CHRONIC GLOMERULONEPHRITIS

Chronic glomerulonephritis - a group of heterogeneous primary glome-rulopaty,

characterized by progressive inflammatory sclerotic and destructive changes with
outcome in chronic renal failure.

Chronic glomerulonephritis is diagnosed at any age, it may be a consequence of

untreated acute nephritis, but more often develops as a primary chronic disease.

CLASSIFICATION

In pediatric practice, traditionally distinguished gematuricheskuyu, oil-roticheskuyu

and mixed forms of chronic glomerulonephritis. When the diagnosis is necessary to
clarify the morphological variant of the disease. In accordance with the morphological
classification of chronic glomerulonephritis distinguish the following forms.

Minimal changes of glomeruli.

Focal segmental glomerular sclerosis (hyalinosis).

Membranous glomerulonephritis (membranous nephropathy).

Mezangioproliferativny glomerulonephritis.

Mezangiokapillyarny (membranoproliferativny glomerulonephritis).

Fibroplastichesky glomerulonephritis (finals of all types).

ETIOLOGY

The cause of the disease can not be established in all cases. Nefritogennyh suggest the
role of streptococcal strains, persistent viruses (hepatitis B, cytomegalovirus,
Coxsackie virus, Epstein-Barr virus, influenza, etc.), genetically determined
characteristics of immunity (eg, a defect in the complement system or cellular
immunity ).Emergence of the disease contribute to congenital renal dysplasia.

PATHOGENESIS

Excrete immunologically immunologically determined and not due to variations of the

disease. Immunologically caused immunocomplex may be, rarely autoantitelnogo
genesis. Most forms of primary chronic glomerulonephritis attributed to
immunocomplex glomerulopathy. Their pathogenesis resembles that of the glomeruli
in acute-nephritis (see above section "Acute glomerulonephritis"). The participation of
immunological processes in glomerulonephritis with minimal changes, the subject of
debate.Despite the fact that many patients the disease can be regarded as an allergic
reaction to vaccines, medications and other factors, the study of glomeruli
immunogistohi-chemical method does not reveal specific changes. Using electron
microscopy reveal a reduction of small appendages podotsi-ing, indicating metabolic
changes in cells.Pathology podocyte leads to disruption of the integrity of glomerular
filter. As a result, proteins and lipids in excessive quantities penetrate into the primary
urine and reabsorbiruyas accumulate in the tubular epithelium. Develops protein and
fatty degeneration of tubular cells, clearly visible under light microscopy, that in
former times has led the call the pathological process of "lipoid nephrosis".

Clinical picture

The clinical picture of chronic glomerulonephritis depends on it (rormy.

Nephrotic form

Children from 1 to 5 years nephrotic form of chronic glomerulonephritis usually

corresponds to a morphological variant with minimal changes in the
glomeruli. Discuss the possibility of moving the minimum changes of glomeruli in
focal segmental glomerulosclerosis, as the major change in the latter - also defeat
podocyte. For nephrotic form of chronic glomerulonephritis characterized by massive
proteinuria (more than 3 g / day), edema, hypo-and Dysproteinemia, hyper-
lipidemia. When nephritis with minimal change nephrotic syndrome is not
accompanied by either hematuria, or arterial hypertension. That is why he wears such
names as "pure" or "idiopatiches-ski" or "primary" nephrotic syndrome. In this
proteinuria may reach 20-30 g / day or more, but it is almost always selective and
represented mainly albumins . Significant loss of protein in the urine leads to
hypoproteinemia and dysproteinemia (mostly hypo-albuminemia), and this in turn
determines the decrease in plasma oncotic pressure, resulting in water passes into the
tissues. CBV diminishes, decreases glomerular filtration, appear edema (mostly
diffuse, with dropsy of the cavities). Edema give patients a characteristic
appearance.The skin is white, cold to the touch. Patients feel thirst, dry mouth,
weakness. Due to accumulation of fluid in the pleural cavity may be complaints of
cough and shortness of breath. Appears tachycardia, in the absence of ascites reveal
enlargement of the liver. Along with the development of hypoalbuminemia increases
the concentration of lipids in the blood serum. Number of urinating and urine volume
decreased, but the relative density of urine is increased. Precipitate meager and mostly
contains cylinders, grease and zhiropererozhdenny epithelium. Erythrocytes identify
rare and very short time. Gross hematuria does not happen. ESR has increased
dramatically. During the exacerbation of IgE, or IgM and fibrinogen might be
improved. The concentration of complement component NW is always
normal. Weather favorable. The majority of children recover.

