Nephrotic syndrome. Causes, symptoms, diagnosis and treatment of pathology. Secondary and congenital nephrotic syndromes in children. Diagnosis Congenital nephrotic syndrome in newborns

Nephrotic syndrome in children is a serious pathology that can be detected in children even in infancy. This disease is characterized by the presence of a generalized form of swelling (distributed throughout the body, and not just in its individual areas), proteinuria, and hyperlipidemia. The basis of this disease is considered to be kidney pathology. 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 disease.

Provoking factors

Depending on the causes that provoke nephrotic syndrome, several of its varieties are distinguished, and for each form a different treatment method is used. The following main types are distinguished:

1. Secondary syndrome. It is a side effect of various systemic diseases in a child. For example, this is lupus erythematosus, kidney pathologies, diabetes mellitus, problems in the circulatory system, viral liver damage, and cancer. The prognosis, course of the disease and the choice of treatment method for such nephrotic syndrome depend on the severity of the disease and the severity of the clinical picture.
2. Congenital and hereditary syndrome. Congenital nephrotic syndrome manifests itself quickly in babies after they are born. This pathology is also called a hereditary form of nephritis. In some cases, the diagnosis is determined during the fetal 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 is 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 there is a high probability of various complications, especially with the kidneys.
4. Tubulointerstitial syndrome. In this nephrotic syndrome, the kidneys are affected in such a way that their functioning is reduced. There are 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, as does the secondary syndrome.

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It is not always possible to determine why a baby began to develop nephritis. But if it is possible to identify such factors, then this helps in selecting the optimal therapy to speed up the baby’s recovery process.

Main symptoms of the syndrome

In nephrotic syndrome, laboratory tests show the following results:

1. The protein concentration 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 - less than 40 g/l.
3. The digestibility of protein in the blood is impaired.
4. The concentration of fats and other fractions in the blood increases.
5. Lipoproteins were found in the 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. Swelling increases rapidly. Usually it appears first on the eyelids, and then moves to the stomach, groin, and legs. Then ascites (water in the abdomen) develops.
2. The distribution of water in the body depends on how the child’s body is positioned. This also affects swelling. For example, if a child just stands for a while, swelling appears on the legs. If he lies on his side, then the body swells on this side.
3. The amount of urine excreted per day gradually decreases. This affects the tests, 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, has a headache and other symptoms corresponding to his condition. If the baby receives medical care, the indicator gradually decreases to normal. If such a painful condition is ignored for a long time, then kidney failure develops.
5. Any infection is dangerous for a child with nephrotic syndrome. Streptococcus or pneumococcus may be activated, so colds and other infectious diseases may arise as complications. It could be erysipelas, bronchitis, or peritonitis.
6. The little patient's appetite worsens and weight loss is possible.

If the pathological condition is ignored, nephrotic syndrome may subsequently develop into chronic renal failure. To prevent this, you need to see a doctor as soon as possible.

Treatment of the disease

If a child has nephrotic syndrome, immunosuppressive therapy is prescribed. Usually, drugs of a non-selective traditional type are used. Medicines from the groups of antimetabolites, glucocorticoids, and cytostatics are suitable. Of the glucocorticoids, Metipred, Medopred, Prednisolone and its analogues, as well as Solu-Medrol are usually prescribed. Cytostatic agents include Chlorambucil and Cyclophosphamide. 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, and pulmonary fibrosis. In addition, selective immunosuppressants are used, such as Cyclosporin-A, Tacrolimus and Mycophenolate mofetil.

Depending on the body's response to the use of hormonal drugs, nephrotic syndrome is of 2 types - steroid-resistant and steroid-sensitive. If a 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 must be used.

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Three main modes are used for therapy:

1. Constant intake of Prednisolone orally. The dosage for a child is calculated by a specialist depending on the weight of the small patient. This regimen is prescribed immediately after diagnostic procedures.
2. An alternative regimen involves only maintenance therapy. The daily dosage of Prednisolone must be taken only every other day in order for the therapeutic effect to be maintained at a sufficient level. There should be no side effects. Among them are insomnia, a state of euphoria, psychosis, excess weight, swelling, myopathy, stretch marks, improved appetite, atrophic phenomena on the skin, hirsutism, increased blood pressure and the development of steroid-type diabetes.
3. Pulse therapy using Methylprednisolone. The substance is administered dropwise through a vein once every 2 days.

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 disease is associated with problems with kidney function. 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 can choose the optimal treatment.

