Congenital nephrotic syndrome in newborns treatment. Causes and mechanism of development of nephrotic syndrome in children. Symptoms and course

Terminology... Congenital nephrotic syndrome is understood as NS that develops in a child up to 3 months of age. Congenital HC can be primary, genetically determined and secondary in congenital cytomegaly, toxoplasmosis, syphilis, tuberculosis, renal vein thrombosis, AIDS. A special place among congenital HC is occupied by the primary hereditary, the so-called congenital nephrotic syndrome of the Finnish type. This is an autosomal recessively inherited pathology that manifests itself from the first days of a child's life with severe nephrotic syndrome with large proteinuria and severe hypoproteinemia. In the "natural" course of death occurs up to 1 year, and it leads to either the development of renal failure, or septic complications.

History and epidemiology... The disease was first described in 1966 by R. Norio. When analyzing parish books in the southwestern region of Finland, where this disease was most common, the founder of pathology was found - a Finn who lived in this region in the second half of the 16th century. Before antenatal diagnostics, the disease occurred with a frequency of 1: 8200 births. Similar cases are reported in the northwestern region of Russia, in the Leningrad region. It is not always possible to confirm the ethnic (Finnish) affiliation of the family. This variant of the pathology has been repeatedly described in various countries of the world in persons of non-Finnish nationality.

Clinical characteristics... The course of pregnancy is difficult, childbirth is usually premature, the mass of the placenta is more than 1 / 4-1 / 2 of the mass of the newborn. More often, a child is born already with pronounced edema, but they may appear somewhat later - by the end of the first month of life. Proteinuria reaches 10 g per day. Hypoalbuminemia is sharply expressed, there is an increase in serum lipids. With a decrease in the edema syndrome after the introduction of diuretics, attention is drawn to the sharp dystrophy of the child, multiple stigmas of dysembryogenesis. The indicators of immune protection are sharply reduced, which is the basis for the development of purulent complications. Thromboembolism is possible. Blood pressure is reduced or within normal limits. The amniotic fluid and blood serum of pregnant women contain a high titer of alpha-fetoprotein. The detection of this phenomenon allowed for timely antenatal diagnostics.

Morphology and pathogenesis... Histological examination of the kidneys reveals microcystosis of the proximal tubules in the corticomedullary zone, multi-glomerularity and other signs of renal tissue immaturity, proliferation of mesangial cells, and fibrotic changes.

Finnish-type congenital nephrotic syndrome refers to glomerular diseases, and the gene product - nephrin - is localized on podocytes. Lack of nephrine causes proteinuria even in the antenatal period of the child's development.

Genetics... Finnish-type congenital HC is inherited in an autosomal recessive manner. M. Kestila et al. In a study of 17 families with this pathology, no defect was found in any of the genes of the alpha-1-, alpha-2-, alpha-3- and alpha-4-chains of type IV collagen, as well as the main genes of the laminin and hepa-aransulfate chains. proteoglycan, encoding the main components of BM glomeruli. Convincing data have been obtained that the mutant gene is localized at 19ql3, this gene - NPHSI - encodes a transmembrane protein, nephrin, inherent in podocytes.

Modern studies have found that in various regions of the world where congenital NS, which is essentially close to Finnish, was detected, there are about 40 mutations of the NPHSI gene. However, in Finland, only 2 identical mutations of this gene were found in patients and carriers. In families with congenital NS, in the process of medical and genetic counseling, pregnant women are necessarily examined for the presence of alpha-fetoprotein in their blood. If found, termination of pregnancy is recommended.

Diagnostics... The birth of a child with congenital HC in a family requires first of all clarification of ethnic roots. It is imperative to exclude secondary NS associated with intrauterine infections. Finnish-type congenital HC should be differentiated from familial HC, which is described in different countries of the world in people of different nationalities (see below). The Finnish type HC is indicated by severe pregnancy, the presence of a very large placenta, detection of microcystosis of the proximal tubules during morphobioptic examination.

Treatment... Despite the fact that in Finland there is an active identification of families where the development of congenital HC of the Finnish type is possible, children with this severe pathology are still being born. Neither symptomatic therapy nor steroids and immunosuppressive drugs cause improvement in patients with congenital HC of the Finnish type.

A high-protein and high-calorie diet is recommended along with the strictest water-electrolyte balanced regimen for up to 10-12 months of a child's life. By this age, it is possible to bring his body weight to 10 kg, to eliminate dystrophy and edema syndrome. After a nephrectomy, a kidney transplant is performed. A ten-year observation of a group of about 40 children convincingly testifies to the good rehabilitation of such patients.

Kidney pathology in childhood is an urgent medical problem that requires urgent diagnosis and timely correct treatment. Nephrotic syndrome in children is a combination of clinical and laboratory symptoms resulting from kidney damage. The most important of them are - severe proteinuria, hypoproteinemia, extensive edema, pathological changes in protein and lipid metabolism. Congenital nephrotic syndrome is already present in children from birth or appears in the first months of life.

Features of nephrotic syndrome in childhood

The incidence of nephrotic syndrome in young patients is low - 14-16 patients per 100,000 children. Up to 3 years, boys get sick more often 2 times.

Nephrotic syndrome is either primary or secondary. Primary forms prevail, they are associated with hereditary kidney disease. Views:

• congenital - diagnosed in the first three months of life;
• infantile - manifests itself during the first year of life;
• idiopathic - begins in toddlers over one year old.

The causes of secondary nephrotic syndrome:

• diabetes;
• infectious diseases such as hepatitis B, toxoplasmosis, rubella, cytomegaly;
• glomerulonephritis.

At the heart of the appearance of nephrotic syndrome, disorders in the functioning of the immune system are distinguished. Antigens stimulate the synthesis of protective antibodies to eliminate foreign pathogens. A triggered immune response supports inflammatory processes in the tissues of the renal epithelium. Podocytes are damaged, cracks appear. Large protein molecules are easily excreted in the urine. Children with congenital nephrotic syndrome inherit a circulating permeability factor from their mothers.

How to recognize a baby's pathology?

A common symptom of the manifestation of the disease is swelling.

Clinical manifestations largely depend on the main diagnosis, which became the cause of the development of the nephrotic symptom complex. But there are leading signs that are present in all patients:

1. The edema is extensive and rapidly spreading. Initially, they are visible only on the face - around the eyes, on the cheeks, forehead and chin. Children have a "nephrotic face". As kidney function deteriorates, swelling of the arms, legs, and lower back occurs. In severe cases, fluid enters the cavity. Varieties:
   • ascites - edematous fluid is contained in the abdominal cavity;
   • hydropericardium - fluid in the pericardium, and compresses the heart;
   • hydrothorax - fluid accumulates in the pleural cavity.
2. Anemia. The main complaints of patients are weakness, frequent dizziness, and difficulty breathing. On examination, attention is drawn to the pronounced pallor of the skin and visible mucous membranes, the poor condition of the nails and hair.
3. Diuresis disorders. Oliguria is a decrease in the daily volume of urine.
4. Disorders in the gastrointestinal tract. Sick children are often worried about nausea and vomiting, abdominal pain, often diarrhea, no appetite.
5. Violations of general well-being.

