Amyloid-lipoid nephrosis in children and adults: causes, electron microscopic and morphological features, diagnosis, treatment

Renal nephroses are common in medical practice quite often. These are dystrophic changes in the tissues of the organ associated with the degeneration of the renal tubules. Nephroses can be characterized by acute or chronic course. In the latter case, the nephrosis proceeds predominantly in the lipoid-amyloid form.

Amyloid-lipoid nephrosis

Lipoid-amyloid nephrosis refers to the dystrophy of the kidney tissues caused by the deposition of lipids and the pathological protein compound( amyloid) in the tubular structures. There is amyloid nephrosis and lipoid renal disease, but most often they occur in combination of amyloid-lipoid nephrosis.

Causes of

Lipoid-amyloid form of nephrosis is formed as a result of a number of pathological factors:

  • Lipid exchange disorders, which lead to an increase in the cholesterol level in the blood;
  • With long-term chronic pathologies such as rheumatoid arthritis, tuberculosis or syphilis;
  • In the presence of purulent foci of chronic infections
    such as osteomyelitis, pulmonary abscess or bronchoectatic disease.

Pathogenesis of

Lipoid nephrosis is formed gradually. The patient has an increase in the kidneys with an expansion of the cortical layer. Doctors often call this organ a white large kidney. The concentration of protein in the urine rises, which immediately leads to a drop in the blood. As a result, extensive puffiness is formed, but due to the absence of a lesion of the vascular renal system, the pressure remains normal.

Usually patients are forced to consult a specialist for complaints of swelling and weakness, frequent lack of appetite and chilliness, a constant thirst. Swelling also does not occur immediately. Initially, patients note blanching of the face and its slight puffiness, accompanied by a decrease in mimic functions.

Lipoid nephrosis or white large kidney

Pathogenesis and symptoms of amyloid-lipoid nephrosis

Puffiness gradually increases, which extends to the entire subcutaneous tissue. The patient begins to deform the face and body, although the maximum expressed puffiness is present on the face, which acquires a puffiness. Eyes narrow, eyelids swell, after inducing patients it is difficult to open eyes.

Surplus fluid accumulates in all intraorganic structures, in the pleura, pericardium, abdominal cavity. Such puffiness can last for several years.

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When the form of pathology is launched, there is a significant decrease in the volume of excreted urine with a characteristic increase in its density. Gradually, destruction and connective tissue replacement of the renal tubules occurs, which leads to atrophy of the glomeruli. As a result, the volume of the kidneys decreases, i.e., a nephrotic wrinkled organ is formed. Pathology can last up to 20 years, alternating with pre-inflammatory and edematous phases, when the patient becomes disabled.

Symptoms

Lipoid-amyloid nephrosis differs from the polyclinic nature of the course and is accompanied by:

  1. Extensive hypertension, but more often it is of a minor nature;
  2. Presence of epithelial, waxy and hyaline cylinders;
  3. Amyloid-lipoid lesions of other inorganic structures such as the spleen or liver;
  4. Proteinuria;Deficiency of protein in the blood;
  5. Absence of arterial hypertension and normal indicators of concentration renal function.

In the urine can be found epithelial kidney cells, white blood cells, molds of the kidney tubules, hyaline and modified epithelial cells.

Nephrotic syndrome develops, manifested by proteinuria and mass fluid retention. The patient is concerned about severe weakness, lumbar gravity, dry mouth and headaches, diarrhea and flatulence. Often patients complain of shortness of breath and stiffness caused by swelling.

Electron microscopic sign of pathology - swelling and subsequent decay of mitochondria, destruction of membranes. Morphological signs of lipid nephrosis at first practically do not run.

Micropreparation in lipid nephrosis

Electron-microscopic and morphological signs of lipid nephrosis

Diagnosis

Amyloid-lipoid renal nephrosis can not be detected at an early stage, especially in children, because its course is characterized by a weak clinical symptomatology. Patients often lack traditional symptoms of kidney lesions, such as pain and discomfort in the lumbar region.

Treatment of

The main goal of therapy for amyloid-lipoid nephrosis is the elimination of the underlying pathological process that caused nephrotic lesions, for example, syphilis, lipid exchange disorders, tuberculosis, etc. If there are hyperthers, then mandatory bed rest is indicated.

Patients in serious condition are subject to careful nursing care, including the control of skin surfaces, to avoid pressure sores and infectious complications, because hyperthermia contributes to their occurrence. When the edema is too extensive, diuretic therapy is shown, often supplemented with the intake of hormonal drugs in order to restore metabolic processes. Such appointments are necessary because of violations of thyroid activity.

Treatment for amyloid-lipoid nephrosis is based on an integrated approach.

In addition to pathogenetic and etiotropic therapy, strict adherence to a dietary diet is prescribed, which is based on the following principles:

  • The diet should be enriched with vitamin and protein-containing foods;
  • The drinking regime must be limited;
  • Food is better to use unsalted, because damaged kidney structures are not able to remove salt excess from the body, as a result, the fluid is even more delayed and causes the appearance of extensive swelling;
  • The diet also requires the exclusion of cholesterol products like animal fats and eggs.

An important therapeutic role is also assigned to physiotherapeutic treatment, which involves the appointment of sweatshops. If swelling does not give in to any effect, then subcutaneous tissue tissues are installed special drainage needles, through which the edematous fluid is eliminated. Needles can be installed for several days, which indicates a severe form of edema. It is important in these days to observe the maximum hygiene, antiseptic and aseptic norms in order to exclude secondary infectious lesions.

Positive effect on the treatment of water and hot air baths, which contribute to increased sweating. Some patients, if possible, are given climatotherapy, presuming to stay in regions with a warm and dry climate.

Forecasts

If the pathology progresses a long time, then the possibility of developing the wrinkling of the kidney and its insufficiency is high. If a secondary infection is attached to the background of the disease, the risk of death for the patient is maximized.
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