Nephrotic syndrome in children is a collective concept and consists of a whole complex of symptoms, as well as laboratory parameters, and is clinically characterized by extensive swelling of both subcutaneous fatty tissue and accumulation of fluid in the cavities of the body.

It should be noted that this process is characterized by the following laboratory indicators:

• protein in urine from 2.5 g/m2/day or 50 mg/kg/day;
• decrease in the amount of protein and albumin in the blood less than 40 g/l;
• impaired absorption of protein in the blood;
• increased content of fats of various fractions in the blood;
• the presence of lipoproteins in the urine.

Nephrotic syndrome in children in most cases occurs in the following groups: newborns, infants and children under 3 years of age.

Clinically divided into types:

1. Idiopathic (primary) nephrotic syndrome. It is the most common and is caused by an unknown cause (disease).
2. Congenital nephrotic syndrome of the Finnish type develops in children under 3 years of age and can be diagnosed in utero. It received this name due to its initial research by Finnish scientists, where the incidence of pathology is the highest in the world.
3. Secondary nephrotic syndrome. Occurs as a complication of diseases such as:

But it is also important to distinguish 2 main groups of nephrotic syndrome:

• The first includes children under 1 year of age, newborns, infants and older children (5–15 years old) who have healthy kidneys or minimal deviations from the norm, confirmed by examining urine under a microscope.
• The second group includes children with obvious kidney problems.

Signs of illness

This pathological condition has early and late manifestations.

Early symptoms include the following:

• asthenic syndrome (lethargy, lack of appetite, muscle atrophy, general weakness);
• swelling of subcutaneous fatty tissue in the eyelids, lower and upper extremities;
• abdominal pain, as well as its enlargement;
• foamy urine;
• pleurisy (accumulation of fluid in the pleural cavity surrounding the lungs) and, due to its occurrence, severe shortness of breath;
• swelling of the joints and scrotum in boys;
• movement of subcutaneous edema from top to bottom, which is manifested by morning swelling of the eyelids, and in the evening, swelling in the ankle joint;
• a gradual decrease in the normal level of blood pressure, up to the occurrence of collapse and shock.

Late manifestations of nephrotic syndrome include the following symptoms:

• underdevelopment of the external genital organs (hypospadias) due to a lack of nutrients;
• severe nutritional deficiency and resulting retardation in growth and development;
• fragility and dullness of skin appendages: nails and hair;
• cryptorchidism (non-descent of the testicle into the scrotum in boys);
• the occurrence of aseptic (sterile) and then septic peritonitis, due to the accumulation of fluid in the abdominal cavity (ascites);
• various thrombosis of intra-abdominal vessels;
• disorders of the brain and cardiovascular system.

Complications

All complications of nephrotic syndrome in children are associated with the loss of a significant amount of proteins. The loss of immunoglobulins leads to a decrease in the body's reactivity to infection and, as a result, colds often occur, complicated by pathologies of the kidneys, liver and heart. A decrease in the amount of iron transport protein causes iron deficiency anemia.

The loss of high- and medium-density lipoproteins leads to disruption of cholesterol metabolism and subsequently contributes to the development of early atherosclerosis.

Cases have been recorded where, during autopsies of children 7–9 years old, atherosclerosis of the aorta and coronary vessels was discovered. At the same time, due to a decrease in the level of vitamin D in the blood, various changes in the skeletal system may occur.

Loss of procoagulant proteins leads to increased bleeding.

Children suffering from nephrotic syndrome often develop thyroid diseases due to loss of the protein thyroglobulin, which entails additional problems with hormone metabolism.

Diagnostic methods

Identifying a pathological condition is not a difficult task. Even congenital nephrotic syndrome can be diagnosed in utero by examining the amniotic fluid, various identifying signs determined by ultrasound (the size of the fetus, its limbs, head, pelvis, etc.).

Laboratory syndrome is diagnosed by examining urine and blood, both by the general method and by the biochemical method. Blood tests measure sodium and potassium levels, as well as various fractions of lipids and proteins.

Therapeutic measures

Today, there is a basic treatment regimen for nephrotic syndrome, which includes immunosuppressive therapy. For these purposes, selective and non-selective immunosuppressants are used. The latter include glucocorticoids (hormones of the adrenal cortex), cytostatics and antimetabolic drugs, and selective ones - Cyclosporin A, Tacrolimus, Mycophenolate mofetil.

