What does the examination of hyperplasia of accessory glomerular organ have?

summary

Bartter's syndrome is an autosomal recessive disorder, which was first reported by Bartter (1962), so it is called Bartter's syndrome. Its clinical features were severe hypokalemia and metabolic alkalosis, accompanied by hyperrenin and hyperaldosteronemia, hyperplasia and hypertrophy of paraglomerular organs, and dysfunction of sodium retention and concentration of renal tubules, but no hypertension and edema, and no response to exogenous angiotensin II. It is considered that the syndrome is caused by mutation of ion channel gene. So what does the examination of hyperplasia of paraglomerular organ have?

What does the examination of hyperplasia of accessory glomerular organ have?

The main points of diagnosis are: 1. Hypokalemia (1.5-2.5 mmol / L); 2. Hyperkalemia (> 20 mmol / L); 3. Metabolic alkalosis (plasma HCO3 - > 30 mmol / L); 4. Hyperreninemia; 5. Hyperaldosteronemia; 6. Insensitivity to exogenous vasopressin; 7. Proliferation of paraglomerular apparatus; 8. Hypochloremia (urine chlorine > 20 mmol / L); 9. Normal blood pressure; 10. Renal biopsy is in accordance with the characteristics of the disease, and the diagnosis can be made according to the clinical manifestations of the disease

The cause of the disease has not been determined. Most scholars believe that it is an autosomal recessive genetic disease. There have been reports of 5 cases of Bartter's syndrome in 9 siblings of a family and 4 cases of Bartter's syndrome in 2 generations of a family. Modern molecular biological techniques have also revealed that Bartter's syndrome is caused by mutation of ion transporter gene in renal tubular epithelial cells. At present, Na + - K + - 2Cl - gene mutation has been found in infantile Bartter's syndrome, which is located in 15q12-21 and has 16 exons, More than 20 mutations have been found in the Na + - K + - 2Cl - channel, which encodes 1099 amino acids. The classical Bartter syndrome is caused by the mutation of cicnkb gene, which is located in 1q38 and encodes the Cl - channel on the basal side of the cell containing 687 amino acids, It is caused by a mutation in the thiazide sensitive Na + - K + channel gene (sci12a3), which is located in 16q913 and encodes 1021 amino acids. Up to 40 mutations have been found. In addition, mutations of potassium channel base were found in some patients. Therefore, battery syndrome can be identified as a clinical syndrome caused by the above ion channel gene mutations. Lipoprotein glomerulopathy is a kind of renal disease, which is characterized by the presence of lipoprotein emboli in the glomerular capillary loops and no lipoprotein emboli outside the kidney. Lipoprotein glomerulopathy was more common in men, the ratio of male to female was 15:8; The average age of onset was 32 years (4-49 years). Most of the cases were sporadic and a few were familial.

Prostaglandin synthetase inhibitors such as indomethacin, ibuprofen and aspirin can improve clinical symptoms and correct hyperreninemia and hyperaldosteronemia. Indomethacin (Xiao Yantong) is the most effective, with a dosage of 2 to 5mg/ (kg * d) to avoid water and sodium retention. Ibuprofen can be used to replace indomethacin resistant cases. Spironolactone (spironolactone) 10-15 mg / (kg · d) or aminopteridine 10 mg / (kg · d) can be used as potassium preserving diuretics.

matters needing attention

There is no definite conclusion on the cause of the disease and no exact preventive measures. After the diagnosis of the disease should be actively symptomatic treatment to prevent complications. Should eat acidic food; It is suitable to eat food with high potassium content; Should eat the food that has diuretic effect. Wax gourd, with a certain diuretic effect, can reduce the burden of kidney and cardiovascular, thereby reducing the risk of proteinuria and new functional failure. Zizyphus jujuba, rich in vitamin C and citric acid, has a good nutritional effect on tissues and nerves.