Shilov E.M. (ed) Nephrology - Textbook for postgraduate education. Mukhin N.A. Nephrology: Emergencies - A Practical Guide

On December 7, 2011 in Kazan, nephrologists, internists, endocrinologists, general practitioners gathered for a very significant event - the republican scientific and practical conference "Actual Issues of Prevention and Treatment of Kidney Diseases 2011".

Yu.V. Arzhanov. He made a presentation on the development of specialized nephrological care, including renal replacement therapy.

To date, all three types of renal replacement therapy are presented in the Republic of Tatarstan, the main one being hemodialysis. There are 14 dialysis centers and departments in the Republic of Tajikistan that provide medical care on an inter-district basis. 11 centers provide outpatient dialysis care, of which 5 are private. “Private public partnership to provide outpatient dialysis care is being implemented successfully”, noted the speaker. Since 2006, an international outpatient dialysis center has been operating in Kazan. In 2008, centers of LLC "Clinic of Modern Medicine" were opened in Nizhnekamsk and Bugulma, and since January 1, 2011 in Kazan. All projects are invested by private companies. The implementation of these projects and the development of hemodialysis departments in the institutions of the republic made it possible to provide full-fledged nephrological care. Since 2006, the Republic's Ministry of Health has begun monitoring patients receiving treatment by program hemodialysis in order to predict the need for this type of treatment in the near and long term. The amount of funding for hemodialysis in 2010 amounted to more than 450 million rubles. The republic also introduced the method of peritoneal dialysis in the conditions of the two largest diversified healthcare institutions of the republic. 15 patients have already been admitted for treatment. The development of this method is in the near future. Since 2005, the indicator of provision with dialysis care in the Republic of Tajikistan has increased and as of November 1, 2011 it is 254 per 1 million population. also, according to A.Yu. Arzhanov in the Republic of Tatarstan has some experience in kidney transplantation. Today, 165 patients are under observation after kidney transplantation. For 11 months, 29 transplantations were performed. The rate of availability of all types of renal replacement therapy was 297.7 per 1 million population. This year, 12 inter-district nephrology rooms have been opened. The Ministry of Health of the Republic of Tajikistan has developed and approved by the relevant order the procedure for providing assistance at the prehospital stage to patients with nephrological diseases within the framework of primary medical and social care. The main functions of a nephrologist in a nephrological office are defined as follows: providing advisory, outpatient medical care to patients with nephrological diseases, as well as to persons with an increased risk of such diseases; dispensary observation of patients with nephrological diseases; keeping records of patients who are planned to be treated with renal replacement therapy; maintaining a register of patients with chronic diseases; carrying out preventive measures for persons with an increased risk of developing nephrological diseases, as well as conducting schools for patients with nephrological diseases.

“In the near future, we see the following tasks:

Ensuring timely detection of patients with signs of kidney damage and their complications, as well as with an increased risk of developing nephrological diseases.

— providing high-quality and affordable nephrological care.

— monitoring the quality of dialysis care”, summed up the speaker.

Further, the main nephrologist of the Ministry of Health and Social Development of Russia, President of the Scientific Society of Nephrologists of Russia, Professor E.V. Shilov. “The end of the autumn of 2011 became a fateful year for Russian nephrologists, important events took place. Domestic Nephrology is 1 year old. The importance of this event cannot be overestimated. Domestic nephrology was born again, because for 5 years it was not officially recognized. We have managed to prove that nephrology cannot be a specialty within therapy. This was the beginning of the development of the "new" nephrology, the creation of an organizational and legal framework. One of the objectives of our conference is to understand at what stage of its development nephrology is, at what it has been all these years and, accordingly, it needs to be corrected,” the chief nephrologist of Russia began his speech with these words. The professor noted that a national meeting on the development of the nephrological service was recently held in Moscow. The decisions of this meeting should be presented shortly.

Evgeny Mikhailovich presented the administrative hierarchy of the Russian Nephrological Service. The problem lies in the fact that the issue of the chief specialists in nephrology of the federal districts of the Russian Federation, which should be 8 people, has not yet been resolved. “It is important that now the chief specialist of the federal district will choose a team of chief nephrologists, that is, a team of like-minded people will be created,” the professor emphasized. Over the past year, we have been working on the creation of a regulatory framework, the center of which is a new procedure for the provision of specialized medical care - a general document that defines the structure of the service, the subordination of elements, the functionality of the elements in the most general form without prescribing details. The second level documents are federal standards of medical care. Clinical guidelines, protocols for managing patients, which are approved by the Ministry of Health and Social Development of the Russian Federation, are the third level of the regulatory framework for the nephrological service.