Nephrotic syndrome is divided into 2 types, depending on sensitivity to hormones: hormonal dependent and, accordingly, independent. In the primary syndrome, in 90% of cases the body responds well to therapy with glucocorticoids (prednisolone), due to minimal disturbances of the glomeruli. If resistance to such therapy occurs, then the syndrome is secondary.

For children, glucocorticoids are prescribed for all cases of new-onset nephrotic syndrome, as well as for relapses of hormonally sensitive and insensitive syndrome, but in combination with other drugs that cause immune suppression. Glucocorticoids are prescribed to children orally and intravenously, depending on the medication (Prednisolone or Methylprednisolone) and the activity of adrenal hormones.

Cytostatic drugs are carried out in a course together with prednisone, for hormonally dependent and independent nephrotic syndrome. It is important to understand that cytostatics are very toxic drugs with many side effects, among which it is necessary to highlight the most dangerous:

• blood cancer (due to damage to the red or white bone marrow);
• drug-induced toxic hepatitis, leading to the early development of cirrhosis;
• complete fibrosis of the lung parenchyma;
• hemorrhagic syndrome;
• insufficiency of sex hormones and much more.

Selective immunosuppressants are used for hormonally dependent and often relapsing nephrotic syndrome. Before prescribing them, a fine-needle biopsy of the kidney must be performed, and a few hours after using drugs of this group, the procedure is repeated. This is done to identify toxic effects on the child’s kidney. During treatment with selective immunosuppressants, constant monitoring of biochemical blood parameters is carried out.

Treatment of focal segmental glomerulosclerosis (FSGS)

Today it is considered the most common cause of nephrotic syndrome and requires the same treatment. With adequate therapy, it causes stable remission and the survival rate of children over 10 years reaches 90-95%. It is important to know that when diagnosing hormonal insensitivity, a kidney biopsy is performed.

The main goal of treatment for focal segmental glomerulosclerosis is to achieve the maximum possible remission. Additionally, it is necessary to carry out protein replacement therapy, since this measure also prolongs the child’s survival.

Treatment of mesanglioproliferative glomerulonephritis

For children with normally functioning kidneys and the absence of nephrotic syndrome, cytostatic and immunosuppressive therapy is not prescribed. If a slight increase in blood pressure occurs, the syndrome is corrected with the help of ACE inhibitors (Captopril, Enalopril). If the disease begins to develop in the form of nephrotic syndrome, then treatment is carried out with glucocorticoids and cytostatics.

Prevention

It is necessary to understand that there is no specific prevention of the development of nephrotic syndrome, but to prevent its occurrence, it is necessary to periodically consult with a nephrologist, especially if there are prerequisites, for example, a genetic history. It is necessary to avoid hypothermia and all kinds of allergic reactions.

It is difficult to predict the consequences of the development of nephrotic syndrome, but it must be taken into account that with proper and timely treatment the prognosis will be positive.

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

• congenital and hereditary nephropathies;
• acquired kidney damage.

In group 1, several fairly large subgroups can be distinguished:

1. anatomical defects of the kidneys (changes in quantity, 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. tubulopathies;
5. tumors;
6. dysmetabolic nephropathies.

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

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

There are primary and secondary congenital nephrotic syndrome.

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

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

Congenital nephrotic syndrome Finnish type VNS(congenital microcystic nephropathy, infantile nephrosis, Finnish type microcystic kidney disease) 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 SUD have been described in monozygotic twins and children from consanguineous marriages. Boys and girls get sick equally.

Pathogenesis.

The pathogenesis of the disease comes down to protein disorders nephrin and podocin. Morphologically, microcystosis of the proximal tubules in the corticomedullary zone, signs of glomerular immaturity, and focal proliferative changes in mature glomeruli are detected.

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 glycosuria, 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 is normal. P is symmetrically enlarged on ultrasound. In such children, the stigma of dysembryogenesis is pronounced. In most cases, with this disease during pregnancy, gestosis, threats of miscarriage and premature birth, and intrauterine growth retardation are observed. The weight of the placenta is sharply increased and reaches 40–50% of the newborn’s body weight.

Treatment.

There is no effective treatment for Finnish type SUD. Intravenous infusions of albumin (3–4 g/kg) followed by (0.5 mg/kg), use of vitamin D2, calcium, prevention of purulent-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 with the use of indomethacin and ACE inhibitors.

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

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

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

Urinary system infection.

Urinary tract infection (UTI)– an infectious-inflammatory process in the urinary system without indicating the level of damage. The term “urinary tract infection” is valid 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 identifying pathology to clarifying the topical level and determining a specific nosological form. UTI is a collective concept that includes urethritis, etc.

