarely occur in adolescence, and one of the most important symptoms: the appearance of blood in the urine. One way pain and can be behind. Pain in the vicinity of the pelvis, thigh or abdomen. Nausea and vomiting. The night of Cysteine ​​can be symptoms if stones are not formed, but when stones are formed, symptoms occur. Causes and factors of the risk of Cysteine ​​A night of Cysteine ​​There are many causes and factors that lead to the Cysetic night: 1. The causes of cystinism are transmitted by genes, and the genetic transition of the disease is a self -generating formal feature, and its appearance all over the world 1: 15000 – 1: 7000 per one. Seven thousand people. So far, mutations have been found in two different genes for the disease, namely: the family of 3 Solu Carrier Family 3 -Member 1). Family 7 Solved Carrier Family 7 Member 9. The mutations lead to the secretion of cystine with urine in different quantities, but no difference is found in the nature and intensity of the disease caused by each. The amino acids are formed when the body consumes and breaks down protein, and is used to perform a variety of physical functions, therefore it is important for your body and is not considered waste, and if these amino acids enter the kidneys, they are usually re -absorbed. In people with the urine of Sistini, the genetic imbalance contrasts with the ability of the transferable protein to reform amino acids. One of the amino acids is not soluble in the urine, and if it is not absorbed, it will accumulate within the kidneys and form Kristin crystals or stones, and then find solid stones in the kidneys, bladder and ureter, and it can be very painful. 2.. The only known factor risk factors that lead to an increase in the risk of infection with the cystinia is the presence of a genetic mutation in the injured parents. The complications of the Cystinian night In the case of non -treatment of the cystinin, the patient may be exposed to the following complications: damage to the kidneys or bladder due to gravel. Urinary tract infection. Take a bacterial infection in the kidneys. Urea blockage. Diagnosis of Cysteine ​​-Diagnostic is made as follows: 1. Urine mounting examination is 24 hours. The diagnosis of the Cysetten night is initially when there are doubts that there are stones in the urine roads, by examining the urine deposits (urine mediment), where we can notice large amounts of cystine crystals. Cysteine ​​quantities are collected in urine within 24 hours to confirm the matter, where the quantities are more than 400 milligrams, while the right limit is 30 milligrams. In some cases, the secretion of cystine per day reaches 3000 milligrams. 2. Photo tests are used computerized tomography to ensure that there are stones in the kidneys. 3.. The genetic investigation is suitable for the genetic investigators who live in the risk of the family of the family of a person who is sick in cystinism. Cysteine ​​-night treatment is treated as the cystinin as follows: 1. The nutritional pattern is limited to the percentage of salts of no more than 2 grams per day as it helps to reduce the formation of gravel. 2. The acid budget is more soluble if it is in a basic urine, and the Cistine melt is increased by lifting the urine base by lifting one of the following: potassium citrate or acetazolamide. In addition to increasing the volume of urine by giving fluids in detail to reduce the concentration of the cystine. 3. Cysteine ​​-crystals are resolved by cystein by converting them to a compound that can solve urine by: penicillinein, or alpha -mothcaptopropionylglycine. Penlamine is more effective than others, but it is more side effects. 4. Surgery If the stones are large, or work to block kidney tubes, it should be surgically removed. In reality, there are not many operations that circulate, and one of the most prominent surgeries performed is the following: the kidney fragmentation by waves from outside the body (extractoreal shock wave). Perkutane nephrostolithotomy (percutaneous nephrostolithotomy) This procedure contains a special tool over the skin in two colleges to remove or break down stones. The prevention of cysteine ​​evenings cannot occur a night with the cystinin, in the case of genetic mutations with the parents, but drinking fluid can help prevent the formation of stones in the kidneys.