When conducting laboratory tests, it is found:

• severe proteinuria - the urine contains a lot of protein (more than 2.5 g);
• hypoalbuminemia - the level of blood albumin is reduced (below 40 g / l);
• dysproteinemia - increased globulins;
• hyperlipidemia;
• hypochromic anemia.

What is the threat?

A complication of nephrotic syndrome, the child has an abscess.

The consequences of nephrotic syndrome in children are infectious diseases with complications (sepsis, peritonitis, abscess, pneumonia). Pulmonary edema with severe respiratory failure occurs due to the accumulation of edematous fluid in the pleural cavity. Long-term hormone therapy leads to the appearance of cushingoid syndrome.

Treatment of congenital nephrotic syndrome in children

Effective drugs

Therapy for sick children in the acute period is carried out only in the nephrology department. The main drugs are glucocorticoids. The choice is given to "Prednisolone". The total duration of hormone therapy is 6 months. First, "Prednisolone" is prescribed at a dosage of 2 mg / kg per day. Duration of admission is 2 months. Then gradually, once every 4 weeks, the dose is reduced by 0.5 mg / kg. This prevents the development of withdrawal syndrome.

Supportive therapy that began in the hospital continues at home. Nephrologist's examinations and control tests of urine and blood are obligatory during this period. An alternating method of taking the hormone is carried out every other day, then every 4 days. It helps to gently get away from glucocorticoids and fully do without them.

It is contraindicated to interrupt therapy on your own - a relapse with a severe course may occur. In all cases of exacerbation, treatment is carried out with glucocorticoids and according to the same scheme.

It is necessary to use "Methylprednisolone" in a strict schedule.

If there is no effect from the use of "Prednisolone" for 1.5 months, then "Methylprednisolone" becomes the drug of choice. It is prescribed for pulse therapy. Pulses are large doses, at regular intervals. If there is still no positive dynamics, nephrotic syndrome is classified as steroid-resistant. In this case, a mandatory kidney biopsy is shown to help determine the type of nephritis. Cytostatics are introduced into the therapeutic protocol. Be sure to add selective immunosuppressants. Preference is given to drugs such as Cyclophosphamide and Cyclosporin.

Nephrotic syndrome in children is a serious pathology that can be found in children even in infancy. This ailment is characterized by the presence of a generalized form of edema (spreads throughout the body, and not only in its individual areas), proteinuria, hyperlipidemia. The pathology of the kidneys is considered the basis of this disease. In order to notice the first symptoms of nephrotic syndrome in a baby in time, you need to know as much as possible about this ailment.

Provoking factors

Depending on the reasons provoking nephrotic syndrome, several of its varieties are distinguished, and a different method of treatment is used for each form. There are the following main types:

1. Secondary syndrome. It is a side effect of various systemic diseases in a child. For example, this is lupus erythematosus, renal pathologies, diabetes mellitus, problems in the circulatory system, viral liver damage, and oncological diseases. The prognosis, the course of the disease and the choice of treatment for such a nephrotic syndrome depends on the severity of the disease and the severity of the clinical picture.
2. Congenital and hereditary syndrome. Congenital nephrotic syndrome appears quickly in babies after birth. This pathology is also called a hereditary form of nephritis. In some cases, the diagnosis is determined even during the intrauterine development of the baby. But there are cases when hereditary nephrotic syndrome manifests itself in a child at a later time (for example, at school age). In both cases, the pathology is difficult to treat. Most children with a congenital or hereditary form of the disease develop kidney failure.
3. Idiopathic syndrome (primary). It is diagnosed only if it was not possible to accurately identify the cause of the disease. Parents should be prepared that this form of nephritis is often found in children. Due to the fact that the doctor cannot determine the main provoking factors, it is difficult to choose the optimal treatment. In addition, in the future, the likelihood of various complications, especially with the kidneys, is high.
4. Tubulointerstitial syndrome. In this nephrotic syndrome, the kidneys are affected in such a way that their functioning is reduced. Allocate acute and chronic forms of the disease. The first is most often triggered by taking certain medications and allergies to them. An infectious agent is also often the cause. The chronic form usually develops against the background of other diseases, like the secondary syndrome.

ARVE Error:

Why the baby began to develop nephritis is not always possible to determine. But if it is possible to identify such factors, then this helps in the selection of the optimal therapy in order to speed up the baby's recovery process.

The main symptoms of the syndrome

With nephrotic syndrome, laboratory tests show the following results:

1. The concentration of protein in urine ranges from 2.5 g / m² per day or 50 mg / kg per day.
2. The concentration of protein and albumin in the blood decreases - the indicator is less than 40 g / l.
3. The absorption of protein in the blood is impaired.
4. The concentration of fats and other fractions in the blood increases.
5. Lipoproteins are found in urine.

Signs of the interstitial form of nephrotic syndrome in children, as with other types of the disease, will be noticeable immediately. Please note the following:

1. The swelling increases rapidly. Usually it appears first on the eyelids, and then goes to the stomach, groin, legs. Then ascites (dropsy in the abdomen) develops.
2. The distribution of water in the body depends on how the child's body is positioned. This also affects puffiness. For example, if a child just stood for a while, then puffiness appears on the legs. If he lies on his side, then the body swells on that side.
3. The amount of urine excreted per day gradually decreases. This affects the analysis, as the concentration of protein in the urine increases.
4. At the beginning of the development of pathology, blood pressure may increase. The child becomes irritable, lethargic, a headache and other symptoms corresponding to his condition appear. If the baby is provided with medical assistance, the indicator gradually decreases to normal. If such a painful condition is ignored for a long time, then renal failure develops.
5. Any infections are dangerous for a child with nephrotic syndrome. Streptococcus or pneumococcus may become active, so colds and other infectious diseases may occur as complications. It can be erysipelas, bronchitis, and peritonitis.
6. In a small patient, appetite worsens, weight loss is possible.

If a pathological condition is ignored, but nephrotic syndrome can later turn into chronic renal failure. To prevent this, you need to see a doctor as soon as possible.

Treatment of the disease

With nephrotic syndrome in a child, immunosuppressive therapy is prescribed. Usually, drugs of a non-selective traditional type are used. Suitable drugs from the groups of antimetabolites, glucocorticoids, cytostatics. Of the glucocorticoids, Metypred, Medopred, Prednisolone and its analogues, as well as Solu-Medrol are usually prescribed. Of the cytostatics, Chlorambucil, Cyclophosphamide are used. Less commonly used are Methotrexate and Azathioprine, which belong to the group of antimetabolites. Cytostatics sometimes cause side effects such as nausea, vomiting, leukopenia, dermatitis, hemorrhagic cystitis, hepatopathy, pulmonary fibrosis. In addition, selective immunosuppressants are used, for example, Cyclosporin-A, Tacrolimus and Mycophenolate mofetil.