Along with the concept of IC, another term is 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 clinical, laboratory and instrumental studies and differential diagnosis.

UTIs and UTIs are diagnosed in 0.7–1% of full-term and 4–25% of premature and post-term newborns, 5 times more often in boys 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 UTIs are gram-negative microorganisms: Esherichia coli, Klebsiella pneumonia, Enterobacter cloaca, Pseudomonas aureginosa; staphylococci and group B streptococci are less common.

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

Clinical picture.

The most common nasological form of UTI in the neonatal period is pyelonephritis. – microbial inflammatory disease of the kidneys with a predominant localization of the pathological process in tubulointerstitial tissue and damage to the pyelocaliceal system.

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

In the clinical picture of urinogenic pyelonephritis, urodynamic disturbances and local symptoms occupy the first place. Half of the children experience anxiety when urinating, while others experience crying and facial redness before urinating, which is considered as 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.

On the other hand, with a pronounced bacterial process, anemia, leukocytosis, neutrophilia with a shift to the left, and accelerated ESR can be observed.

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

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

Treatment.

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

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

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

Nalidixic acid and nitrofurans are used with caution in newborns due to the risk of developing acidosis and increasing 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, protracted or recurrent course and mixed infection, nonspecific agents are used ( echinacea, interferonogens – cycloferon) and specific (lysozyme, interferon - viferon) immunocorrection. The administration 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 at 1/3–1/4 age-appropriate dose.

Interstitial nephritis.

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

In newborns, this disease is 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.

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, and lymphohistiocytic infiltration.

Infants may experience increased body temperature, adynamia, and decreased diuresis.

Urinary syndrome is characterized by proteinuria in the range of 0.033–0.99 g/l, microhematuria (10–30 red blood cells 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, titrated acidity and ammonium excretion decrease, and the excretion of sodium and potassium in the urine often increases. The development of hidden edema is possible, which is clinically manifested by excessive weight gain. In the most severe cases, acute renal failure develops.

Diagnostics.

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

When increased in size (especially in thickness).

Absolute confirmation of the diagnosis of IN is the results of a morphological study of nephrobiopsy (during the newborn period, nephrobiopsies are practically not performed).

There are isolated reports in the literature about the development of acute renal failure in the first months of life. Initially, 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 (N.D. Papayan, A.V. Savelyeva, 1997).

Treatment.

Treatment of IN is a very complex task, requiring a differentiated approach depending on its cause.

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

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

The issue of prescribing diuretics is decided individually, taking into account the state of renal function.

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

Tubulopathies.

Tubulopathies– diseases united by the presence of a violation of the membrane transport of various substances in the kidney tubules. Primary tubulopathies are diseases in which disruption of the transport of substances occurs primarily in the renal tubules. Secondary tubulopathies are diseases in which the disruption of substance transport is diffuse in nature and is observed not only in the kidneys, but also in other organs.

Clinical picture.

Despite the qualitative and quantitative diversity of transport disorders 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;
• disorders 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 congenital tubulopathies clinically manifest from 2–3 months of life or in the 2nd half of life, sometimes in the 2nd year, when tubular disorders already lead to rickets-like changes in the skeletal bones and delayed psychomotor development.

B.S. Kaplan (1998) provides the following data on disorders of tubular function, the onset of which manifests itself in the neonatal period: renal Debreu de Toni-Fanconi syndrome; renal tubular acidosis: distal type I (Lightwood-Buttler-Albright syndrome), proximal type II; pseudohypoaldosteronism; nephrogenic diabetes insipidus, X-linked.

Renal Debrede Toni–Fanconi syndrome.

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

Diagnostics.

A blood test reveals hypophosphatemia, hypokalemia, acidosis, alkaline phosphatase activity increases. In urine analysis - hyperaminoaciduria (Alanine, arginine, etc.), phosphaturia, glucosuria, natriuria, kaliuria.

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 Magurlit or Blemaren 0.5 g 3 times a day after feeding.

Distal tubular acidosis type I.

Distal tubular acidosis type I(Lightwood-Buttler-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 H+ secretion and excretion, the inability of the distal tubules to maintain a pH gradient, loss of potassium and sodium in the urine, and aldosterone deficiency. Initially, the syndrome manifests itself as delayed 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 intersitial nephritis or pyelonephritis are noted.

Diagnostics.

