Polycystic kidney disease

Medicine2

Polycystic kidney disease is a congenital disease characterized by the formation and growth of cysts in both kidneys.

Polycystic kidney disease is a congenital anomaly of their development. This is the most frequent developmental defect, it is often combined with polycystosis of other organs (liver, pancreas, lungs, etc.). The disease is often of a family nature. As a rule, both kidneys are involved in the process.

Cysts are formed at the stage of the secondary kidney as a result of the absence or incorrect attachment of the secretory and excretory elements of the nephron. This leads to a violation of patency for primary urine, increased pressure in the tubules of the kidneys and the expansion of their lumen. Cysts contain uric acid, urea, mineral salts, etc. The pressure of the cysts on the kidney parenchyma leads to its ischemia and atrophy.

In polycystosis, the size and mass of the kidneys are significantly increased, their surface is uneven, bumpy due to protruding cysts. The wall of cysts consists of connective tissue, the inner surface is lined with cubic or flat epithelium. Cysts contain a liquid with low density, yellow or brown in color, reminiscent of urine. Between the cysts are the remains of the parenchyma in a state of dystrophy. Loewers and cups are significantly enlarged and deformed. With polycystic pyelonephritis, tuberculosis of the kidneys, kidney stone disease, etc. are often observed.

Symptoms of polycystic kidney disease

  • Aching in low back.
  • Blood or pus in the urine.
  • Infections.
  • High blood pressure.
  • Nausea, vomiting.

The causes of polycystic kidney disease

There are two types: the infantile and the adult. Possible polycystic kidney disease with autosomal dominant type of inheritance, if this disease affects one of the parents, less often - with autosomal-resistive type of inheritance, if both parents are carriers of the disease. Polycystic kidney disease occurs due to impaired formation of the kidneys of the fetus in the womb of the mother. Sometimes, the cells necessary for kidney function are insufficiently developed. Cysts are also formed in the absence of a connection of the renal tubules with glomeruli or in the degeneration of the kidney glomeruli.

Polycystic kidney disease in children is usually extremely unfavorable and early ends with death from uremia.

Polycystic kidney disease in adults flows relatively slowly, passing through 3 stages: compensation, subcompensation and decompensation. In the compensation stage, complaints and symptoms of kidney damage are still absent. After about 10-15 years, there are complaints of pain of an uncertain pressure in the lumbar region, a feeling of fullness in the abdomen, caused by a significant increase in the kidneys. There is fatigue, in connection with the increase in blood pressure, headaches occur, periodically marked as "unmotivated" hematuria. During the examination during this period, significantly enlarged, often painless kidneys are revealed. The kidney function is not broken yet. With intravenous urography, there are significantly enlarged kidneys with a deformed cup-and-pelvis system. Diagnosis is assisted by ultrasound of the kidneys, nuclide scintigraphy and selective angiography of the kidneys.

The stage of subcompensation is characterized by the appearance of signs of renal insufficiency. There is thirst, dry mouth, headaches become worse, hypertension becomes high and resistant. The function of the kidneys decreases, hypo- and isostenuria is noted, erythrocytes, cylinders are present in urine tests, when pyelonephritis is attached to leukocyturia.

Suppuration of cysts is accompanied by fever, chills, leukocytosis, increased ESR. Kidney stones can cause attacks of renal colic.

The stage of decompensation is characterized by the development of terminal uremia. The disease is relatively slow, for years, but differs by an unfavorable prognosis as a result of gradual formation of chronic renal failure. A sharp deterioration occurs when secondary infection, such as influenza, pneumonia, etc., suppuration of cysts with the development of urosepsis, etc., lasts. The life expectancy of a patient with polycystosis after diagnosis is usually 10-15 years.

Treatment of polycystic kidney disease

Polycystic kidney is treated symptomatically. In the absence of chronic renal failure, a diverse, high-grade and sufficiently energetically valuable diet is prescribed. For the treatment of hypertension, antihypertensive drugs are prescribed, with pyelonephritis - antibiotics and uroseptics, taking into account the functional state of the kidneys. Suppuration of cysts, obstruction of urinary tract with stone and some other complications may require surgical intervention.

Of single cysts, fluid is usually removed by puncture. In this case, under local or general anesthesia, a thin hollow needle is made by puncturing the kidney from which the fluid is sucked, in addition, using a needle, take a tissue sample of the cyst. Sometimes before a puncture it is necessary to make a small operation for simplification of access to a kidney. However, polycystic kidney disease in this way will not be cured. The onset of symptoms and their distinctness depends on the size of the cysts, their nature and quantity. In the worst case, the ability of the kidneys to filter urine is impaired. Uremia begins, the patient is connected to an artificial kidney, hemodialysis is performed. With a donor, a kidney transplant is done.

Unfortunately, you can not do anything on your own.

In all cases of kidney dysfunction, you should consult your doctor. Kidney disease can be manifested by pain in the lumbar region, the presence of blood in the urine and infection of the urinary tract,

The doctor after careful collection of anamnesis, will examine the patient. With the progression of the disease, he will discover an enlarged kidney and establish a preliminary diagnosis. Will make an analysis of urine. The urine often contains blood or pus. To clarify the diagnosis, you can use other research methods: urography - ultrasound, kidney scintigraphy, computed tomography. With the help of these studies, the doctor can determine what kind of cysts.

Course of the disease

Congenital polycystic kidney can begin when the fetus is formed, in this case the disease is diagnosed already in the newborn. With congenital polycystosis of the kidneys and the presence of multiple cysts, a child is born dead or dies in the first year. In the presence of fewer cysts, the patient's condition worsens gradually with the growth and intensification of cyst pressure on the kidney tissue. The disease can begin in childhood, but then the cysts are very small and often invisible. As the child grows, the disease progresses, the kidneys increase. Polycystic kidney is formed for many years. The first symptoms appear in an adult: back pain, blood in the urine, recurring urinary tract infections, renal colic (it is associated with bleeding from the cysts). The cause of approximately 10% of all cases of kidney failure is congenital polycystic kidney disease. Usually this happens at the age of 40-60 years.

Sometimes there is a transformation of the cavities of the body, covered with a mucous membrane, if a blockage of their excretory ducts in them accumulates liquid. Cysts can form in the place of chronic hematomas, as well as in the place of blockage of the sebaceous glands.

 

Related:
1. Amyloidosis of the kidneys
2. Glomerulonephritis
3. Интерстициальный нефрит
4. Acute Renal Failure
5. Chronic Renal Failure