Depending on the body's response to the use of hormonal agents, nephrotic syndrome is of 2 types - steroid-resistant and steroid-sensitive. If the child has a primary form of nephrotic syndrome, then treatment with Prednisolone is effective. The disease goes into remission. If there is no positive reaction to such drug treatment, then other methods are required.

ARVE Error: id and provider shortcodes attributes are mandatory for old shortcodes. It is recommended to switch to new shortcodes that need only url

For therapy, 3 main modes are used:

1. Continuous oral administration of Prednisolone. The dosage for the child is calculated by the specialist depending on the weight of the small patient. This mode is assigned immediately after the diagnostic procedures.
2. The alternative regimen involves only maintenance therapy. The daily dosage of Prednisolone should be consumed only every other day in order to maintain the therapeutic effect at a sufficient level. In this case, side effects should not appear. Among them are insomnia, euphoria, psychosis, excess weight, edema, myopathy, striae, improved appetite, atrophic phenomena on the skin, hirsutis, increased blood pressure and the development of steroid-type diabetes.
3. Pulse therapy with Methylprednisolone. The substance is injected 1 time in 2 days by drip through a vein.

Conclusion

Nephrotic syndrome in children is a common occurrence, so parents should definitely know about this disease in order to identify it in time and go to the hospital for medical help. This ailment is associated with problems with the functioning of the kidneys. The child develops massive swelling throughout the body, as well as hyperlipidemia and proteinuria. To prevent the development of complications, it is necessary to notice the development of pathology in time and consult a specialist. Only a doctor will be able to choose the optimal treatment.

Congenital nephrotic syndrome- NS, which developed in children from the moment of birth or in the first 3 months of life. In children, it is heterogeneous, there are primary and secondary forms of congenital NS. In most cases, primary forms dominate, which represent a heterogeneous group of diseases with different etiology and prognosis: VNS of the Finnish type, diffuse mesangial sclerosis, isolated or in a complex syndrome, and lipoid nephrosis. Secondary forms of ANS often develop against the background of infectious diseases such as cytomegaly, syphilis, toxoplasmosis, HIV.

A. Primary forms:

Finnish-type congenital NA

Congenital NS of the French type

Other NS (with minimal changes, FSGS, membranous GN)

Syndromic anomalies (Galloway-Mowat syndrome, congenital NS with nervous system abnormalities and other syndromes)

B. Secondary forms:

Against the background of infectious diseases (congenital syphilis, toxoplasmosis, rubella, cytomegaly, malaria)

With SLE in the mother

NS associated with renal vein thrombosis

Finnish-type congenital NA- the most common variant of congenital NA. The disease is more common in Finland - 1: 8200 births, but it can also be registered in other regions of the world in persons of different nationalities, in Russia it is more often registered in the western part of the country. It is a genetic disorder that is inherited in an autosomal recessive manner. In 1994, a gene located on chromosome 19 was isolated. This gene (NPHS1) encodes a protein called nephrin, which is a transmembrane protein of the immunoglobulin superfamily. In the kidneys, this protein is found on the slit membrane between the legs of podocytes. In Finnish families, 4 mutations of this gene are recorded, the most frequently recorded: Fin-major and Fin-minor mutations. Other mutations (46 variants) are recorded in persons of non-Finnish nationality. Girls and boys get sick equally often.

Clinic and diagnostics. Most babies are born prematurely between the 35th and 38th weeks of pregnancy. The placenta mass reaches more than 25% of the newborn's body weight. The ratio of the placenta mass to the child's body mass was increased to 0.43 (with a norm of 0.18). During pregnancy, in women from the 16th to the 20th week of pregnancy, an increased level of α-fetoprotein in the amniotic fluid or in the maternal serum is determined. The disease manifests itself as a complete clinical and laboratory symptom complex of hormone-resistant NS, often with microhematuria. BP is normal. Massive proteinuria, represented in 90% of cases by albumin, begins in the prenatal period. In 25% of cases, massive edema appears from birth, in 90% - in the first week. Hypoalbuminemia often reaches a critical level (below 5 g / l), hyperlipidemia is defined. Renal dysfunction progresses. CRF develops by the age of 4. On ultrasound, the kidneys are enlarged with increased echogenicity of the parenchyma or total hyperechogenicity in the absence of clear corticomedullary differentiation. Morphologically, in the early stages of the disease, a pathognomonic histological picture may be absent, from 3 months microcystic dilatation of tubules, mainly proximal, is noted. The glomeruli themselves may not be changed, or proliferative processes are noted in them. The prognosis is unfavorable.

Congenital NS of the French type- symptom complex of NS, manifested in the first 3 months of life and characterized by morphologically diffuse mesangial sclerosis. The disease has an autosomal recessive inheritance pathway. Girls and boys get sick equally often. Babies are born full-term with a normal birth weight. The placenta mass is normal. Maternal α-fetoprotein levels are normal during pregnancy. NS in 67% is combined with microhematuria and is characterized by resistance and lack of effect from other immunosuppressive therapy. Arterial hypertension is determined in 71% of patients. CRF usually develops by 1.5-2 years.

Treatment: The goal is to bring the child to a suitable age for transplantation, which is the only cure. The protocol proposed by Finnish pediatricians in the early 1990s includes:

1) Compensation of hypoalbuminemia (20% albumin) in combination with furosemide to a serum albumin level of 15-20 g / l.

2) Substitution therapy (vitamin D, thyroxine, vitamins, calcium).

3) Nutrition (enteral through a nasogastric tube 130 kcal / kg, 4 g / kg / day of protein, liquid 100-130 ml / kg / day; 10-14% protein, 40-50% lipids, 40-50% carbohydrates).

4) Prevention and treatment of thrombotic complications (courantil, heparin, low molecular weight heparins).

5) The use of ACE inhibitors (kapoten).

6) Prevention and treatment of infectious complications

This tactic continues until the child reaches a body weight of about 7 kg - the age at which the binephrectomy is performed. In the future, the child is on peritoneal dialysis or hemodialysis until the parameters required for kidney transplantation are achieved, which is carried out after reaching a body weight of 9 kg.

Hereditary jade

Hereditary jade - genetically determined non-immune nephritis-like disease, manifested by hematuria and (or) proteinuria and often combined with hearing pathology and less often vision. The disease is transmitted by an autosomal dominant type linked to the X chromosome (80-85%), an autosomal recessive or autosomal dominant mode of inheritance. Gene mutations lead to disruption of the three-helix structure of collagen (type 4 alpha collagen chain), which causes a change not only in the basement membranes of the kidney, but also in similar structures of the ear and eye. There are 3 variants of hereditary nephritis.