Blood tests revealed hypokalemia, hyponatremia, metabolic acidosis. In urine analysis - alkaline reaction, hyperkaliuria, hypercalciuria (more than 4 mg/kg · day), decreased concentration ability, decreased total excretion of titratable acids and ammonium.

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 impaired renal concentration function, urolithiasis and nephrocalcinosis. The syndrome can be isolated or combined with other proximal disorders (Debra de Toni-Fanconi syndrome, etc.). Mostly boys are affected.

Clinical picture.

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

Diagnostics.

Blood tests revealed hypochloremia and metabolic acidosis. In the urine there is an acidic reaction, high excretion of potassium, preserved excretion of titratable acids and ammonium, the decrease in concentration ability is less pronounced.

Kidney diseases 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 alkalizing drinks. In case of severe acidosis and dehydration, intravenous administration of sodium bicarbonate solution is indicated at the rate of V = VE of the patient · 0.5 · Body weight.

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

Pseudohypoaldosteronism.

Pseudohypoaldosteronism (renal salt diabetes) inherited in an autosomal dominant manner. For newborns, type I is typical - primary (renal); Type II – secondary (multiple organ). It is characterized by low sensitivity of the tubular apparatus to aldosterone, which leads to low sodium reabsorption by the renal tubules. Clinically, from the first days of life it manifests itself as polyuria, anorexia, adynamia, and arterial hypotension. Due to the large loss of water and sodium, dehydration develops with high hyponatremia and natriuria, hyperkalemia, and metabolic acidosis. Subsequently, there is a delay in body weight, growth and ossification of skeletal bones, and a lag in mental development. 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 (with a norm of 2.5 mcg).

Treatment.

Replacement therapy with sodium chloride is carried out in a volume of 3–6 g/day.

Nephrogenic diabetes insipidus.

X-linked nephrogenic diabetes insipidus is inherited recessively.

Mostly boys are affected. The disease is associated with the insensitivity of the renal tubules to antidiuretic hormone and the excretion of large amounts of urine with low relative density, which leads to the development of severe dehydration and electrolyte disturbances (hypernatremia, hyperchloremia). Typically, the disease manifests itself from 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 case of significant polyuria, megatestis, megaureter, and hydronephrosis may develop.

Diagnostics.

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

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

Treatment.

The main component of treatment for renal diabetes insipidus is providing 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 non-steroidal anti-inflammatory drugs - indomethacin – 2 mg/kg daily.

The combined use of the above drugs is effective. 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 syndrome is an autosomal recessive disease in which three different hereditary protein defects are identified, which is accompanied by hypokalemia, hypochloremic metabolic alkalosis, extremely high levels of aldosterone and renin in the blood 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. The disease is believed to be associated with impaired chloride reabsorption. Clinically, from birth, poor appetite, vomiting, muscle hypotonia, constipation, polyuria (diuresis can reach 12–50 ml/kg h), polydipsia, hypokalemic convulsions, and paresthesia are noted. In the future, children lag behind in physical development. With this syndrome, the clinical picture of nephrocalcinosis may appear in 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. Today, the best treatment for the disease is the use of prostaglandin synthesis inhibitors – indomethacin at a dose of 2 mg/kg daily.

Renal vein thrombosis.

Renal vein thrombosis (RVT) develops mainly in premature newborns during the 1st month of life due to severe perinatal hypoxia, dehydration, shock, sepsis, and “blue” heart defects.

Predisposing factors are complicated childbirth, diabetes mellitus in the mother, and pathological decrease in the newborn's body weight.

Clinical picture.

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

Urinary syndrome is characterized by albuminuria and gross hematuria. With bilateral TPV, acute renal failure develops rapidly.

Diagnostics.

From the blood side, the most constant signs are anemia, thrombocytopenia, leukocytosis.

Hypercoagulation is noted, the ethanol test is sharply positive. The level of plasma fibrinogen and 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 significance. Excretory urography (not recommended in the initial stages of TPV and due to great technical difficulties in premature newborns) reveals a “silent” kidney with a unilateral process.

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

Treatment.

The treatment uses antiplatelet agents, anticoagulants (heparin at a starting dose of 50 U/kg every 6 hours under Lee-White coagulation control), fibrinolytics (fibrinolysin, urokinase, streptase) - 10 ml/kg dropwise for 1 hour together with heparin.

Physiotherapeutic methods are used– electrophoresis of heparin, aminophylline, nicotinic acid on the kidney area.

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

Renal artery thrombosis.

