Forecast renal disease is associated primarily with the development of renal failure functional abilities.That is why the special studies are conducted to study the risk factors for the progression of nephropathy and the development of chronic renal failure.At the present time it is clearly defined that in childhood a risk factor for chronic kidney disease is the presence of a child signs of structural renal dizembriogeneza (violation of the kidney tissue), which may depend on genetic and teratogenic effects.Risk groups of chronic renal failure in children are many congenital kidney disease.
One of the main methods of treatment of chronic renal failure in hereditary and congenital diseases of the kidneys is kidney transplantation.However, when deciding on the renal transplantation should be evaluated as a potential donor parents, as genetic defect they can be hidden.
grafts can be divided into 4 groups:
1) autologous (taken from the same individual) that is not in renal transplantation is carried o
2) syngeneic (taken from immunogenetically identical individual, such as identical twins);
3) allogeneic (from the individuals of the same species, is immunologically distinct from the recipient);
4), heterologous (from another individual of the species, is immunologically distinct from the recipient).
During the autologous transplantation of monozygotic twins is no immunologically-mediated graft rejection.For difficulties in organ transplantation in allogeneic and xenogeneic conditions transplant compatibility antigens responsible various tissues of living organisms.The sum of all transplantation antigens controlled by a specific genetic locus is histocompatibility system.
genetically programmed antigen - HLA system (.. MHC genes, ie tissue compatibility between them) are the biggest obstacle to successful allogeneic renal transplantation.The number of the currently known (several tens of) different antigens allows for a very large number of combinations.
Yet a number of systems (minor histocompatibility complex) is important for transplantation should be considered when choosing the donor and recipient.These include the ABO blood groups, Rh-factors and Lewis-factor, since they are found not only in red blood cells, but also in other cells.Antibodies, which are located within cells, playing transplantation more subordinate role.
Some immunological processes are very important for a kidney transplant.Immunological tolerance (tolerance), which is observed in the fetus or a newborn baby can be achieved, and with a special immunological effects on the body of an adult.One of these methods is the whole body ionizing irradiation, followed by bone marrow replacement.
Many mechanisms may be useful for these purposes, such as warning output alien to the recipient (ie. E. A man who carried a donor organ transplantation) antigens of the graft, warning the immune response to antigens of the transplant or organ protection with specific antibodies.The progress made on ways to improve measures aimed at the prevention of transplant rejection, closer to practice than the hope of achieving complete immunotolerance (tolerance recipient immunity in transplantation of organs and tissues).
Along with steroids (hormones) and cytostatics in renal transplantation great warning significance for the prevention of rejection is cyclosporin A.
treatment. Treatment of renal disease associated with genetic and teratogenic (pathogenic to the fetus in utero) influences - very difficult, but not hopeless task.
number of diseases related to the urinary system, which can be diagnosed using the methods of antenatal (prenatal) diagnosis of small.However, due to the development of fetal surgery (surgery of the fetus), the identification of severe congenital malformations ultrasonic methods of research have become critical.Among the various defects that can be diagnosed by fetal echolocation undoubtedly promising is the identification of malformations of the urinary system.With increasing frequency in the literature report the success of surgical intervention when the child is still finding suffering from a hereditary disease in the womb.In addition to this non-invasive method of investigation, as ultrasound, with the aim of prenatal (antenatal) diagnostics are currently used amnio-and Fetoscopy, contrast radiography, biopsy of chorionic cells and amniocentesis followed by cytogenetic, biochemical and immunological studies of amniotic fluid and cell culture.Timely identification of amniotic fluid alpha-fetoprotein is practically decided in Finland, the Finnish version of congenital nephrotic syndrome problem.Equally promising definition of α-fetoprotein for the diagnosis of this severe syndrome with renal polycystic what is Meckel syndrome.The families of high-risk groups such diseases is carried out such studies in pregnant and upon detection of said protein pregnancy promptly interrupted.In cases where the diagnosis of monogenic inherited diseases the child is confirmed, the decision about the child's birth family must be taken that raises many ethical problems.The latter is due to a large extent by the fact that the idea of the inevitability of the disease of the urinary system organs in the population is minimal, and the disease can manifest itself not only in childhood but also in the later age periods.With the development of medicine and clinical genetics in the future it will be possible impact on the level of the cell's DNA.
currently used palliative effects.Widespread is diet therapy, which is called "Engineering environment."In particular, for the oxalate nephropathy is widespread antioksaluricheskaya diet, which is recommended to be used 3-4 non-individual courses at an early age when it detects signs of oxalate diathesis.This diet is recommended for the next of kin, since the origins of the disease - family.
Palliative therapies - is the use of drugs, thereby stabilizing the membrane of the body, preventing the development of cells of many pathological processes.These drugs include dimsfosfon, ksidifon and complex B vitamins, A and E, which are also antioxidants.Improving kidney function is achieved by using ATP, cocarboxylase, as shown in some cases dizembriogeneza renal (kidney disorders intrauterine development).With the development of nephropathy (kidney disease) in connection with purine metabolism disorders widespread receives allopurin that blocks the formation of uric acid.It is clinically manifested after 24-48 h a decrease in the level of urate in the blood and urine.However, you need to remember about the possibility of complications - raising the level of xanthine in blood, which is fraught with the complications.
Diagnosis of hereditary or congenital disorders without layering of kidney damage in violation of the functions of the immune system and provides for limitation of therapeutic activity, especially in the use of steroid medications (hormones), and immunosuppressive drugs (drugs that reduce the activity of the immune system).Immunosuppressants and corticosteroids are contraindicated in hereditary nephritis, apparently because they can cause an imbalance of the coping mechanisms that develop in cases of renal defect of genetic origin.Detecting kidney biopsy evidence of structural damage is a contraindication for appointment of indomethacin, as these children revealed a decrease of renal function.With the development of end-stage chronic renal failure in children with hereditary and congenital nephropathies can be performed kidney transplantation leads to a distinct improvement of patients, partly dependent on the fact that the transplanted organ compensates for the missing synthesis of proteins and enzymes, which are absent or present in the minimum quantities in hereditarykidney disease.Currently, however, there were opportunities for the development of data rapidly progressive glomerulonephritis in patients with Alport syndrome after renal transplantation, which is associated with the presence in the graft antigen absent in patients prior to organ transplantation.To improve the development of children with kidney transplant using growth hormone.Children who are on dialysis, used erythropoietin (a substance that stimulates the formation in the bone marrow into the bloodstream and yield of mature red blood cells), the use of which also solves the problem of the treatment of anemia.
Due to the small capacity of drug therapy of hereditary nephropathy in the first place nominated by the prevention of the problem of development.Currently defined range of diseases and conditions for which necessary to carry out medical and genetic counseling in terms of the possible development of diseases of the urinary system.The task of genetic counseling include a definition of the role of heredity in the development of nephropathy detected and determination of disease prognosis in the offspring.All these issues are increasingly being studied in nephrology and should be the subject of attention pediatrician, nephrologist, physician, obstetrician-gynecologist and urologist.