1. Alport syndrome, which is characterized by hereditary nephritis with hematuria, hearing loss and eye damage. The disease is inherited according to the dominant type of inheritance linked to the X chromosome. The course of nephritis is progressive with an outcome in chronic renal failure.

2. Hereditary nephritis without hearing loss, characterizing a progressive course with an outcome in chronic renal failure. The disease is inherited according to the dominant type of inheritance linked to the X chromosome.

3. Familial benign hematuria, which is benign with a favorable prognosis. The disease is inherited in an autosomal dominant or autosomal recessive mode of inheritance. With an autosomal dominant type of inheritance, thrombocytopenia is noted.

Morphological examination determines dysplastic, dystrophic, proliferative changes, focal segmental glomerulosclerosis. The progression of the lesion leads to atrophy and degeneration of the tubules, interstitial fibrosis. Electron microscopy reveals thinning, splitting, and disruption of the basement membrane structure. The clinical picture is diverse in development, manifestations and course. There are 3 stages of nephritis: in the first stage, the child's well-being does not suffer, isolated urinary syndrome is noted, there is no renal dysfunction; the second stage is characterized by a deterioration in well-being, an increase in changes in the urine and renal failure of the tubular type; the third stage - terminal - develops by the age of 20-30, sometimes earlier.

The first signs of kidney damage in Alport syndrome are usually detected between the ages of 3 and 10 years. Usually they are detected by chance, in the form of an isolated urinary syndrome. The most common and first symptom of the disease is hematuria of varying severity. But sometimes proteinuria or, less often, hearing loss is an early sign of the disease. Usually, these signs are detected on average at 6 years of age.

Hematuria with hereditary nephritis can spontaneously appear or disappear. It is very often triggered by an acute respiratory viral infection. Erythrocytes in urine are usually dysmorphic, usually erythrocyte casts are found. Proteinuria may not be present in the first years, it is often minimal and intermittent. Rarely, proteinuria more than 2 g / day and the development of nephrotic syndrome are noted.

Possible hereditary nephritis with thrombocytopenia and leiomyomatosis. Initially, esophageal leiomyoma (a benign tumor emanating from the muscular membrane) with a predominant localization in the thoracic part is detected. Tracheobronchial localization is less common, but it can be fatal due to bronchospasm. Somewhat later, genital leiomyoma appears. Cases of localization of leiomyomas in the clitoris, labia minora and labia majora are described.

In girls, the disease is more often manifested by recurrent hematuria. In boys, the clinical course of the disease is more severe than in girls. Intercurrent diseases, increased physical activity, and insolation contribute to the deterioration of the condition.

Deafness is more common in boys than girls and develops by about age 10. Hearing loss is diagnosed in 74% of boys and 5% of girls. It has a neurogenic origin, expressed in varying degrees, progresses from moderate to full with age. In the early stages, hearing loss occurs at high frequencies, spreading later to lower frequencies, passing from sound-conducting to sound-perceiving hearing loss. At an early stage of the disease, audiometry reveals the immunity of sounds with a frequency of 6-8 kHz, and subsequently of lower frequencies (4.1-2 kHz). The defeat of the VIII pair of cranial nerves or organ of Corti is more often bilateral. Early hearing loss indirectly indicates the severity of the renal process. Histological examination of the inner ear reveals various changes, among which most often - the loss of neurons and hair cells, atrophy of the spiral ligaments, degeneration of the stria vascnlaris.

Eye abnormalities are manifested by changes in visual fields, lens and corneal abnormalities. Alport syndrome is characterized by cataracts, posterior lenticonus, posterior polymorphic corneal dystrophy, pseudo-edema of the papillae, retinal dystrophy, retinal telangiectasia, color perception disorders, coloboma, strabismus, nystagmus, progressive bilateral keratoconus. Nystagmus and myopia are often detected. Ophthalmological examination often reveals decreased visual acuity, anterior lenticonus, spots on the retina, cataracts, and keratoconus in patients.

Microneurological symptoms occur in 90% of patients with hereditary nephritis. A third of patients have symptoms of autonomic dysfunction - fluctuations in blood pressure, emotional lability, headache, hyperhidrosis of the palms and feet. Sometimes symptoms of pyramidal insufficiency (hyperreflexia, etc.), flattening of the nasolabial folds, asymmetry of tendon reflexes are determined. Memory impairments and decreased intelligence are rare.

Hereditary nephritis is characterized by signs of dysembryogenesis. Excretory urograms sometimes reveal pelvic ectasia, double kidney, pathological mobility, incomplete turn of the kidney.

With hereditary nephritis, there is a decrease in the levels of T- and B-populations of lymphocytes, IgA, a tendency to increase the concentrations of IgM and IgG. Reduced phagocytic activity. A decrease in the general resistance of the body predisposes to pyelonephritis, purulent otitis media, and frequent colds.

The functional state of the kidneys is preserved in the stage of latent clinical manifestations or compensation. In the stage of subcompensation, renal dysfunctions of the tubular type prevail with an outcome in total chronic renal failure. With hereditary nephritis in kidney biopsies in children, the interstitium / cortex ratio and the number of sclerosed glomeruli, which are markers of renal scarring, increase with age.

In the early stages of the disease, it is difficult to diagnose the disease, since there are no pathognomonic symptoms. The diagnosis of Alport's syndrome is established on the basis of the detection of nephropathy with hematuria in a child in the presence of a patient with a similar pathology in the family and a combination of kidney damage with deafness in the patient himself or one of the family members. Therefore, in order to make a diagnosis, it is important to draw up the pedigree of the patient's family.

According to Clifford et al. (1993), the diagnostic criterion is the presence of 3 out of 5 signs, one of which relates to the kidneys: 1) hematuria or death from chronic renal failure in a family history; 2) hematuria or nephrotic syndrome in a patient; 3) changes in glomerular basement membranes (with electron microscopy of a kidney biopsy); 4) hearing loss (according to audiogram data); 5) congenital vision pathology.

A kidney biopsy is used to confirm the diagnosis. Alport's syndrome is characterized by uneven contours of the glomerular basement membrane, stratification or reticularity of its dense plate.