“Thanks to the health care modernization program, one of the elements of his tasks was the element of implementing standards. I hope that by the end of the year we will receive an order from the minister to approve the procedure for providing medical care. What's new in the structure of nephrology care? Previously, everything was reduced to the departments of nephrology and dialysis, now the service has moved to the primary link, a good example of this is the Republic of Tatarstan, where nephrology rooms have been created, ”said Professor E.M. Shilov. Yevgeny Mikhailovich mentioned violations in the provision of quality medical care. So, during the year, problems with the quality of consumables were discovered. “So far, we have not been able to convey to the leadership the extent of our problems due to incorrect statistics,” Evgeny Mikhailovich emphasized. Approximately, every year in the country there are about 6 thousand patients with renal failure. In total, there are about 45,000 adults with CRF, according to the Ministry of Health and Social Development of the Russian Federation. 171 people per 21 million people in Russia on average receive renal replacement therapy. The problem also lies in the fact that the exact number of nephrologists in Russia as a whole is not completely clear (there are approximately 1,700 of them). “The number of nephrologists should equal the number of cardiologists – this is the opinion in the USA. If this happens, the mortality rate from CRF will decrease,” Evgeny Mikhailovich emphasized. Unfortunately, the growth of CKD exceeds the growth in the number of nephrological patients. Also, according to the speaker, there are considerable problems in the field of laboratory diagnosis of CKD. Evgeny Mikhailovich believes that the way out of this situation is the full development of nephrology in the regions. “In general, the way out is in the creation of federal programs (this year a program was developed to improve the nephrological service in the Russian Federation, which is under consideration)”, summed up the country's chief nephrologist.

The following report was presented by the chief specialist - nephrologist of the Ministry of Health of the Republic of Tajikistan, Professor O.N. Sigitova - "Differentiated approach to nephro- and cardioprotection in CKD - ​​standards and the formation of new directions." According to official data, CKD is not included in the ICD-10 as an independent disease. Diabetes mellitus is the cause of CKD in 50% of cases. Many patients with chronic kidney disease die from cardiac causes, 95% of patients have arterial hypertension (AH), which in turn increases the risk of stroke, coronary artery disease, heart failure. According to the speaker, the level of proteinuria affects the development of CKD. Glomerular filtration rate (GFR) and diseases of the cardiovascular system in hypertension are interrelated. It is necessary to pay attention to patients with diabetes mellitus. “Dialysis therapy is an expensive treatment, therefore, early detection of such patients is necessary, Professor Olga Nikolaevna noted, moreover, it is important to control blood pressure levels – without this, the risk of mortality increases by 30%. A low-protein diet and CHAF are the basis for reducing proteinuria. The professor also noted the effectiveness of MBD: a decrease in urea, proteinuria, anemia, dyslipidemia, phosphates, and hypertension. In the correction of hyperlipidemia, the progression of CHD slows down with a decrease in lipid levels. The principles of nephroprotection were also noted in the message.

The next presentation was devoted to modern approaches to the treatment of secondary hyperparthyroidism. He was voiced by Professor K. Ya Gurevich. Correction of hyperphosphatemia as a key factor in the treatment of mineral phosphorus disorders was discussed by G.V. Volgin. For decades, phosphorus has been reportedly used to treat appetite disorders. The level of phosphorus begins to rise when the glomerular filtration rate (GFR) decreases and becomes less than 30 ml. Hyperphosphatemia for a long time is compensated by increased excretion of phosphates by the kidneys. Fibroblast growth factor-23 plays a central role in the control of serum phosphorus. Highlighted the clinical consequences of hyperphosphatemia. Phosphate contributes to the development and progression of chronic nephropathy and cardiovascular diseases. Hyperphosphatemia is a key factor in extraosseous calcification. Also, with this disease, cardiovascular disorders and an increased risk of mortality are noted. Causes of death in dialysis patients with hyperphosphatemia include ischemic heart disease, sudden death, CVD, infections, and more. The professor singled out modern strategies for correcting phosphorus imbalance. In patients with stage 3-5 CKD, the phosphorus level should be in the range of 80-1.45 mmol/liter. According to the speaker, a comprehensive solution should always be used, which allows keeping the level of phosphorus in the required range. At the end of her presentation, the professor mentioned the use of phosphate binders in treatment, as well as educational programs for patients. The following message was devoted to the features of kidney damage in a patient with hyperuricemia. Speaker - Associate Professor of the Department of Hospital Therapy of KSMU A.N. Maksudov. In the second part of the meeting, the attention of the participants was focused on such issues as the detection and elimination of proteinuria, albibuminuria, ischemic nephropathy, treatment of nephrogenic hypertension at the stages of CKD, and much more. The conference ended with a discussion of reports and an exchange of views.