Renal artery thrombosis (RAT) is a rare disease in newborns that occurs due to dehydration, maternal diabetes, embolism through the patent ductus arteriosus, or as a complication of catheterization of the renal arteries.

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

The most serious complication of TPA is renal infarction.

Treatment.

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

Arterial hypertension is diagnosed when blood pressure rises to more than 90/60 mmHg. Art. in full-term and more than 80/45 mm Hg. Art. in premature newborns. Newborns born to mothers with hypertension will have slightly higher blood pressure at birth. Arterial hypertension is rare in newborns, but in children in intensive care, its incidence 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: infantile polycystic kidney disease, 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 (hydralysine, apressin– 0.2–2 mg/kg intravenously or orally every 6–12 hours, diazoxide– 1–3 mg/kg intravenously, nitroprusside– 0.2–10 mcg/kg min); adrenergic blockers (obzidan, anaprilin– 0.5–2 mg/kg · day orally, labetolol – 0.5–1.0 mg/kg · hour intravenously); angiotensin-converting factor inhibitors (captopril– 0.01–0.5 mg/kg orally every 8–12 hours, Enap – 5–15 mcg/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).

Family (genetic) associated with the presence of gene mutations of a predominantly recessive nature. As a rule, these mutations lead to disruption of the biosynthesis and expression of proteins that form the slit diaphragm between the small processes of podocytes, which leads to their melting and disruption. The most studied mutations are NPHSI (congenital Finnish type - disorder of nephrin synthesis) and NPHS2 (familial autosomal recessive steroid-resistant - disorder of podocin synthesis).

Another type of mutation that leads to nephrotic syndrome (NS) is mutation of the WT-1 gene.

Congenital NS of the Finnish type- a disease of an autosomal recessive nature, described in detail using the example of the Finnish population, where two main types of nephrin mutations prevail - fin-major and fin minor in the nephrin gene, located on the 19th chromosome. There are numerous descriptions of cases of this disease in people of other nationalities, however, in the non-Finnish population, other spontaneous type mutations, of which more than 60 have been described, are responsible for its development.

Clinic

At the birth of a child with Finnish type NS, a significant increase in the weight of the placenta attracts attention. During pregnancy, an increase in the level of a-fetoprotein in the mother's blood can be recorded. All signs of nephrotic syndrome appear from birth or the first days of life. The disease is characterized by a steady progressive decline in renal function with the development of ESRD on average by 3-4 years of age. Morphologically, a pronounced and widespread expansion of the tubules is characteristic. The glomeruli may appear intact or have moderate signs of compaction of mesangial structures with subsequent formation of sclerosis. Immunofluorescence study is uninformative; electron microscopy reveals diffuse melting of the small podocyte footsteps.

Diagnostics of congenital NS of the Finnish type is based on clinical data, family history and the detection of known gene mutations. Kidney biopsy is recommended at 2-3 months of life. In earlier periods, pathognomonic changes may not be expressed. It is necessary to exclude congenital and CMV infection as causes of congenital or infantile NS.

Treatment

Continuous replenishment of protein loss in urine is carried out through infusions of a 20% albumin solution and a high-protein diet. It is used to treat edema. In severe cases, a unilateral nephrectomy is performed or a so-called pharmacological nephrectomy is performed by prescribing high doses of indomethacin and ACE inhibitors (ACE inhibitors). As the disease progresses, combination antihypertensive treatment (ACE inhibitors, calcium antagonists) and dialysis may be necessary.

Due to the constant loss of protein, protein-energy deficiency develops and the risk of infections increases. It is possible to perform a bilateral nephrectomy at about 6 months of age with the start of dialysis therapy carried out before kidney transplantation.

The disease does not return after transplantation, but cases of the development of autoimmune nephritis due to the formation of a nephrin recipient by the body have been described.

In familial steroid-resistant NS with NPHS2 disorders, the podocin gene is located on chromosome 1. More than 30 mutations of this gene are known, leading to the development of familial steroid-resistant NS. Some mutations are accompanied by the onset of NS in the first year of life, and other NS in adolescence or adulthood. Although FSGS is typical of NS associated with a podocin mutation, early stages of the disease may show minimal changes in the glomeruli, usually without fluorescence or complement.

In patients with NPHS2 mutations, immunosuppressive therapy is not used. Preference is given to ACE inhibitors and AT II receptor blockers. Return to a transplanted kidney is rare. In this case, cyclophosphamide, steroids and plasmapheresis are effective, although podocin is not detected. Families with cases of autosomal recessive NS are subject to medical genetic counseling.