There are no effective methods of pathogenetic therapy for hereditary nephritis. Treatment involves the organization of a sparing regimen. Limit physical activity, do not carry out preventive vaccinations. The diet is high in calories, balanced, taking into account the functional state of the kidneys. In the absence of signs of impaired renal function, a diet with a sufficient content of proteins, fats and carbohydrates is prescribed. But a diet with restriction of proteins, lipids, calcium and phosphorus delays the timing of the development of chronic renal failure. It was reported about the successful use of angiotensin-converting enzyme inhibitors in the complex treatment of hereditary nephritis, which reduce the severity of proteinuria and slow down the progression of the disease. Metabolic activators are used, such as pyridoxine (2-3 mg / kg / day in 3 doses for 2-4 weeks), cocarboxylase (50 mg intramuscularly every day; 10-15 injections), ATP (1 ml intramuscularly through 1 day; 10-15 injections), vitamin A (1000 units / year of life per day in 1 dose; 10-14 days), vitamin E (1 mg / kg / day for 1 dose; 10-14 days). These drugs are prescribed in courses 2-3 times a year. Herbal medicine is also effective. As immunostimulants, levamisole (decaris) is prescribed at 2 mg / kg / day 2-3 times every 1 week with a 4-day break. With the development of chronic renal failure, hemodialysis and kidney transplantation are performed. The success of dialysis and transplantation depends on the selection of the graft and the presence of antibodies to GBM. Antibacterial, immunosuppressive and steroid therapy are indicated in the pre- and post-transplant periods. Vision correction is performed using glasses or contact lenses. The positive experience of lens implantation and surgical treatment of the anterior lenticonus is described.

Patients with hereditary nephritis are on dispensary records throughout their lives. Prognostically unfavorable criteria for the course of hereditary nephritis are: belonging to the male sex; early development of chronic renal failure in family members; proteinuria (protein level more than 1 g / day); thickening of the glomerular basement membranes (by electron microscopy); neuritis of the auditory nerve and a deletion in the COL4A5 gene.

The main kidney diseases in newborns can be divided into two main groups (systematization of the authors):

• congenital and hereditary nephropathy;
• acquired kidney damage.

In the 1st group, several rather large subgroups can be distinguished:

1. anatomical defects of the kidneys (changes in the number, shape, size, etc.);
2. histological abnormalities of the kidneys ( cystic disease, including congenital nephrotic syndrome of the Finnish type, dysplasia, reflux nephropathy);
3. hereditary nephritis;
4. tubulopathy;
5. tumors;
6. dysmetabolic nephropathy.

The second group of lesions of the urinary system in newborns is quite small. The following groups of diseases are most often recorded:

1. microbial inflammatory diseases (primary and secondary pyelonephritis, urinary tract infections);
2. tubulointerstitial nephritis;
3. secondary kidney damage (hemolytic uremic syndrome, renal candidiasis, renal vascular thrombosis, burn and drug diseases);
4. kidney and urinary tract injuries.

Of all the variety of renal diseases in the neonatal period, the most frequently diagnosed congenital nephrotic syndrome (primary or secondary), urinary tract infections, interstitial nephritis, renal vein thrombosis.

Under the term "congenital nephrotic syndrome" (ANS) understand the corresponding disease diagnosed in the first 3 months of life. The most complete classification of nephrotic syndrome in the world literature, including congenital, is presented in the works of N.D. Savenkova, A.V. Papayana (1996, 1997, 1999).

There are primary and secondary congenital nephrotic syndrome.

In a group primary nephrotic syndrome more often the so-called Finnish type (microcystic disease) and the French type (diffuse mesangeal sclerosis) are recorded.

Secondary nephrotic syndrome may be associated with hypothyroidism, renal vein thrombosis, toxoplasmosis, tuberculosis, some genetic syndromes (Lowe), etc.

Finnish type ANS congenital nephrotic syndrome(congenital microcystic nephropathy, infantile nephrosis, microcystic kidney of the Finnish type) is the most common cause of nephrotic syndrome in newborns. The disease is transmitted in an autosomal recessive manner. The incidence of the disease is 10–12 cases per 100,000 births. Cases of ANS have been described in monozygotic twins and children from related marriages. Boys and girls are equally ill.

Pathogenesis.

The pathogenesis of the disease is reduced to protein disorders nephrin and podocin... Morphologically revealed microcystosis of the proximal tubules in the cortico-medullary zone, signs of glomerular immaturity, focal-proliferative changes in mature glomeruli.

The clinical picture.

Clinically congenital nephrosis of the Finnish type is manifested by the clinical and laboratory symptom complex of NS (severe edema, proteinuria, sometimes hematuria, severe hypoalbuminemia - less than 10 g / l, hypogammaglobulinemia, hypercholesterolemia, may be glucosuria, generalized aminoaciduria) in the first days of a child's life (less often in the first 4–8 weeks) or from birth.

Thyroxine levels are low and TSH levels are normal. P with ultrasound is symmetrically enlarged. In such children, the stigma of dysembryogenesis is expressed. In most cases, with this disease during pregnancy, there are gestosis, threats of termination and premature birth, intrauterine growth retardation of the fetus. The placenta mass is dramatically increased and reaches 40-50% of the newborn's body weight.

Treatment.

There is no effective treatment for VNS of the Finnish type. Intravenous infusions of albumin (3-4 g / kg) followed by (0.5 mg / kg), use of vitamin D2, calcium, prevention of septic complications.

Patients with the Finnish type of nephrotic syndrome do not respond to corticosteroids and cytostatics, but positive dynamics of the condition can be achieved when using indomethacin and ACE inhibitors.

The prognosis of the disease is unfavorable. Children mainly die in the 1st year of life as a result of infections, renal failure, cerebral edema, cachexia.

Cases of successful symptomatic therapy of VNS of the Finnish type are described, when children reach the age-related ability of peritoneal dialysis and kidney transplantation. However, one third of patients after kidney transplantation develop post-transplant nephrosis.

With other types of ANS in the kidneys, diffuse mesangeal sclerosis, minimal changes, and focal segmental glomerulosclerosis can be morphologically detected. Clinically, these variants can be detected at a later age, are easier, and sometimes spontaneous remissions are observed. The final morphological diagnosis of congenital nephrotic syndrome can be established only after nephrobiopsy.

Urinary tract infection.

Urinary tract infection (UTI)- an infectious and inflammatory process in the urinary system without indicating the level of damage. The term "infection of the urinary system" is legitimate when there are signs of microbial damage to the urinary tract, but it is not possible at the moment to determine the level of its localization. This diagnosis is temporary and can be used from the moment of detection of pathology to clarification of the topical level and determination of a specific nosological form. IMS is a collective concept that includes urethritis, etc.

Along with the concept of IC, another term is also used - “ urinary tract infection "(UTI)... This is an inflammatory process in the urinary tract (pelvis, ureters, urethra) without damage to the renal parenchyma. The exact localization of the infectious process can be determined after conducting clinical laboratory and instrumental studies and differential diagnosis.

IMS and UTI are diagnosed in 0.7-1% of term infants and in 4-25% of premature and post-term infants, in boys 5 times more often than in girls. In the neonatal period, urinary tract infections often manifest clinically in the form of secondary pyelonephritis (with urinary tract obstruction, sepsis).

