April
21
23:02
Urinology

Multicystic kidney disease

Polycystic kidney

multicystic kidney disease Polycystic kidney disease - a genetic disorder that is characterized by the formation and subsequent growth of cysts in the kidneys.This disease often runs in families, it is most commonly diagnosed between the ages of twenty to forty years, but may also occur both in children and in the elderly.According to the statistics - is the most common malformation of the kidneys.Typically, this process involves two kidneys

etiology, pathogenesis

causes of this anomaly is not known.Currently we identified several genetic defects that lead to polycystic kidney disease.Some of them are caused by dominant genes, and a very rare type - recessive gene (disease develops only in those cases where one person has inherited from each parent, or from any one of the parents copy of the gene).If recessive inheritance - a disease begins to develop in early childhood, while dominant - after reaching the age of majority.

in both kidneys begin to form multiple cysts that gradually increase in

size, squeeze and destroy small and sometimes a considerable part of functional renal tissue.

As the disease, increasing the cysts leads to a reduction of blood flow through the kidneys, resulting in the formation of scarring.Also, kidney stones are formed that often leads ultimately to kidney failure.

This genetic defect may also be accompanied by the development of cysts and other internal organs (pancreas, liver, and so forth.)

Symptoms and flow

In most cases, this disease occurs dominant type and for many years runs virtually asymptomatic.Sometimes polycystic found quite by accident in surveys, during surgical procedures, or in some cases even at autopsy.

When a recessive form of the disease (very rare) that develops in early childhood, and an increase in the number of kidney cysts reached so high indicators that this leads to a clearly pronounced protrusion of the abdomen.In severe cases, the risk of death of the child shortly after birth is very high, as is already near the fetus develops renal failure, which is accompanied by underdevelopment of the lungs.The liver is also affected, whereby the age of ten children affected by the disease are at increased pressure in the blood vessels, connecting the liver and intestines, resulting eventually develop liver and kidney failure.

The most common symptoms include: discomfort and some soreness in the stomach or in the upper quadrant, intensive periodic cramping (due to kidney stones), frequent urination, blood in the urine (possibly with pus).Also, there is a general fatigue, depression, nausea and other symptoms gradually developing renal failure due to progressive loss of kidney function of the tissue.Frequent urinary tract infections may also accelerate the development of renal failure.

have about 30% of patients suffering from the dominant form of polycystic kidney disease, there is the presence of cysts in the liver, but they do not affect the proper functioning of the liver.In 10% of patients the areas of expansion of the blood vessels located in the brain - the so-called aneurysms, which are often expressed in quite severe headaches

Treatment

In this disease only symptomatic treatment.Patients should avoid the long walk, bumpy ride, excessive exercise, colds, closely monitor the condition of the teeth and nasopharynx.In the case of gross hematuria is necessary to observe strict bed rest, which in most cases leads to stop bleeding without a hemostatic drugs.In the case of acceding to polycystic pyelonephritis - appointed uroantiseptiki and antibiotics.Reduced kidney function leads to high-calorie diet rich in vitamins, with a mandatory restriction of the use of salt and protein.If decreased urine output are used diuretics (aldactone, hydrochlorothiazide, Lasix and so on.), And in severe hypertension - antihypertensive agents.Surgical intervention (opening of cysts) is appropriate only in cases of festering.

timely and effective treatment of hypertension and urinary tract infections can significantly slow down the progressive destruction of renal tissue, but even in spite of this, more than 50% of patients eventually develop kidney failure, which without chronic dialysis or without kidney transplant inevitably leads todeath.

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