Mutations of the WT-1 gene may cause the syndrome Denys-Drash, manifested by early onset and rapid progression of NS. The disease is often combined with male pseudohermaphroditism and Wilms tumor, but the development of incomplete variants of the syndrome is possible. The morphological basis of Denys-Drash syndrome is diffuse mesangial sclerosis. Differential diagnosis, based on morphological features, can be made with diffuse mesangial sclerosis against the background of congenital CMV infection. Immunosuppressive therapy is not indicated; NS return does not occur in the transplanted kidney.

Another disease associated with the WT1 mutation is Fraiser syndrome, also manifested by NS, but with a later onset and less rapid progression. Also characteristic is a combination with male pseudohermaphroditism. Changes in the kidneys include FSGS, resistant to steroids and immunosuppressants. Patients undergo gonadectomy and plastic surgery with the formation of female phenotypic characteristics.

NS may be part of a symptom complex of various genetic diseases. These include various syndromes (Charcot-Marie-Tooth, Schimke, Galloway-Mowat, Pierson, etc.) (A.N. Tsygin, 2010), in which NS and terminal chronic renal failure develop in combination with abnormalities of the central nervous system, skeleton, or ocular disorders .

Familial cases of NS may also be associated with renal amyloidosis due to periodic illness or other variants of amyloidosis of a genetic nature. About 30 families with autosomal dominant NS associated with a mutation of the subcytic protein α-actinin, the gene of which is located on the 19th chromosome, have been described. The disease debuts at the age of 30-40 years with further progression. To date, about 90 different podocyte proteins are known, so the discovery of new types of mutations responsible for the development of familial cases of NS cannot be ruled out.

Thus, if NS develops in children under 1 year of age, the probability of having podocyte gene mutations is 80%. Genetic and syndromic causes of NS determine resistance to immunosuppressive therapy. Such patients are prescribed infusions of albumin, ACE inhibitors, and NSAIDs to reduce. In the long term, for severe cases, radical treatment is kidney transplantation.

Initially, swelling appears on the face, eyelids, and is observed on the ankles of the legs, then the fluid drops lower, affecting the back, abdomen, and genitals.

Nephrotic syndrome (NS) belongs to the category of kidney pathologies, although it is not considered a disease. This is a complex of symptoms that characterizes a pathological condition. It manifests itself as changes in laboratory test parameters. NS is characterized by a massive increase in protein concentration in the urine, a drop in its level in the blood (especially albumin), hyperlipidemia (high serum cholesterol), and severe edema. But there are forms of NS in which swelling is not observed, and laboratory test results indicate impaired renal function. The disease most often develops in adults after 40 years of age. Nephrotic syndrome occurs very rarely in children and is more common in boys at an early age. Later (in adolescents), pathology is observed equally often in both sexes.

NS in children

The syndrome in children is the primary form and its treatment is simple. In pediatrics, severe disease that is difficult to treat is extremely rare. The appearance of symptoms in children is a reason for inpatient treatment, since the hospital will conduct the necessary examination and find out what triggered the syndrome.

Causes of the syndrome

There are primary and secondary forms of development of the disease. Primary NS is of 3 types:

• congenital;
• infantile;
• idiopathic.

Congenital nephrotic syndrome is observed in newborns and infants in the first three months of life. The reasons lie in heredity (Finnish type), and are also caused by congenital infectious lesions (rubella, toxoplasmosis, hepatitis B, HIV). Primary glomerulonephritis can cause congenital nephrotic syndrome in children.

Infantile NS is observed in children under one year of age. Infantile syndrome, which is diagnosed between the ages of 4 and 12 months, just like congenital syndrome, is characterized by a severe course, and by the age of 5 years, most of these patients experience renal failure.

Idiopathic nephrotic syndrome in children is caused by unknown causes. It is characterized by minimal changes in the renal glomeruli.

The secondary form of NS in childhood develops as a consequence of the following pathologies:

• amyloidosis;
• diabetes mellitus;
• lupus;
• rheumatoid arthritis;
• infections;
• chronic kidney pathologies;
• renal vein thrombosis.

In accordance with the reasons that caused the organ dysfunction, treatment is prescribed.

Varieties

According to the clinical manifestation of the syndrome, the following types are distinguished:

• clean;
• mixed;
• full.