M.: GEOTAR-Media, 2007. - 688 p. The textbook - one of the first dedicated to nephrology - is intended primarily for the system of postgraduate education and can be used both in primary specialization and in the future, when passing thematic advanced courses. Along with the traditional ones, it includes a special section on the relationship between nephrology and general medical practice, as well as chapters that cover topics related to urology.
The training manual presents current data on the etiology, pathogenesis, diagnosis, clinical picture, treatment and prevention of kidney diseases.
Separate chapters are devoted to modern methods of studying nephrological patients, kidney damage in systemic diseases, amyloidosis, diabetes mellitus, infective endocarditis, as well as modern aspects of hemodialysis and nephroprotective strategy.
It is intended for therapists, nephrologists, general practitioners, family doctors, as well as residents, interns and senior students of medical universities.
Content:
Nephrology and modern clinic of internal diseases
Problems of Nephrology in General Medical Practice
Fundamentals of anatomy and physiology of the kidney
Kidney function, evaluation methods, clinical significance
Renal regulation of fluid volume, sodium and potassium balance
Clinical Significance of Acid-Base Disorders
Methods of examination of a nephrological patient
Clinical study of urine
Radiation methods of diagnostics in nephrology
Kidney biopsy
Hematuria
Proteinuria and nephrotic syndrome
Renal arterial hypertension
Arterial hypertension in parenchymal kidney disease
Renovascular hypertension
Malignant arterial hypertension
Glomerulonephritis
Kidney damage in systemic diseases
Lupus nephritis
Kidney damage in systemic vasculitis
Polyarteritis nodosa
Vasculitis associated with antibodies to the cytoplasm of neutrophils
Schönlein-Henoch purpura
Mixed cryoglobulinemia
Goodpasture's syndrome
Systemic scleroderma
Thrombotic microangiopathies: hemolytic uremic syndrome, thrombotic thrombocytopenic purpura
Antiphospholipid Syndrome
Amyloidosis
Tubular dysfunction
Tubulointerstitial nephropathies
Pyelonephritis
Urolithiasis disease
Gouty nephropathy
diabetic nephropathy
Ischemic kidney disease
Alcoholic Nephropathy
Cystic kidney disease
Autosomal dominant polycystic kidney disease in adults
Autosomal recessive polycystic kidney disease
Kidney damage in infective endocarditis
Kidneys and pregnancy
Urological problems in the practice of a nephrologist
Urological aspects of hematuria
Abnormalities of the kidneys and upper urinary tract
Tumors of the kidney
Tuberculosis of the kidneys
Acute renal failure
Chronic renal failure
Renal Replacement Therapy
Hemodialysis and peritoneal dialysis
Nephrological aspects of kidney transplantation
chronic kidney disease
Lifestyle and chronic kidney disease
Renoprotective strategy
Principles of evidence-based medicine in nephrology

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For citation: Tareeva I.E., Shilov E.M. MODERN CONCEPTS ABOUT GLOMERULONEFRITIS // BC. 1997. No. 23. S. 3

The article highlights modern ideas about the etiology, clinical and morphological features of glomerulonephritis. The characteristic of glomerulonephritis is given not only as an independent nosological form, but also within the framework of systemic diseases (systemic lupus erythematosus, hemorrhagic vasculitis, Wegener's granulomatosis).