In school-age children with nephrotic form of chronic membranous

glomerulonephritis is commonly found mezangioprolifera and have ready
morphological variants. For membranous variant is characterized by the presence of
subepithelial deposits and thickening of glomerular basement membrane in the
absence of significant proliferation of endothelial and mesangial
cells. Mezangioproliferativny variant is less common. It is characterized by
proliferation of mesangial cells and matrix. The disease can develop gradually with
the appearance of proteinuria and progressive growth of edema. Possible and rapid
start with nephrotic syndrome. Proteinuria poor selectivity, indicating that a heavy
defeat on the glomerular capillaries. Hematuria is expressed in varying degrees - from
the micro to gross hematuria. Gipokomplementemiyu exhibit - the index of activity of
pathological process. The course of the disease undulating, azo-tovydelitelnaya long-
term renal function remains intact, but half of patients after 5-10 years of jade leads to
the development of CRF. Remission of nephrotic variant more frequently noted in
patients with membranous glome-rulonefritom.

Gematuricheskaya form

The main manifestation of this form - persistent hematuria. Possible slight proteinuria
and anemia. The state of health of patients is usually not disturbed. In some cases,
mark pastoznost century. Morphologically this mezangioproliferativny
glomerulonephritis (a version with the deposition in the glomeruli of IgA and
complement component E).

One option gematuricheskoy forms of chronic glomerulonephritis - Berger's disease

(IgA-nephropathy). The disease is diagnosed in children of any age. The boys are sick
2 times more girls. Characterized by recurrent gross hematuria arising during ARI
accompanied by fever in the first days or even hours of illness ( "sinfaringitnaya
hematuria), at least - after other disease or vaccination. As a possible etiological factor
in discussing the role of gluten. Some patients exhibit elevated serum titers of IgA to
the AT-class food proteins gliadin. The course of IgA-nephropathy relatively
favorable. The majority of patients recover. The outcome of renal failure is
rare. Resistance to therapy noted in children with Berger's disease. Prognostically
unfavorable development of nephrotic syndrome and hypertension.

Mixed form

Develops in older children and has a heavy and steadily progressive course, torpid to
therapy. When mixed form of all possible morphological variants (except for minimal
changes). Most often reveal mezangiokapillyarny variant characterized by
proliferation of mesangial cells and thickening or dvukonturnostyu capillary walls due
to infiltration of mesangial cells. Progress-tion of the pathological process leads to the
development of sclerosis and the formation of fibroplasticheskogo glomerulonephritis
- the finale of most forms of chronic glomerulonephritis. Sclerosis developed capillary
loops glomerulus, formed fibroepitelialnye and fibrous crescents, sclerosis and
thickening of the capsule body.

The disease often begins with an acute nephrotic syndrome with sudden development
of hematuria, severe non-selective proteinuria, edema and persistent
hypertension. The disease can rapidly increasing renal insufficiency. The peculiarity
of the mixed form - gipokomplementemiya with reduced concentrations of NE and /
or C4 components of complement. Prognosis is poor quite quickly in children
developing chronic renal failure.

