multicystic kidney disease

Polycystic Kidney - congenital disorder in which both kidneys appear and progressively increase cysts, leading to atrophy of parenchymal functioning. It refers to the hereditary anomalies of development and is often found in families. The disease progresses in the course of life, most often seen and diagnosed at the age of 20-40 years, but sometimes found in children, in the elderly.

Etiology, pathogenesis. The cause of the anomaly is unknown. Pathogenesis is caused by defect of embryonic development of the tubules, some of which is transformed into a cyst. The kidneys are increased in most patients, comprise a plurality of cysts of various sizes, which are located between the portions of parenchyma preserved, sometimes substituted by connective tissue. Cups and pelvis are compressed and deformed. Cysts can fester.

Symptoms within. Usually the disease for many years asymptomatic. Polycystic can be discovered by accident in the survey on the operation or at autopsy. This is due to low specificity of symptoms in the early stages of the disease. When the mass of functioning parenchyma is significantly reduced, disturbed concentration ability of the kidneys, patients note polyuria and thirst, and then deterioration of appetite, decreased ability to work, appear dull pain and feeling of heaviness in the lumbar region, headache. These most frequent subjective symptoms of polycystic kidney disease are complemented with objective data. Polyuria sometimes reaches 3-4 liters / day. The urine is colorless, low relative density. Observed nocturia and izostenuriya. Proteinuria and the daily loss of protein are minimal; and expressed little cylindruria. The urine sediment continuously detect red blood cells observed gross hematuria. Occasionally gross hematuria is the nature of profuse, life-threatening kidney bleeding. Pyuria are often indicative of concomitant infections, sometimes leading to the attack of pyelonephritis and festering cysts. In these cases, fever (sometimes with a fever), worse pain in the kidneys, increases toxicity. On palpation palpable enlarged, nodular, dense and painful kidney.

Polyuria promotes the excretion of metabolic products, and for a long time, azotemia may not be, but over time azotovydelitelnoy kidney function is impaired and there is azotemia. During this period, the patient's condition worsens, an unpleasant taste in the mouth, nausea. Usually azotemia with polycystic kidney disease progresses slowly, but renal bleeding, festering cysts, as well as trauma, surgery, pregnancy and childbirth often accelerate the development of azotemia. Progression of the disease also contributes frequently concomitant hypertension, which in most cases is mild, but sometimes occurs malignant. Hypertension disorders accompanied by cardiac hemodynamics and left ventricular hypertrophy. In the later stages of the disease there are other signs of anemia and renal failure (see. Renal insufficiency).

The diagnosis of polycystic kidney disease is based on anamnesis, often pointing to the same disease in relatives, on palpation of data (sometimes the patient himself said that the probes in his hypochondria dense, hilly education) and the study of kidney function. In early polycystic kidney function is impaired concentration and found gipoizostenuriya. Glomerular filtration is reduced significantly slower. Although radionuclide renal scan often gives a picture of inherent polycystic, radiographic methods are the most accurate in detecting the disease. On plain film see the shadow of enlarged kidneys on urograms intravenous and retrograde pyelogram - deformation characteristic elongated cups and pelvis. With renal angiography determined the cysts in the kidneys increased.

Polycystic kidney must be differentiated from chronic glomerulonephritis and pyelonephritis, when the fore symptoms of renal failure, and tumors of the kidney, if the disease appears asymptomatic hematuria. Unlike glomerulonephritis and pyelonephritis with polycystic kidney increased in size and give a characteristic X-ray picture. Unlike kidney tumor disease always bilateral and accompanied by dysfunction of both kidneys.

Treatment is only symptomatic. Patients should avoid excessive loads, long distance and bumpy ride, beware of infections, colds, monitor the status of the nasopharynx and teeth. When gross hematuria patient must comply with strict bed rest, which in most cases provides hemostasis hemostatic drugs without prescription. During the attack, joining pyelonephritis prescribe antibiotics and uroantiseptiki (see. Pyelonephritis} given reduced kidney function. Reduced kidney function causes the diet with restriction of protein and salt, but the food should be sufficiently nutritious and rich in vitamins. A strict protein restriction is necessary only when the level of .. plasma creatinine more than 0.02 g / l (see Renal insufficiency} In severe hypertension used antihypertensive agents, and with reduced urine output - diuretics: Lasix, hydrochlorothiazide, aldactone, etc. Surgery - opening cysts - only useful when their festering. . In end-stage renal failure can be applied hemodialysis and kidney transplantation.

Forecast. In most cases the disease leading to renal failure in different periods from the beginning.