The paper covers the current views of the etiology, clinical and morphological fears of glomerulonephritis. It characterizes glomerulonephritis not only as an independent nosological entity, but also as a component of systemic diseases (lupus erythematosis, hemorrhagic vasculitis, Wegener’s granulomatosis)

I.E. Tareeva - Dr. med. Sciences, Professor, Corresponding Member RAMN, head. Department of Nephrology, Faculty of Postgraduate Education, Head. Department of Nephrology NRC MMA them. THEM. Sechenov
EAT. Shilov - Dr. med. Sci., Professor, Department of Nephrology, Faculty of Postgraduate Education, MMA named after THEM. Sechenov
Prof. I.Ye.Tareyeva, MD, Corresponding Member ofthe Russian Academy of Medical Sciences; Head, Department of Nephrology, Faculty of Postgraduate Training; Head, Department of Nephrology, Research Center, I.M. Sechenov Moscow Medical Academy
Prof. Ye. M. Shilov, MD, Department of Nephrology, Faculty of Postgraduate Training, I.M. Sechenov Moscow Medical Academy

G Lomerulonephritis (GN) is an immunoinflammatory disease of the kidneys, predominantly affecting the glomeruli but involving both tubules and interstitial tissue. The main clinical types of GN are acute, chronic, and rapidly progressive. GN are independent nosological forms, but can also occur in many systemic diseases: systemic lupus erythematosus, hemorrhagic vasculitis, subacute bacterial endocarditis, etc.
In the etiology of GN play the role of infection (most clearly in acute poststreptococcal GN), toxic substances (organic solvents, alcohol, mercury, lead, etc.), exogenous antigens that act with the involvement of immune mechanisms, including in within the framework of immediate type hypersensitivity (atopy), rarely - endogenous antigens - DNA, uric acid, tumor (Table 1). The etiological factor can be established in 80-70% of patients with acute GN and in 5-10% of patients with chronic GN. In the remaining patients, the cause of the disease remains unknown.
In the vast majority of cases, GN develops with the participation of immune mechanisms. In acute post-streptococcal GN, this is the formation of antibodies 10-12 days after pharyngitis or tonsillitis, in chronic GN, it is more often the slow formation of immune complexes containing the antigen and antibodies to it, which are deposited in the glomeruli with insufficient mechanisms for their removal, in most patients with rapidly progressive GN - this is the formation of antibodies to glomerular tissue (basement membrane of capillaries). Immune complexes and anti-basement membrane antibodies can be detected and identified in kidney tissue by immunohistochemistry. The renal glomerulus responds to immune damage with two types of pathological reactions: the proliferation of renal glomerular cells (mesangial, endothelial, epithelial) and the production of intercellular substance by these cells. Both processes stimulate cytokines (primarily interleukin-1, tumor necrosis factor, platelet-derived growth factor, transforming growth factor beta), which are secreted by cells that infiltrate the renal glomerulus.
Table 1. Established causes of GN and the development of nephritis in other diseases

Minimum changes
Medications, especially non-steroidal anti-inflammatory drugs
Lymphogranulomatosis
Focal segmental glomerulosclerosis
Intravenous drug use
AIDS

Tumors
Lithium treatment
IgA nephropathy
Cirrhosis of the liver
celiac disease
Dermatitis herpetiformis
Seronegative arthritis
Tuberculosis
Membranous nephropathy
Tumors - 15% of cases (frequency increases with age)
Medications (especially gold and penicillamine)
Infections: hepatitis B, syphilis, malaria
Systemic lupus erythematosus
sickle cell anemia
Mesangiocapillary GN
Tumors and chronic lymphocytic leukemia
Infections: endocarditis, hepatitis B, schistosomiasis

Table 2. Clinical manifestations of GN

Chronic GN more often it develops slowly, with an imperceptible onset, less often there is a clear connection with acute GN. In the pathogenesis, the main role is played by immune mechanisms, but non-immune factors of progression are quickly switched on.
The issue of classification is complex. In world practice, the classification is based on a complete morphological picture of GN, which requires mandatory puncture biopsy of the kidney. This approach is quite legitimate, and we will further dwell on this classification, although for Russia, where only a few nephrological centers perform a kidney biopsy and, moreover, not all of these centers have qualified (in terms of nephrological) morphologists, it often turns out to be unacceptable. Therefore, we still put the clinical classification in the forefront, highlighting the following variants of GN (a slightly modified classification by E.M. Tareev): latent, hematuric, nephrotic, hypertensive and mixed.