Etiology.

The most common pathogens of IMS are gram-negative microorganisms: Esherichia coli, Klebsiella pneumonia, Enterobacter cloaca, Pseudomonas aureginosa, less often staphylococci, group B streptococci are found.

Among the risk factors for the development of IMS in newborns, the pathological course, hereditary burden of renal pathology, congenital anomalies of the urinary system, vesicoureteral reflux, etc. are considered.

The clinical picture.

The most common nasological form of UTI in the neonatal period is pyelonephritis. - microbial-inflammatory kidney disease with a predominant localization of the pathological process in the tubulointerstitial tissue and lesions of the calyx-pelvic system.

In the clinical picture of pyelonephritis, the following syndromes are distinguished. Intoxication syndrome is characteristic of the hematogenous route of infection. they suck sluggishly, up to a complete refusal to feed, regurgitate, vomiting, loose stools appear, which leads to loss of body weight and the development of electrolyte imbalance. Hypo- or hyperthermia, increased excitability are noted. Hepatomegaly, jaundice, hemolytic anemia are often observed.

In the clinical picture of urinogenic pyelonephritis, urodynamic disorders and local symptoms are in the first place. Half of the children have anxiety when urinating, others - before urinating, crying, facial redness, which is considered equivalent to the syndrome of dysuric disorders (A.V. Papayan, N.D. Savenkova, 1997).

Urinary syndrome is characterized by diagnostically significant bacteriuria (100,000 microbial bodies in 1 ml), neutrophilic leukocyturia (more than 10-15 in the field of view; more than 2000 in 1 ml according to Nechiporenko), proteinuria up to 1 g / l, and inconsistent microhematuria.

From the side, with a pronounced bacterial process, anemia, leukocytosis, neutrophilia with a shift to the left, accelerated ESR can be observed.

If there are signs of secondary pyelonephritis, there are grounds for conducting excretory urography. Secondary pyelonephritis is understood as a microbial-inflammatory process in the interstitium and the renal pyelocaliceal system, which occurs against the background of congenital anomalies, malformations of the urinary system, hereditary or acquired diseases or functional disorders of urodynamics. Such obstructive pyelonephritis.

In the case of a microbial-inflammatory process in the renal tissue against the background of dysmetabolic disorders, congenital and acquired immunodeficiency states, endocrine dysfunctions, secondary pyelonephritis is non-obstructive.

Treatment.

In the acute period, infusion detoxification therapy, correction of homeostasis disorders is carried out in the hospital. The main etiotropic therapy is antibacterial, taking into account the sensitivity of the pathogen and minimal toxicity to the newborn.

The most suitable for this purpose are β-lactamase penicillins thanks to the addition of clavulanic acid to their formula (amoxiclav, augmentin, clavocin, ticarcillin) or sulbactam ( ampicillin + sulbactam, piperacillin + tazobactam - tazocin, unazine), cephalosporins of the 2nd and 3rd generations, aminoglycosides ( netromycin, amikin, amikacin, tobramycin, sisomycin), macrolides ( erythromycin ascorbate, sisomycin). The duration of the course of antibiotic therapy in the treatment of pyelonephritis is 10-14 days.

In the absence of normalization of urine and blood tests, it is advisable to continue antibiotic therapy, taking into account the sensitivity of the isolated flora.

Nalidixic acid and nitrofurans in newborns are used with caution because of the risk of acidosis, increased cerebrospinal fluid pressure.

As the inflammatory process subsides, 5-7 days after the start of antibacterial therapy, antioxidant agents are prescribed for a course of 2-3 weeks ( vitamin E - 10 mg / kg day, vitamin A - 1000 IU / kg day). In the case of severe pyelonephritis, prolonged or recurrent course and mixed infection, nonspecific ( echinacea, interferonogens - cycloferon) and specific (lysozyme, interferon - viferon) immunocorrection. The appointment of immunomodulators is indicated when the infectious and inflammatory process subsides.

If necessary, subsequent anti-relapse therapy is carried out for 4-6 months with antibacterial drugs in a 1 / 3-1 / 4 age dose.

Interstitial nephritis.

Interstitial nephritis (IN, TIN)- nonspecific abacterial inflammation of tubulointerstitial tissue of allergic, toxic, infectious genesis with involvement of tubules, blood and lymphatic vessels of the renal stroma in the pathological process.

In newborns, this disease is more often an acute, transient condition caused by damage to the tubulointerstitium due to hypoxia, impaired renal blood flow and increased vascular permeability with the development of interstitial edema.

The clinical picture.

The clinical picture of IN is nonspecific. It is dominated by manifestations of the underlying disease that led to kidney damage. The morphological substrate of interstitial nephritis, regardless of its cause, is interstitial edema, circulatory disorders, lymphohistiocytic infiltration.

In infants, an increase in body temperature, weakness, and a decrease in urine output may be observed.

Urinary syndrome is characterized by proteinuria in the range of 0.033–0.99 g / l, microhematuria (10–30 erythrocytes per field of view), mononuclear leukocyturia (15–30 per field of view), and decreased urine density. The excretory and secretory functions of the tubules decrease: the osmotic density of urine is in the range of 50-100 mosm / l, the titration acidity and excretion of ammonium decrease, and the excretion of sodium and potassium in the urine often increases. The development of latent edema is possible, which is clinically manifested by excessive weight gain. In the most severe cases, acute renal failure develops.

Diagnostics.

In the general analysis of blood, there is a slight leukocytosis with a moderate shift to the left, eosinophilia, accelerated ESR. In biochemical analysis - an increased content of α2-globulin, β2-microglobulin, lysozyme, creatinine and urea.

When increased in size (especially in thickness).

The absolute confirmation of the diagnosis of IN is the results of morphological examination of nephrobiopsy (nephrobiopsy is practically not performed in the neonatal period).

In the literature, there are isolated reports on the development of acute renal failure in the first months of life. In the beginning, symptoms of tubular insufficiency due to IN dominate. By the end of the first - second year of life, these patients develop chronic renal failure in combination with portal fibrosis of the spleen. The basis of the disease has not been established (ND Papayan, AV Savelyeva, 1997).

Treatment.

Treatment of ID is a very difficult task that requires a differentiated approach depending on its cause.

With the development of acute renal failure, urgent measures are required, including the restoration of the BCC, correction of water-electrolyte disturbances, acidosis, etc.

When treating IN of an infectious nature, etiotropic therapy is carried out, medicinal IN is desensitizing (the damaging drug is immediately canceled). In severe cases of toxic-allergic TIN, corticosteroids are prescribed in a short course in small doses (0.5–1 mg / kg · day).

The question of the appointment of diuretics is decided individually, taking into account the state of kidney function.