The pure type of course is characterized by symptoms of proteinuria, hypoalbuminemia, and an increase in lipids in the blood serum. The mixed type is characterized by the presence of blood in the urine or vascular hypertension. The full type of disease is characterized by a combination of all the symptoms of the pure and mixed variant. There is also an incomplete form, which is observed if any of the symptoms are absent.

Development mechanism

The main symptom of nephrotic syndrome is massive proteinuria (high). If glomerular filtration is impaired, protein molecules penetrate into the urine and are excreted from the body. Such disorders develop due to pathological changes in the structure of epithelial cells of renal tissue. The connection between them is lost, “gaps” are formed, through which protein molecules enter the bloodstream.

With nephrotic syndrome, the level of anticoagulants decreases, that is, blood clotting increases. This is fraught with the formation of blood clots.

A decrease in protein in the blood reduces the osmotic pressure in the plasma, which leads to the accumulation of fluid in the body and the appearance of edema. Against the background of NS, membranous and membranous-proliferative glomerulonephritis develops, which is characterized by impaired hemostasis and the formation of blood clots in the capillaries of the renal glomeruli.

Clinical manifestations

The main symptom of NS is edema, which develops in a generalized manner. Initially, they appear on the face, eyelids, and are observed on the ankles of the legs, then the swelling drops lower, affecting the back, abdomen, and genitals.

A lesion such as ascites (fluid accumulation in the abdominal cavity) or anasarca (large-scale swelling of the legs, genitals, and lower abdomen) may develop. Children experience rapid formation of tissue swelling. The reason for this is the imbalance between the accumulation and removal of protein from the body.

If a patient experiences swelling for a long time, then a change in the skin occurs. They become dry and cracks form through which liquid leaks out. The skin is pale. Children are not active. They exhibit the following clinical signs:

• impaired diuresis;
• constant thirst;
• decreased activity;
• nausea;
• vomit.

The swelling is soft to the touch; when pressed with a finger, a depression remains. With further progression of the disease, the swelling becomes hard. If a child develops ascites, an enlarged abdomen and shortness of breath are observed. It is possible that hydrothorax (fluid in the pleural area) and hydropericardium (accumulation of moisture in the cardiac membrane) may be present. This leads to swelling of the entire body.

Congenital NS is difficult, since children with this pathology are born prematurely with imperfect renal function. A large loss of protein leads to the development of complications of NS in the form of pulmonary edema, which is accompanied by characteristic manifestations of respiratory failure (difficulty breathing, cyanosis of the lips, inability to lie down). After treatment of the syndrome, gastrointestinal dysfunction, Cushing's syndrome, pathological fragility of bones, and growth retardation occur.

Diagnostic measures

In the diagnosis of nephrosis, special attention is paid to the study of urine and blood (protein levels). Diagnosis includes a general examination of the patient for the presence of swelling, collecting an anamnesis regarding the hereditary etiology of the disease or concomitant pathologies.

The patient is prescribed the following diagnostic procedures:

• UAM (urinalysis);
• CBC (blood test);
• biochemistry of urine and blood;
• , Nechiporenko;
• immunological blood test;
• culture of urine.

A study of urine sediment, ultrasound and x-ray examination of the kidneys and urinary tract are carried out.

Treatment

Treatment is carried out only in a hospital, preferably in the nephrology department, where there are all conditions for high-quality monitoring of a sick child and provision of emergency care in the event of the development of complications.

Primary childhood NS is easily treated with corticosteroid drugs. The child's body is susceptible to such medications, so the recovery process is successful.

Therapy lasts two months. Initially, a higher dose of the drug is prescribed, which, if the treatment results are positive, is gradually reduced every month until the drug is completely discontinued. This maintenance therapy is carried out at home. It is strictly forbidden to interrupt the course of treatment, even if symptoms of the disease are not observed, since this can provoke a new round of pathology, but in a more severe form.

If the child has a secondary form of NS, then prednisolone therapy is carried out in combination with medications prescribed for the treatment of the underlying disease. If corticosteroid therapy is ineffective, cytostatics may be prescribed.

Along with drug therapy, a diet is used that excludes the intake of salt and limits the intake of liquids, fatty and spicy foods. During this period, it is necessary to strictly monitor the child’s daily diuresis and observe the drinking regime (the ratio of the liquid drunk and excreted). Further follow-up is indicated.

Forecast

The outcome of the disease will be favorable if the pathology is diagnosed in the early stages of development. The longer the process of providing medical care is delayed, the more difficult the treatment is and the higher the risk of complications, including kidney failure.