Table 3. Morphological classification of chronic GN

Latent GN- the most common form of chronic GN, manifested only by changes in urine (proteinuria up to 3 g / day, small erythrocyturia), sometimes slightly elevated blood pressure. The course is usually slowly progressive, the prognosis is better with isolated proteinuria, worse with a combination of proteinuria with erythrocyturia, a 10-year survival rate is 85-70%.
Hematuric GN accounts for 6-8% of cases of chronic GN, is manifested by constant hematuria, sometimes with episodes of gross hematuria, the course is quite favorable. Further in the article, we will dwell in more detail on the most common form of hematuric GN, established during a kidney biopsy - the so-called IgA nephropathy.
Nephrotic GN(10-20% of cases of chronic GN) proceeds, as the name suggests, with nephrotic syndrome (proteinuria above 3.5 g/day, hypoalbuminemia, edema, hypercholesterolemia). Nephrotic syndrome is usually recurrent, rarely has a persistent course with gradual addition of arterial hypertension and renal failure. The prognosis is worse when combined with severe erythrocyturia. The course, prognosis, response to treatment are largely determined by the morphological variant of nephritis.
Hypertensive nephritis- a slowly progressive form of nephritis, very rarely requiring active therapy. It is characterized by a minimal urinary syndrome (proteinuria is usually not higher than 1 g / day, slight erythrocyturia) in combination with severe arterial hypertension.
Mixed GN characterized by a combination of nephrotic syndrome with arterial hypertension (nephrotic-hypertonic GN), a steadily progressive course.
According to the clinical classification of M.Ya. Ratner and V.V. Serov, nephritic, nephrotic and nephrotic-hypertonic types of nephritis are distinguished; among the nephritic - the most active, active and inactive options.
Table 4. Main forms of GN with crescents

