Chronic glomerulonephritis is a disease, usually with a progressive course leading to the development of chronic glomerulonephritis in most morphological variants already in childhood. In pediatric nephrology, chronic glomerulonephritis ranks second in the structure of the causes of chronic renal failure after a group of congenital and hereditary nephropathies.
The course of chronic glomerulonephritis in children can be recurrent, persistent and progressive. The recurrent course is characterized by drug or spontaneous remissions of varying duration. A persistent variant is characterized by a continuous activity of the process with a preserved function of the kidneys in the early stages. However, after several years, the outcome of CRF occurs. With a progressive course, the faster development of chronic glomerulonephritis in children is noted - in 2-5 years from the onset of the disease. The prognosis of chronic glomerulonephritis depends on the clinical and morphological variant and on timely adequate therapy.
Nephrotic form of chronic glomerulonephritis or idiopathic nephrotic syndrome.
Ill children aged from 3 to 7 years old, in the anamnesis - a previously acute acute first episode. Subsequent exacerbations with proper monitoring of patients are rarely accompanied by significant edema. Exacerbation of the process usually (60-70%) develops after intercurrent diseases (ARI, childhood infections) or against the background of complete health (30-40%). Parents note a small pastosity of the eyelids. In the analysis of urine, an increase in the protein content is determined. In rare cases, with bad observation, pronounced edema is formed. At laboratory researches find all deviations, typical for НС.
The most frequent (85-90%) morphological variant is the disease of minimal changes (NSME). This term is due to the fact that with light microscopy, the glomeruli are not changed. With electron microscopy, one can see the "melting" of the small legs of podocytes. Most patients in this group report a high sensitivity to glucocorticoid therapy. Focal-segmental glomerulosclerosis (FSGS) occurs less frequently (10-15%). With light microscopy, the glomeruli look unchanged or a small proliferation of mesangial cells is seen. Electron microscopy reveals a thickening of the podocytes, but a characteristic feature is the presence of segmental mesangial sclerosis in the part of the glomeruli. There is also atrophy of tubular epithelium, infiltration and fibrosis interstitium.
For the clinical picture of the disease in FSHS characterized by adherence to nephrotic syndrome of hypertension and hematuria, as well as the development of hormone resistance. In this case it is necessary to carry out a biopsy of the kidneys to clarify the morphological picture of the disease in order to decide the question of the tactics of further treatment.
There are several variants of the course of chronic glomerulonephritis with nephritic syndrome:
- often recurrent course (at least 4 relapses per year or 2 relapses in 6 months);
- rarely recurrent course (less than two relapses in 6 months). The prognosis in most patients with NSME is favorable.
Relapses of HC are reduced after 5 years from the onset of the disease. Progression of the disease in the main group with NSMI does not occur, the kidney function is not impaired. A small group may continue to recurrence of exacerbations, usually manifested only by proteinuria. If the NS is represented by a morphological variant - FSSS, the forecast is unfavorable. The majority of patients are resistant to therapy, they have a gradual decrease in kidney function, progression of hypertension and development of CRF for 1-20 years.
The hematuric form of chronic glomerulonephritis in children
The hematuric form of chronic glomerulonephritis is characterized by recurrent isolated (without edema and AH) macro- or microhematuria in combination with proteinuria less than 1 g / day or without it, occurring 2-5 days after acute respiratory tract disease. Most often, the disease is based on a peculiar form of GB, which was called Berger's disease, or IgA-nephropathy. Currently, Berger's disease is considered one of the most common hematuric glomerulopathies in many countries around the world. Almost half of the children with hemaguric form of glomerulonephritis are diagnosed with Berger's disease. The disease is more common in boys and children older than 10 years.
The etiology is associated with viral or bacterial infection of the upper respiratory tract, as well as with the carriage of HB5 antigen. However, more often the cause remains unsettled.
In the pathogenesis of the hematuric form, the main role is played by immunocomplex mechanisms. If the composition of the IC is IgA, then this option is called IgA-nephropathy, or Berger's disease.
Morphologically it is mesangioproliferative glomerulonephritis, which is characterized by the proliferation of mesangial cells, the expansion of mesangial matrix, the deposition of IC in mesangium and subendothelium.
Clinically, the following variants of the course are distinguished:
- recurrent macrohematuria, which provokes ARVI. The duration of episodes of macromeduction can range from a few weeks to several years. Between the episodes, urinalysis can remain normal;
- the only episode of macrohematuria with the subsequent persistence of microhematuria.
The course of the disease is recurrent or persistent with slow progression. The prognosis worsens with the adherence of arterial hypertension and nephrotic syndrome.
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Mixed form of chronic glomerulonephritis
A relatively rare disease in children is more common in adolescents. The onset of the disease is associated with a previous viral infection, the persistence of the hepatitis B virus. However, in most cases the cause of the onset and exacerbations remains unknown.
The most frequent morphological variant is membrane-proliferative (mesangiocapillary) glomerulonephritis. The morphological picture is characterized by diffuse mesangial proliferation and an increase in the mesangial matrix with its interposition between the basal membrane of the glomerulus and endothelial cells, which leads to thickening and two-contouring of the basal membranes.
Symptoms of chronic glomerulonephritis in children are manifested by a combination of nephrotic syndrome with hematuria and / or hypertension. The diagnosis is confirmed by kidney biopsy.
The course of the disease is progressive with persistent AH, severe persistent proteinuria and early impairment of renal function with development of CRF for 10 years from the onset of the disease. In some cases, it is possible to achieve clinical remission. Relapse of the disease can develop even in a transplanted kidney.