Polycystic kidney - Causes, symptoms and treatment. MF.

Polycystic kidney disease( autosomal dominant polycystic kidney disease of adults) is a kidney disease characterized by the formation and gradual increase of fluid-filled cysts in the tissues of both kidneys, which can result in terminal renal failure. Prevalence: 1: 500-1: 1000

The figure shows a healthy kidney on the left and affected by polycystic on the right.

Possible causes of polycystic kidney disease.

The disease is genetically conditioned, that is, inherited, with a dominant type of inheritance( that is, if the disease is in one of the parents, there is a high probability that it will manifest in all the children of the couple).

Reason in mutation of genes. There are variants of mutations of the polycystic kidney gene: a mutation in the short arm of chromosome 16;mutation in the chromosome 4. The localization of gene disorders affects the natural course of polycystosis in adults: at the 1 st type there is a rapid rate of development of renal failure, while at the second stage of polycystosis the favorable and development of terminal renal failure occurs after 70 years.

With this mutation, the formation of cysts begins in utero and continues throughout life. How does polycystic kidney disease occur in adults and the mechanism of cyst formation at the present time is not definitively determined.

Symptoms of polycystic kidneys

For a long time the disease does not manifest itself by itself, and cysts can become an accidental finding with ultrasound, and no person will be presented any complaints until a certain time. As a rule, the first symptoms of polycystic kidney appear to 40-50 years, in rare cases, to 60-70.The main symptoms are nonspecific, they can accompany many other diseases, but the clinical picture as a whole, together with the results of laboratory and instrumental research methods, allows the doctor to quickly diagnose.

Symptoms:

1. Pain. Localized pain in the lumbar region on both sides, as well as in the abdomen. The nature of pain is aching, pulling. These painful sensations are caused by a significant increase in the kidney, as a result of the expansion of the capsule of the kidney and compression of neighboring organs.
2. Hematuria( the appearance of blood in the urine).Symptom is optional, can be transient.
If you have blood in your urine, you should immediately contact your doctor( urologist, nephrologist), as the causes of bleeding may be different, and treatment should be selected depending on the provoking factor.
3. The increase in general weakness, loss of appetite, fatigue.
4. Abundant urination, often more than 2-3 liters per day, urine, usually light, "diluted"
5. Cutaneous itching
6. Increased blood pressure. Kidney diseases generally have concomitant arterial hypertension, and with this disease, the pressure is often also increased, the patient can not feel it, is detected with a random measurement of blood pressure.
7. Weight loss, loss of appetite.
8. Nausea, stool( diarrhea, constipation).

Also, with polycystic kidney complications are possible, in which you need to immediately seek medical attention:
- Inflammation of the cyst or pyelonephritis - i.e.infection. Since the cyst is a closed space, there is a high probability of suppuration of the infected cyst, so when pain intensifies in the lumbar region and the body temperature rises, it is necessary to consult a specialist( urologist, nephrologist) during 24 hours to select therapy - antibacterial, and in neglected cases - nephrectomykidney).
- Heart rhythm disturbance - slowing or appearance of extrasystoles. This is due to a violation of electrolyte blood composition, in particular, a violation of the excretion of potassium. To avoid this, you need to regularly take tests and, together with your doctor, adjust the therapy.
- Cyst rupture. It shows with pain and bleeding. It is also necessary to consult a nephrologist or urologist who will prescribe appropriate treatment and bed rest.

Diagnosis for suspected polycystic disease.

So, the person turned to the doctor with the above complaints or in connection with the genetic predisposition to this disease. What kind of research will need to be done?

- Clinical blood test. By results it will be possible to determine whether the patient has anemia( often associated with polycystic kidney disease), and whether there is inflammation in the body.
- Biochemical blood test( especially worth paying attention to the level of urea, creatinine, total protein, potassium, sodium, iron)
- General urine analysis( whether there are inflammatory changes, the presence of blood)
- Urine culture
- ultrasound of the kidneysdiagnosis of polycystic kidneys, allows to determine the presence of the disease, the size of the kidneys)
-the liver and pelvis of the liver( in some patients polycystic kidney is accompanied by cysts of the liver and ovaries)
- ECG( for assessing heart function, presence or absence of rhythm disturbances)

Leix polycystic kidney

Before the onset of the disease in childhood and adolescence, there is no specific recommendations, 1 every 1-2 years to do ultrasound of the kidneys, testing, and try to avoid colds.