Morphological variants of GN are as follows (Table 3): 1) minimal glomerular changes, 2) focal segmental glomerulosclerosis (FSGS), 3) membranous GN (membranous nephropathy), 4) mesangioproliferative GN, 5) mesangiocapillary GN, and 6) fibroplastic GN. The first three variants, in terms of their morphological characteristics, do not fully correspond to the idea of ​​GN as an inflammatory disease (primarily due to the absence of mesangial cell proliferation), and therefore the term "nephropathy" is often used in foreign literature, and the unifying concept is "glomerular diseases"; we consider it possible to use the term "glomerulonephritis" in all cases.
Minimal glomerular changes (lipoid nephrosis) characterized by the absence of changes in light microscopy and immunofluorescence studies. Only electron microscopy reveals the fusion of pedunculated processes of epithelial cells (podocytes), which is recognized as the main cause of proteinuria in this form.
This morphological form is observed more often in children, but also occurs in adults. Most patients have a nephrotic syndrome with severe edema, anasarca, massive proteinuria, severe hypoalbuminemia, hypovolemia, and very pronounced lipidemia; 10-20% of patients have erythrocyturia and arterial hypertension. Often a combination with atopic diseases, allergic disorders (asthma, eczema, milk intolerance, hay fever). According to one of the pathogenesis hypotheses, the main importance is attached to the factor that increases glomerular vascular permeability, produced by T-lymphocytes. It is with this form that corticosteroid therapy is most effective, sometimes resulting in one week to the disappearance of edema. In the future, the disease often takes a relapsing course with the development of steroid dependence, but chronic renal failure (CRF) rarely develops. The prognosis is quite favorable, the best among all morphological variants.
Focal segmental glomerulosclerosis (FSGS)- segmental glomerulosclerosis (individual segments of the glomeruli are sclerosed) of parts of the glomeruli (focal changes); the rest of the glomeruli are intact. Immunohistochemical examination reveals IgM. Often this type of morphological changes is difficult to distinguish from minimal changes in the glomerulus, the possibility of transition of minimal changes to FSGS is discussed. It is clinically characterized by persistent proteinuria or nephrotic syndrome, in most patients it is combined with hematuria, in half with arterial hypertension. Despite seemingly moderate morphological changes, the course of the disease is progressive, complete remissions are rare. The prognosis is serious, this is one of the most
adverse variants of GN, rarely responding to active immunosuppressive therapy.
Membranous GN (membranous nephropathy) characterized by diffuse thickening of the walls of the glomerular capillaries with their splitting and doubling, cell proliferation is absent or minimal. Immunohistochemical examination and electron microscopy reveal deposits of immune complexes (deposits of electron-dense material) on the epithelial side of the capillary basement membrane. Interestingly, it is with this type of nephritis that quite often (in 30-35% of patients) it is possible to establish a connection with known antigens - the hepatitis B virus, tumor, drugs. Therefore, in clinical practice, patients with membranous nephropathy for the purpose of possible detection of a tumor (especially of the lungs, kidneys) or infection with the hepatitis virus.
The disease often develops in men, is characterized by proteinuria or nephrotic syndrome; hematuria and hypertension are observed in 15-30% of patients. The course is relatively favorable (especially in women), spontaneous remissions are possible, renal failure develops in only half of the patients, and therefore some authors believe that not all patients should be actively treated.
Mesangioproliferative GN characterized by proliferation of mesangial cells, expansion of the mesangium, deposition of immune complexes (containing IgA and IgG) in the mesangium and under the endothelium. This is the most common morphological type of GN, which (unlike the previous variants) meets all the criteria for GN as an immunoinflammatory disease. It is clinically characterized by proteinuria, hematuria, in some cases nephrotic syndrome, hypertension are noted. The current is relatively favorable. In our observations, the 10-year survival rate (before the onset of terminal renal failure) was 81%.
As a separate option, mesangioproliferative GN is isolated with deposition of immunoglobulin A in the glomeruli - IgA nephritis, IgA nephropathy, Berger's disease. The leading clinical symptom is hematuria. The disease develops at a young age, more often in men. In 50% of patients, recurrent gross hematuria is observed, which occurs with febrile respiratory diseases, in the first days or even hours of illness ("synpharyngitis macrohematuria"). Often, gross hematuria is accompanied by non-intense dull pain in the lower back, transient hypertension. In other patients, IgA nephritis is latent, with microhematuria, often with mild proteinuria. In 10-20% of patients (often older and / or with microhematuria) in the later stages, nephrotic syndrome may join, in 30-35% - arterial hypertension. In the blood serum of many patients, the content of immunoglobulin A is increased. The course is relatively favorable, especially in patients with gross hematuria. In recent years, an increase in IgA nephritis has been noted in the world, in some countries, such as Japan, it has become the predominant type of nephritis. Along with this, the deterioration of the prognosis draws attention.