Shows the use of vitamins A and E, pyridoxal phosphate. For the purpose of immunocorrection, it is possible to prescribe lysozyme, which improves the phagocytic functions of neutrophils.

Tubulopathy.

Tubulopathy- diseases combined by the presence of a violation of the membrane transport of various substances in the kidney tubules. Primary tubulopathies are diseases in which a violation of the transport of substances occurs mainly in the renal tubules. Secondary tubulopathies are diseases in which the violation of the transport of substances is of a diffuse nature and is observed not only in the kidneys, but also in other organs.

The clinical picture.

Despite the qualitative and quantitative variety of disorders in the transport of various substances in the kidney, the clinical picture of tubular disorders consists of several main clinical and laboratory syndromes (A.V. Papayan, I.S. Styazhkina, 2002):

• polyuria;
• electrolyte disorders;
• violations of the acid-base state of the blood;
• rickets-like syndrome (renal osteopathy);
• nephrolithiasis.

It should be noted that very few tubular disorders appear in the first months of life. Most of the congenital tubulopathy clinically manifests itself from 2-3 months of life or in the 2nd half of life, sometimes in the 2nd year, when tubular disorders lead to rickets-like changes in the bones of the skeleton, retardation of psychomotor development.

B.S. Kaplan (1998) cites the following data on tubular dysfunction, the onset of which is manifested in the neonatal period: Debre de Toni-Fanconi renal syndrome; renal tubular acidosis: distal type I (Lightwood-Butler-Albright syndrome), proximal type II; pseudohypoaldosteronism; nephrogenic diabetes insipidus linked to the X chromosome.

Renal syndrome Debrede Toni - Fanconi.

Debrede Toni - Fanconi renal syndrome(glucose-phosphate-amine diabetes) are inherited in an autosomal dominant manner. Some authors point to the possibility of an autosomal recessive type of inheritance. This syndrome is manifested by reduced reabsorption of water, phosphates, sodium, potassium, bicarbonates, glucose, amino acids and other organic acids in the proximal tubules. The first signs of the disease are: lethargy, weakness, anorexia, vomiting, low-grade fever, physical retardation in combination with rickets-like changes in the skeleton.

Diagnostics.

The blood test reveals hypophosphatemia, hypokalemia, acidosis, the activity of alkaline phosphatase increases. In the analysis of urine - hyperaminoaciduria (alanine, arginine, etc.), phosphaturia, glucosuria, natriuria, potassiumuria.

Treatment.

Treatment includes the appointment of an alkalizing drink (2 g of citric acid, 3 g of sodium citrate, 3.3 g of potassium citrate per 100 ml of water; 1 ml of solution contains 1 mmol of sodium and potassium), 45-60 ml per day. In order to prevent stone formation, it is necessary to take Magurlite or Blemaren 0.5 g 3 times a day after feeding.

Type I distal tubular acidosis.

Type I distal tubular acidosis(Lightwood-Butler-Albright syndrome) is inherited in an autosomal dominant manner. The syndrome is caused by a defect in the acidogenetic functions of the distal tubules and is accompanied by impaired activity of secretion and excretion of H +, inability of the distal tubules to maintain a pH gradient, loss of potassium and sodium in the urine, and aldosterone deficiency. Initially, the syndrome is manifested by a delay in weight gain, anorexia, sometimes vomiting, and constipation.

Subsequently, growth retardation, rickets-like changes in the skeletal system, crises of dehydration and polyuria, nephrocalcinosis and urolithiasis with concomitant interstitial nephritis or pyelonephritis are noted.

Diagnostics.

In the analysis of blood - hypokalemia, hyponatremia, metabolic acidosis. In the analysis of urine - alkaline reaction, hyperkaliuria, hypercalciuria (more than 4 mg / kg

Proximal tubular acidosis (type II).

Proximal tubular acidosis (type II) is based on a defect in bicarbonate reabsorption, which leads to the development of decompensated metabolic acidosis. With this syndrome, children do not have violations of the concentration function of the kidneys, urolithiasis and nephro-calcification. The syndrome can be isolated or combined with other proximal disorders (Debre de Toni - Fanconi syndrome, etc.). Mostly boys are ill.

The clinical picture.

The primary forms are characterized by a delay in physical development in combination with rickets-like changes, metabolic acidosis to acidemic coma, vomiting, fever, polyuria, and nephrocalcinosis.

Diagnostics.

In the analysis of blood - hypochloremia, metabolic acidosis. In urine - acidic reaction, high excretion of potassium, intact excretion of titratable acids and ammonium, decrease in concentration capacity is less pronounced.

Kidney disease in newborns - Treatment.

Therapeutic measures for renal tubular acidosis are aimed at limiting the intake of animal proteins, increasing the amount of fluid consumed, and prescribing an alkalizing drink. With severe acidosis and dehydration, intravenous administration of sodium bicarbonate solution is indicated at the rate of V = BE of the patient · 0.5 · Body weight.

In the first 6 hours, approximately 1/3 of sodium bicarbonate is introduced. During the period of subsidence and remission of the disease, the amount of sodium bicarbonate per day in distal renal acidosis is 1–3 meq / kg in 4 doses, in the proximal - 5–15 meq / kg in 4–6 prems.

Pseudohypoaldosteronism.

Pseudohypoaldosteronism (renal saline diabetes) inherited in an autosomal dominant manner. For newborns, type I is characteristic - primary (renal); Type II - secondary (multiple organ). It is characterized by a low sensitivity of the tubular apparatus to aldosterone, which leads to low reabsorption of sodium by the renal tubules. Clinically, from the first days of life, it manifests itself as polyuria, anorexia, adynamia, arterial hypotension. Due to the large loss of water and sodium, dehydration develops with high hyponatremia and natriuria, hyperkalemia, and metabolic acidosis. In the future, there is a delay in body weight, growth and ossification of the bones of the skeleton, a lag in mental development. The sodium level in the blood is less than 130 mmol / l, acidosis. The concentration of aldosterone in the urine is sharply increased - up to 60-80 mcg (at a rate of 2.5 mcg).

Treatment.

Sodium chloride replacement therapy is carried out in the amount of 3–6 g / day.

Nephrogenic diabetes insipidus.

X-linked nephrogenic diabetes insipidus is inherited recessively.

Mostly boys are ill. The disease is associated with the insensitivity of the renal tubules to antidiuretic hormone and the excretion of large amounts of urine with a low relative density, which leads to the development of severe dehydration and electrolyte disturbances (hypernatremia, hyperchloremia). Typically, the disease manifests at birth with polyuria, polydipsia, repeated periods of hypernatremic dehydration, vomiting, constipation, growth retardation, and malnutrition. With severe dehydration, hyperthermia ("salt fever") and convulsions may develop. In the case of significant polyuria, megatestis, megaureter, hydronephrosis may develop.

Diagnostics.