TREATMENT

At the present stage, consider the optimal treatment in the light of morphological
variants of chronic glomerulonephritis, but it is not always possible, especially in
children, and you have to focus on the clinical manifestations of disease. The
treatment prescribed depending on the activity of the pathological process. In the
active period of the disease in the presence of edema, gross hematuria and blood
pressure shows a pronounced increase bed rest. He expanded the fading of clinical
manifestations of pathological process. Diet prescribed given the state
azotovydelitelnoy kidney function. With its secure child gets protein in an amount
corresponding to the age norm (2-3 g / kg / day), with azotemia - 1 g / kg / day.
Pronounced hyperlipidemia need to reduce intake of fats. When edema and
hypertension appoint gipohloridnuyu diet. The volume of fluid dose out depending on
diuresis. However, under polyuria such restrictions are unnecessary and even harmful.

In view of the pathogenesis of glomerulonephritis used drugs that affect both the
immune mechanisms (glucocorticoids, cytotoxic agents), and in non-immune [anti-
coagulants (heparin at 200-400 U / kg / day for 2-4 weeks or more), antiagreganty
(dipyridamole and 3 -5 mg / kg), ACE inhibitors (enalapril, lisinopril)], as prescribed
lipid-lowering drugs [lovastatin, fluvastatin (leskol) and others].

When first mooted nephrotic syndrome with minimal change glomerular recommend
starting treatment with prednisolone at a dose of 2-3 mg / kg body weight or 60 mg /
m 2. The drug in this dose prescribed for 4-8 weeks. In nephrotic syndrome, sensitive
to hormonal therapy (most children), there comes a clinical and laboratory
remission.Relapse, which developed after the abolition of glucocorticoids suggests
hormonal dependence of nephrotic syndrome. Treatment of first relapse of conduct as
well as therapy in early disease. With frequent relapses adding cytotoxic agents -
cyclophosphamide 2 mg / kg chlorambucil (leykeran, hlorbutin) of 0,1-0,2 mg / kg,
while reducing the dose of prednisolone to 40 mg / m 2 and transferring the patient to
the alternating mode of administration of the drug (one day). Lack of effect of
hormone replacement therapy for 8 weeks indicate nephrotic syndrome resistant to
hormonal therapy. In this case, treatment is carried out selective cytostatics or
immunosuppressants cyclosporine at a dose of 3-5 mg / kg.

Patients with focal segmental glomerular sclerosis in the present long-term courses
(up to 6 months) designate glucocorticoids and cytotoxic agents, to achieve remission
in patients previously judged unsuitable.

In the most severe mixed form of chronic glomerulonephritis using 4-component

scheme of treatment (glucocorticoids, cytostatics + + + antiagreganty
anticoagulants). In the absence of the effect of this therapy sessions conducted with
plasmapheresis pulse therapy metilpred-nizolonom in a dose of 10-20 mg / kg and / or
cyclophosphamide for 5-15 mg / kg, followed by transfer of the patient to receive
immunosuppressive drugs inside.

When gematuricheskoy form of chronic glomerulonephritis (mesangium-

proliferative glomerulonephritis) and IgA-nephropathy in renal function, proteinuria, a
small (less than 1 g / day) with episodes of "syn-faringitnoy gross hematuria" can be
assigned to ACE inhibitors (with nephrotoxicity protective order) and dipyridamole
. In the case of nephrotic syndrome should be used glucocorticoids and cytostatics.
NSAIDs are widely used in earlier gematuricheskoy form of chronic
glomerulonephritis, is currently in connection with the side effects are rare.

When fibroplasticheskom variant of glomerulonephritis (pronounced sclerotic

changes in glomeruli), glucocorticoids are not shown.

PREVENTION

Prevention of chronic glomerulonephritis aimed at preventing relapses (restriction of

physical activity, elimination of stress, cooling, protection from intercurrent diseases,
readjustment of foci of chronic infection, Vaccine individual plan). Patients shown to
outpatient observation and monitoring of kidney function.

FORECAST

Forecast depends primarily on morphological features of jade. The most unfavorable

for the mixed form of chronic glomerulonephritis. Chronic renal insufficiency may
develop in time from several months to 15-20 years after onset. In end-stage renal
failure and hemodialysis are shown kidney transplantation.