Mesangiocapillary (membranoproliferative) GN. There are two types of this form of jade, the differences between which can only be established using electron microscopy. In type I, immune deposits are localized under the endothelium and in the mesangial region of the glomeruli, in type II (“dense deposit disease”), linear electron-dense deposits are present inside the basement membrane. In both cases, there is a proliferation of mesangial cells, creating a lobulation of the glomeruli, and a characteristic appearance of the basement membranes - bypass - due to the penetration (interposition) of mesangial cells into them. The clinical picture is the same: isolated urinary syndrome (proteinuria and hematuria) or nephrotic syndrome (in most cases with elements of acute nephritic). Arterial hypertension is often detected, and in almost 1/3 of patients the disease can manifest itself as rapidly progressive renal failure with the presence of crescents in the renal biopsy (we will discuss semilunar nephritis later). Some patients have partial lipodystrophy. Along with the idiopathic form, mesangiocapillary nephritis is detected in a number of other diseases (see Table 1).
Mesangiocapillary nephritis is one of the most unfavorable forms; if untreated, terminal renal failure develops after 10 years in almost 50%, after 20 years in 70% of patients.
Rapidly progressive GN (RPGN)- a relatively rare clinical syndrome that includes active nephritis and a rapid deterioration in renal function (50% decrease in glomerular filtration rate within 3 months). RPGN can develop (Table 4) after infections, contact with organic solvents, but more often the cause of RPGN is systemic vasculitis (Wegener's granulomatosis, microscopic polyarteritis, hemorrhagic vasculitis, essential cryoglobulinemia, etc.) and systemic lupus erythematosus (SLE). RPGN can occur in association with other types of primary GN and also as primary or idiopathic RPGN.
The clinical picture is determined by rapidly progressive renal failure. Usually (but not necessarily) nephrotic syndrome and malignant hypertension are observed, erythrocyturia is always noted. Only post-infectious forms of RPGN have a relatively good prognosis: without additional therapeutic measures, about 50% of patients spontaneously recover, partially - 18%, but 32% develop chronic renal failure; in RPGN associated with systemic diseases, as in idiopathic RPGN, aggressive therapy is required, cases of spontaneous recovery are not observed. In this regard, RPGN is considered as an urgent nephrological situation that requires rapid diagnostic and therapeutic measures to preserve kidney function. In the treatment of patients with a rapidly progressive course of nephritis, significant progress has been made in recent years: the 5-year "renal" survival rate has increased from 10 to 7
5%.
Systemic vasculitis is an important and, most importantly, treatable cause of rapidly progressive renal failure. GN is one of the characteristic manifestations of vasculitis affecting small vessels, Wegener's granulomatosis and microscopic
polyarteritis. So-called idiopathic crescentic GN is thought to be a special form of small vessel vasculitis limited only to the kidneys. Approximately 70% of patients with active vasculitis have specific antibodies directed to the neutrophil cytoplasm. When these antibodies are detected using the method of indirect immunofluorescence, they give two main types of luminescence - cytoplasmic and perinuclear. Most patients with Wegener's granulomatosis have a cytoplasmic type, while the perinuclear type predominates in patients with microscopic polyarteritis and idiopathic RPGN.
Systemic lupus erythematosus (SLE). When observing patients with RPGN, especially young women, one should always think about the possible presence of SLE, the clinical signs of which may be erased during the development of this form of nephritis. Lupus RPGN was previously the leading cause of death in SLE patients. However, with the development of methods of immunosuppressive therapy, the use of dialysis and transplantation, the situation has changed in recent years, it is possible to save the life of 50-80% of patients.
The morphological basis of RPGN is more often extracapillary GN (nephritis with crescents).
In typical cases, light microscopy of a renal biopsy reveals active necrotizing GN with extracapillary cell proliferation of the Shumlyansky-Bowman capsule - crescents consisting of cell layers and connective tissue, which are present in 10 - 70% of the glomeruli.
Based on the results of the biopsy immunofluorescence study, RPGN is divided into: 1
) anti-GBM nephritis (linear glow along the basement membrane of the glomerulus - GBM; simultaneous involvement of the lungs forms Goodpasture's syndrome); 2) "weakly immune" GN - nephritis with no significant deposits of immune deposits in the glomeruli, usually as a manifestation of vasculitis) and 3) immunocomplex GN (granular glow of deposits in the glomeruli).
Anti-GBM nephritis (and Goodpasture's syndrome) is a rare kidney disease that causes rapid, irreversible damage to the kidneys. Clinically manifested by RPGN, often in combination with pulmonary hemorrhage, which is caused by antibodies to the basement membrane of the renal glomerulus. These antibodies cross-react with the basement membranes of the alveoli of the lungs, causing pulmonary bleeding (hemorrhagic alveolitis), especially in smokers. Histological picture: GN with crescents in most of the glomeruli and a linear glow of antibodies along the BMC. Antibodies to BMK can also be detected in the circulation. Untreated patients quickly die either from pulmonary hemorrhage or from kidney failure. However, in recent years, the prognosis has improved after the introduction of plasmapheresis in combination with immunosuppression.
The glomeruli show focal and segmental necrotizing GN, often with crescents, but with little or no immune complex deposits.