For the differential diagnosis of polyuria caused by diabetes insipidus, a test is performed with desmopressin (10 μg is administered intranasally), which causes a long-term and pronounced antidiuretic effect.

Urine is collected at 2-hour intervals. Its osmolarity is assessed. With an osmolarity below 200 mosm / kg, it can be stated that a newborn has a renal form of diabetes insipidus. A patient with renal diabetes insipidus has normal blood levels of antidiuretic hormone. In the biochemical analysis of blood against the background of clinical dehydration, hypernatremia, hyperchloremia, and possibly an increase in creatinine levels are noted. The relative density of urine does not exceed 1000-1003.

Treatment.

The main component of treatment for renal diabetes insipidus is to provide the child with sufficient fluids. Drug therapy includes three main drugs: hydrochlorothiazide (thiazide diuretics) - 2 mg / kg · day, amiloride (potassium-sparing diuretics) - 2–5 mg · day (2.5–5 mg / m2 · day) and nonsteroidal anti-inflammatory drugs - indomethacin - 2 mg / kg · day.

Effectively combined use of the above drugs. In newborns and children under 6 years of age, the most effective is the use of a combination of hydrochlorothiazide with indomethacin (prescribed every other day).

Butler's Syndrome.

Butler's syndrome is an autosomal recessive disease in which three different hereditary defects in proteins are detected, which is accompanied by the phenomena of hypokalemia, hypochloremic metabolic alkalosis, extremely high blood levels of aldosterone and renin while maintaining normal blood pressure, increased urinary excretion of chlorides, potassium, prostaglandin E2, low platelet aggregation activity.

The pathogenesis of the disease remains unclear today. It is believed that the disease is associated with a violation of chloride reabsorption. Clinically, from birth, poor appetite, vomiting, muscle hypotension, constipation, polyuria (diuresis can reach 12-50 ml / kg · h), polydipsia, hypokalemic convulsions, paresthesia are noted. In the future, children lag behind in physical development. With this syndrome, a clinical picture of nephrocalcinosis may appear during the neonatal period.

Treatment.

Aimed at correcting hypokalemia with the introduction of potassium chloride - 1-3 meq / kg or more. The amount of potassium administered depends on the potassium excreted in the urine. To date, the best in the treatment of the disease is the use of inhibitors of prostaglandin synthesis - indomethacin at a dose of 2 mg / kg · day.

Renal vein thrombosis.

Renal vein thrombosis (TPV) develops mainly in premature infants within 1 month of life due to severe perinatal hypoxia, dehydration, shock, sepsis, "blue" heart defects.

Predisposing factors are obstructed childbirth, diabetes mellitus in the mother, pathological decrease in the body weight of the newborn.

The clinical picture.

The manifestations of renal vein thrombosis are not typical. Against the background of a state of shock with repeated vomiting, flatulence, palpation reveals an increase in one or two kidneys, arterial hypertension in the first day of life is not typical.

Urinary syndrome is characterized by albuminuria, gross hematuria. With bilateral TPV, ARF develops rapidly.

Diagnostics.

On the part of the blood, the most constant signs are anemia, thrombocytopenia, and leukocytosis.

Hypercoagulability is noted, the ethanol test is sharply positive. The level of plasma fibrinogen, factor V plasminogen decreases with an increased content of fibrin degradation products.

The most informative modern methods for diagnosing TPV are ultrasound using Doppler studies and computed tomography, NMR. Isotope renography and renal venography have retained their diagnostic value. Excretory urography (not recommended in the initial stages of TPV and due to the great technical difficulties in premature infants) reveals a "dumb" kidney in a unilateral process.

The most severe complication of TPV is renal infarction. Typical signs of the latter: oliguria, rapidly alternating with polyuria, gross hematuria, decreased urine osmolarity. Echoscopically, hyperechoic or hypoechoic areas are detected, which may have a heterogeneous tumor-like structure. Doppler studies confirm a decrease or absence of blood flow.

Treatment.

In treatment, antiplatelet agents, anticoagulants are used (heparin at a starting dose of 50 U / kg every 6 hours under the control of coagulation according to Lee-White), fibrinolytics (fibrinolysin, urokinase, streptase) - 10 ml / kg drip for 1 hour together with heparin.

Physiotherapy methods are used- electrophoresis of heparin, aminophylline, nicotinic acid on the kidney area.

The development of ARF is an indication for the use of dialysis therapy (peritoneal dialysis or hemodialysis).

Renal artery thrombosis.

Renal artery thrombosis (RTA) is a rare disease in newborns that occurs against the background of dehydration, maternal diabetes mellitus, with embolism through a patent ductus arteriosus, or as a complication of renal artery catheterization.

The lesion may be asymptomatic or, in severe cases, manifest as a clinical picture of acute renal failure.

The most formidable complication of HAT is kidney infarction.

Treatment.

Reduced to the use of thrombolytics, correction of hypertension and homeostasis. If necessary, dialysis is used in severe cases.

Arterial hypertension is diagnosed with an increase in blood pressure of more than 90/60 mm Hg. Art. in full-term and more than 80/45 mm Hg. Art. in premature newborns. In newborns born to mothers suffering from essential hypertension, blood pressure at birth will be slightly higher. Arterial hypertension in newborns is rare, but in children in intensive care, its frequency ranges from 1 to 2.5%. In 1/3 of newborns, hypertension may be asymptomatic.

Arterial hypertension in the neonatal period is often caused by a combination of high cardiac output, increased blood viscosity, high peripheral vascular resistance, increased activity of the sympathetic nervous system, baroreflex reactions, and an imbalance of vasoconstrictors and vasodilators. The development of arterial hypertension in newborns is observed with the following renal pathology: polycystic kidney disease of the infant type, renal failure, severe obstructive uropathy, as well as thrombosis of the renal arteries or their branches and coarctation of the aorta.

Treatment.

The following drugs can be used to treat neonatal hypertension: diuretics ( furosemide- 1-2 mg / kg every 12-24 hours, veroshpiron, hypothiazide- 2–5 mg / kg · day); vasodilators (hydralisin, apressin- 0.2-2 mg / kg intravenously or by mouth every 6-12 hours, diazoxide- 1-3 mg / kg intravenously, nitroprusside- 0.2–10 μg / kg · min); adrenergic blockers (obzidan, anaprilin- 0.5–2 mg / kg · day orally, labetolol - 0.5–1.0 mg / kg · h intravenously); angiotensin-converting factor inhibitors (captopril- 0.01–0.5 mg / kg by mouth every 8–12 hours, enap - 5–15 μg / kg intravenously every 8–12 hours; 0.1 mg orally 1 time per day); calcium channel blockers (nifedipine- 0.25-0.5 mg / kg every 8-12 hours), central action (methyldopa- 2.5 mg / kg every 8 hours, a single dose can be increased to 15 mg / kg).