Polycystic kidney disease in children

Polycystic kidney disease is a hereditary disease that affects children in infancy, in adolescence and adolescence. This disease, in which a number of cysts are formed in the kidney parenchyma, which progressively increase in size, cause compression of nephrons and gradual atrophy. Often this kidney disease in children is combined with polycystosis of the pancreas, liver, spleen, lungs, ovaries.

What are the forms of polycystic kidney disease in children?

There is an infant and adult form of polycystic kidney disease. In children, the infantile form of polycystic kidney is more than 30% of the cases of this disease and is characterized by intensive development in the kidneys of small-cystic changes during intrauterine development. Polycystic form of adults in children is characterized by a slow increase in kidney size and signs of kidney failure. The presence of polycystic kidneys( in the parenchyma) a large number of immature and dysplastic nephrons, sclerotized vessels, is a factor in the occurrence of polyenitis.

In polycystic kidneys in infants macroscopically the kidneys are quite large in size with many cysts. About 90% of the parenchyma covers the cyst, mostly the collecting tubes of the kidneys( both).There are frequent vomiting in newborn children, rapid increase in weight, palpated enlarged kidneys. Reduces the concentration ability of the kidneys, increasing the amount of urea and residual nitrogen in the blood. In some cases, there is an increase in blood pressure. After a short while in children the liver failure grows and later about 80% of them die within the first month of life, and the rest - up to a year of life.

This kidney disease in early and older children is similar to polycystic in infants in terms of morphological changes, but it persists in the kidneys from 40 to 75% of the parenchyma intact. This explains the less severe manifestation of the disease, as well as its duration( up to several years).But later, due to the interstitial sclerosis, the size of the kidneys decreases. The kidney failure progresses, liver function disorders simultaneously increase.

In polycystic patients, cysts occupy about 10% of the total volume of the kidney parenchyma. In the clinical picture, portal hypertension and liver function disorders are at the forefront of the disease. This is due to periportal fibrosis. Progression of changes in the renal parenchyma is observed in the future due to cystic dilatation of nephrons( functioning).Further, compression develops, as well as sclerosis of the renal tissue. The glomeruli can be hypertrophied, but as the process develops, their hyalinosis arises. Later, chronic liver failure develops.

How is polycystosis diagnosed in children?

Diagnosis of polycystic kidney disease in children is based on a clinical picture of the disease. The diagnosis can be confirmed only after a series of instrumental examinations. This is a computed tomography, scintigraphy, ultrasound examination. Analyzes of urine and blood are also needed. To clarify the polycystosis of the kidneys, puncture biopsy of the liver is often used.

Treatment of polycystic kidneys

There is no specific treatment for polycystic kidney disease in children. With the development of this disease in children of early and adolescence produce therapeutic measures that are aimed at stopping the progress of renal and hepatic insufficiency. The main focus in this regard is on the treatment of urinary tract infections and the treatment of hypertension. With the development of renal failure, there are no specific features of treatment for children. Treat kidney failure with the usual methods for this case. In recent years, with the development of portal hypertension, children are using splenorenal or portovascular shunting. Children with chronic terminal renal failure, as well as with severe renal failure, are recommended to combine kidney transplantation and liver transplantation.