PAGE OF THE CHIEF NEPHROLOGIST

^BE 1561-6274. Nephrology. 2011. Volume 15. No. 1

UDC 616.61-082

Dear friends and colleagues!

I consider it necessary to congratulate all of us, our professional community on the first small victory (achievement) - the restoration of the position of the chief specialist of the MHSD in nephrology after a long break (Order of the MHSD dated 19.10.10). It was a complex work at different levels of government and healthcare management, in which many of us took part (Public forum in Kolomna, hearings at the Commission on Health and Social Policy of the Federation Council, discussion of the problem in the MHSD, discussion in the press). But this is just the first step, giving a chance to create a unified system of nephrological care within the framework of the healthcare reform being carried out in Russia. In order not to miss your chance, it is necessary from the very beginning to unite the efforts of all three nephrological societies.

From the available data and the opinions of fellow experts emerge (one can imagine) the scope and depth of the problem we are tackling. Despite a certain development of the dialysis service and a slow increase in the number of renal replacement therapy sites (according to the RDO in 2008 - 142 per 1 million population, which is 5 times less than in Europe, 10 times less than in the USA and 20 times less than in Japan ), the “conservative” nephrological care is declining (decreasing) - departments are closing, the number of beds is decreasing, there is no increase in the number of specialists.

One of the reasons is the lack of official statistics on the prevalence and mortality from kidney diseases, hiding the true extent of the problem from legislators and healthcare organizers - creating a false impression of the low significance of renal pathology compared to the leading one in the existing healthcare model - cardiovascular. Meanwhile, in the United States, "nephritis" was the 9th cause of death in 2000, with the overall prevalence of chronic kidney disease (GFR< 60 мл/мин с высоким риском сердечнососудистых катастроф) - 10% (что соответственно в России может составить до 14 млн человек). Отсутствуют также точные данные о материальных и кадровых ресурсах нефрологичес-кой службы - количестве и видах ЛПУ, оказывающих нефрологическую помощь, числе врачей-нефрологов.

The first important stage in the reconstruction of a unified nephrological service in Russia was the Working Meeting within the framework of the VII Congress of the NORR (21.10.10), in the Resolution of which priority measures were outlined.

The Order of the MHSD on the "Procedure for the provision of nephrological care ..." was developed and posted on the website of the MOHSP for analysis and discussion. Next in line is the development and approval of "Standards" and "National recommendations" on the most important problems of nephrology, "Working groups" from among the leading experts will be created to prepare each of the documents. After public discussion and revision, these documents will be approved at conferences, plenums or congresses of our scientific professional Societies.

Chief Freelance Specialist Nephrologist of the Ministry of Health and Social Development of the Russian Federation, Head of the Department of Nephrology and State Duma of the First Moscow State Medical University

Professor E.M. Shilov

ISSN 1561-6274. Nephrology. 2011. Volume 15. No. 1.

By order of the Minister of Health and Social Development T.A. Golikova on October 19, 2010 Evgeniy Mikhailovich Shilov was appointed Chief Nephrologist of the Ministry of Health and Social Development of the Russian Federation.

Shilov E.M. (born October 22, 1949, Moscow), Doctor of Medical Sciences (1994), Professor (2002), Head of the Department of Nephrology and Hemodialysis, FPPOV, First Moscow State Medical University. THEM. Sechenov (2001), Vice-President of the Scientific Society of Nephrologists of Russia (2010). Student E.M. Tareeva, I.E. Tareeva.

In 1973 he graduated from the medical faculty of the 1st MMI. THEM. Sechenov. Since that time, he has been working at the I MMI (MMA, First Moscow State Medical University) named after. THEM. Sechenova: resident of the Clinic of Nephrology, Internal and Occupational Diseases (1973-1975), postgraduate student of the academic group of Academician of the Russian Academy of Medical Sciences E.M. Tareeva (1975-1978), junior (1978-1981), senior (1981-1995), leading (1995-2001) researcher of the laboratory, and then of the Department of Nephrology of the MMA Research Center and at the same time (1995 -2001) Professor of the Department of Nephrology. Since 2001 - head of the department of non-

phrology and hemodialysis of the faculty of postgraduate professional education of MMA doctors.

EAT. Shilov is one of the pioneers of domestic experimental immunonephrology, the author of studies devoted to the study of the mechanisms of development of immunoinflammatory and vascular diseases of the kidneys, the development, justification and evaluation of the clinical effectiveness of programs for immunosuppressive therapy of glomerulonephritis, including in systemic diseases (systemic lupus erythematosus, vasculitis). In 1994 he defended his doctoral thesis "Immunosuppressive therapy of active forms of nephritis (clinical and experimental study)", in which, based on the identified differences in the effect of glucocorticosteroids and cyclophosphamide (CFA) on the processes of collagen deposition in the kidney tissue, he experimentally substantiated the therapy ultra-high doses of CFA, which is still widely used in all nephrological centers of our country.

Today, under the guidance of E.M. Shilov at the Department of Nephrology of the First Moscow State Medical University, new scientific directions have been formed: the study of various aspects (including genetic ones), vascular nephropathies, primarily thrombotic microangiopathies, the study of the characteristics of kidney damage in metabolic syndrome, epidemiological studies in the field of chronic kidney disease. EAT. Shilov is the author of more than 200 scientific papers, including 2 monographs and 3 manuals on nephrology. Under the editorship of Evgeny Mikhailovich, for the first time in domestic practice, the textbook "Nephrology" was published for the system of postgraduate professional training of doctors. Under the leadership of E.M. Shilov defended 3 doctoral and 15 master's theses.

EAT. Shilov was a member of the Presidium of the Scientific Society of Nephrologists of Russia, and in 2010 he was elected vice-president of the NONR; member of the Coordinating Council of the Russian Dialysis Society (RDS), is a member of the editorial boards of the journals Nephrology and Dialysis, Nephrology, Clinical Nephrology.

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