In case of complaints and changes in the analysis, the patient is advised to start seeing the nephrologist at the nephrological center. For the maximum long-term preservation of kidney function, the patient should regularly go to the nephrologist and take a number of tests to timely correct possible violations.

First of all, the patient is explained the peculiarities of a way of life most favorable for his kidneys and the body as a whole. First, a certain diet: with signs of increasing potassium levels in the biochemical blood test, potassium-containing foods( nuts, bananas, potatoes, spinach, chips, dried fruits) are limited;restriction of the use of table salt;restriction of meat consumption. Secondly, do not limit drinking, provided that there are no edema, drinking should be even abundant - 2-3 liters / day. Thirdly, the strictest control over the level of arterial pressure - the target level is not more than 130/80 mm Hg. Fourthly, avoid contact sports, intense running, lifting weights. And fifth, do not allow chronic foci of infection in your body( carious teeth, inflamed tonsils, sinusitis) and hypothermia.

Actually, the treatment itself is aimed at the prevention and treatment of complications:

Antihypertensive therapy( beta blockers, calcium channel blockers, ACE inhibitors, less often diuretics) - in various combinations, depending on the concomitant diseases, as directed by the cardiologist and nephrologist. Funds that normalize blood pressure are very important, because with increased pressure, the kidney vessels are hypertrophied and gradually sclerosed, which leads to a disruption of blood supply and nutrition and an inadequately healthy kidney. All this leads to a fairly rapid loss of kidney function, which is why the control of blood pressure is so important.

Antibacterial therapy in the presence of infection( cephalosporins, fluoroquinolones) -dosage is adjusted depending on the renal function. When trying to cure inflammation in people's ways, the infection can spread or the cyst may be suppurated, which in advanced cases leads to the removal of the kidney. Therefore, prescribe antibiotics immediately with signs of inflammation in the tissue of the kidney, after receiving urine culture treatment can somehow be adjusted, the course lasts at least 10-14 days, can go up to 1 month, and then you can resort to taking phytospores: kidney tea, cowberryleaf, etc., but as an adjunct to the main treatment, but not in place of that.

Treatment of protein-energy deficiency - if necessary, designate Ketosteril or Supro, preparations of amino acids that at least partially can make up the protein deficit, while the restriction on the consumption of animal protein( meat) remains in force.
Treatment of anemia - depending on the level of hemoglobin and serum iron level, iron preparations and erythropoietin preparations are prescribed, a mandatory condition is the constant monitoring of the hemoglobin level, since its excess increase is also harmful.

Treatment of phosphate-calcium disorders: depending on the level of calcium, phosphorus and par-hormone in the blood serum, calcium or alfa-calcidol preparations are prescribed.
The outcome of the disease is terminal renal failure - that is, a state where the kidneys are no longer capable of filtering blood from harmful substances, and in order to save the patient's life, it is necessary to begin substitutive renal therapy. The task of the nephrologist is to determine the moment when it is necessary to begin hemodialysis. This is determined by the level of glomerular filtration rate, the calculation is based on a formula based on laboratory data. Hemodialysis - a constant outpatient procedure, usually performed 3 times a week for 4-5 hours, is to cleanse blood from metabolic products and surplus water. As a rule, a fistula is formed in advance on the arm for a more convenient and painless procedure. Visits to hemodialysis procedures should be permanent. With terminal renal failure, skipping two or three procedures can be completed for a patient with a lethal outcome. Hemodialysis from a certain stage becomes permanent( 3 times a week) and inalienable.

Patient during hemodialysis procedure.

With regular visits to hemodialysis procedures, observance of all the recommendations of the attending physician, careful monitoring of blood pressure, the life expectancy of such patients is compared with the overall life expectancy in the population.

Also a possible method of treatment in the future is kidney transplantation with a constant intake of immunosuppressive therapy.

There is no prophylaxis in the disease. Currently, scientists are developing drugs that suppress the growth of cysts from the beginning, but research so far only at the experimental stage, the drug has proved successful in mice.

Article written by a nephrologist doctor Sirotkina EV