polycystic kidney disease, or polycystic disease (degeneration) - a genetic malformation that affects one or simultaneously two kidneys.
When polycystic kidney tissue cysts formed numerous small, fluid-filled and breaking normal kidneys.
Causes
The incidence of the disease - 1 in 10,000 newborns in autosomal-recessive type of inheritance.
If the family previously been cases of cystic renal abnormality, the frequency is increased to 1: 250-300 newborns, and is usually detected autosomal dominant pattern of inheritance.
Development of polycystic kidney disease and other similar anomalies associated with impaired renal bookmarks in the early weeks of intrauterine life.
It happens in severe toxemia of pregnancy, the development of infectious diseases in terms of up to 6-8 weeks of pregnancy, when taking nephrotoxic medications (antibiotics), etc.
The role of heredity, especially if kidney disease identified through the maternal line.
There are two types of the disease:
- polycystic kidney type of child,
- polycystic kidney adult type.
development mechanism
As a result of the impact of adverse factors in the process of laying the kidney tissue, the formation of kidney nephrons, but the collecting ducts are not available or do not have the kind of normal tubule, these tubules are not duct. As prenatal development of the child, the nephron is finally formed and begins to function.
However, he does not have access to the urinary tract, expands and is transformed into a typical cyst. Most cysts are formed in the cortex, they are completely isolated, and have no communication with lying underneath excretory ducts.
If all, or nearly all, lays the nephrons do not have excretory ducts, cystic degeneration exposed almost all kidney parenchyma.
Such a defect is determined before birth and called multicystic kidney. Lesion may be single- or double-sided.
If a considerable part of collecting ducts of nephrons no significant amount of cysts (50 or more) is formed on the surface of the kidney. This condition is called polycystic kidney on child type.
As a child grows, the cysts grow in size, are attached infectious complications, because of what quickly perish areas initially healthy kidney tissue. In this case, the clinical manifestations occur in early childhood.
If collecting ducts are not connected to only a small portion of nephrons, cysts are formed (in different numbers - 20 to 50) and thus remains sufficient mass renal tissue, completely functioning. This form is called polycystic kidney adult type, clinical manifestations may appear late or may even be completely absent.
Manifestations of polycystic kidney
As a result of proliferation of existing cysts, kidney greatly increase in size, become deformed, becoming like a bunch of grapes. Inside the cysts contained liquid tight and they compress portions of normal renal tissue.
Clinical symptoms of polycystic kidney disease depend on whether it will be one-sided or two-sided lesion, and also depends on the prevalence of cystic tissue.
In severe polycystic kidney disease manifests in early childhood with symptoms such as:
- stable leucocyturia (Identifying each assay a large number of leukocytes urine)
- transient haematuria (periodic appearance in the blood urine)
- proteinuria (albuminuria),
- gipoizostenuriya (Constant decrease of urine density, ie the kidneys are not able to perform its primary function of concentration of urine)
- soon joins arterial hypertension (Persistent elevation of pressure).
Progressive for polycystic kidney disease, especially in children, results in the development chronic renal failure.
When adult polycystic type symptoms appear early in a school or a teenager, there are complaints:
- a dull ache in the lower back,
- of fatigue,
- headaches, high blood pressure,
- revealed signs pyelonephritis (Kidney inflammation).
Diagnostics
Treatment of polycystic kidney disease - this is the task of Urology.
Polycystic kidney disease is detected at this stage still in utero at skrinningovom ultrasound pregnant period after the 30th week.
After the birth of a child diagnosed based on
- family history data (have relatives with abnormalities or renal diseases previously born children with polycystic)
- child's examination with the identification of the typical complaints
- changes in urine analysis (increase in white blood cells, erythrocytes, The density decreases).
Suspected abnormalities kidney structure allow recurrent pyelonephritis in the first year of life.
Visually polycystic kidney change is detected at ultrasoundOr during the excretory urography.
Additional methods are used angiography (study of the kidney blood vessels with contrast) and computer or magnetic resonance imaging to clarify the kidneys of polycystic type.
When held polycystic kidney liver ultrasound, Ovary and pancreas, as polycystic kidney disease is often associated with cystic these bodies.
It is necessary to distinguish the individual from polycystic kidney cysts and neoplastic processes - Wilms tumor or nephroblastoma.
Treating polycystic kidney
All treatments can be distinguished on the conservative and surgical.
For conservative treatment of polycystic kidney disease include:
- pyelonephritis secondary active treatment (antibiotics and uroseptiki, diet, fluid intake, special solutions)
- elimination of hypertension,
- Correction fluid and electrolyte disturbances due to the inability of the kidneys to concentrate urine.
With the development of chronic renal failure, hemodialysis is necessary (blood purification on artificial kidneys) and renal transplantation.
Surgical treatments
With the rapid increase in the amount of cysts are used surgery:
- puncturing cysts thin needle through the abdominal wall under ultrasound guidance with the removal of liquid,
- Laparoscopic emptying cysts in order to reduce their volume and to improve blood supply of normal kidney tissue.
- kidney transplant.
forecasts
The prognosis of polycystic kidney depends on the severity of lesions and the development of complications.
With recurrent pyelonephritis, persistent increase of pressure and the development of renal failure poor prognosis. Often require a kidney transplant.