The basic elements of diagnosis
- Generalized edema.
- Proteinuria greater than 3.5 gm per day.
- Hypoalbuminemia less than 3 gm/100 ml.
- Hyperlipidemia: Cholesterol greater than 300 mg/100 ml.
- Lipidurija: fat free, oval fat bodies, fatty casts.
General considerations
Renal tissue obtained by biopsy or necropsy, showing the changes that are characteristic of the underlying disease, for example. of disseminated lupus erythematosus, amyloidosis, diabetic nephropathy. However, in many cases, the development of nephrotic syndrome is not attributable to a specific disease. These idiopathic nephrosis are classified according to the nature of glomerular lesions.
1 Minimal glomerular lesions: (about 20% of cases of idiopathic nephrosis). An ordinary microscope can not see the pathological disorders. Electron microscope, the Mõigu videtd change glomerular basement membrane. These characters are swelling, vacuolization and loss of the prstastih extensions epitclnih cells called lipoic or pure nephrosis, disease Earleovih epithelial cells, disease prstastih (nežičastih) extensions.
2 Membranous type of the disease: (about 70% of cases of idiopathic nephrosis). Under an ordinary microscope shows thickening of the basement membrane. Under the electron microscope, in addition to thickening of the basement membrane shows distortion, blunt ost and connect prstastih (nežičastih) extensions of epithelial cells.
3 Proliferative lesions: (about 5% of cases of idiopathic nephrosis). An ordinary microscope can quickly identify the increase in the number of epithelial cells and the formation of polumesečastih Bowmanovoj thickening in the capsule, glomerular capillary turning into the scar tissue of different degrees.
4 Meat vite membranousand proliferative lesions: (make up about 5% of cases of idiopathic nephrosis).
Clinical Features
A. Symptoms and signs: edema can occur insidiously and slowly increasing. Often, however, appears suddenly and builds up quickly. If the fluid builds up in scroznim cavity, the abdomen is enlarged and the patient may have problems in terms of anorexia, and breathlessness. Other symptoms that are related to the mechanical effects of edema in serous cavities are not significant.
At physical examination revealed signs of generalized edema. There are often signs hiđrotoraksa and ascites. Edema emphasizes pale skin color, and stretch marks often appear on the skin of the extremities istegnutoj. Hypertension, changes in the retina and its blood vessels and cardiac and cerebral manifestations of hypertension are much more pronounced in the presence of collagen disease, diabetes mellitus or renal insufficiency.
B. Laboratory findings: urine contains a large amount of protein, 1-10 gm/24 hours or more. Sediment containing cylinders, including oily and waxy types, renal tubular cells, some of which contain fat droplets (oval fat bodies) and erythrocytes in varying numbers. The road is normochromic anemia light degree, but anemia can be much more difficult if the kidney damage higher. Nitrogen retention varies to the degree of renal function. Plasma is often lipemic and cholesterol in the blood is usually very high. Protein levels were really low. Albumin fraction may fall below 2 gm or even below 1 gm/100 ml. In pure nephrosis there is some reduction of gamma globulin, while on the contrary in systemic lupus erythematosus, they can be very elevated. Electrolyte concentration in the serum is often normal, although serum sodium can be easily reduced. Total kalcijuni levels may be low, according to the degree of hypoalbuminemia, which leads to a reduction in the amount of calcium bound to proteins. Sodium little is excreted in the urine, and urinary excretion of aldosterone is increased. If there is renal failure, blood tests and urine characterizes the change (see renal insufficiency). To confirm the diagnosis and prognosis of disease, renal biopsy is essential.
Differential Diagnosis
Nephrotic syndrome (nephrosis), may occur in different renal diseases, including glomerulonephritis, (membranous and proliferative), collagen diseases (disseminated lupus erythematosus, poliartervtis iLd.), Amyloidosis, renarnih vein thrombosis, diabetic nephropathy, syphilis, and reactions to toxins such as poisons, Rhus antigen drugs like trimetadion (Tridion), and heavy metals. In young children nephrosis can occur without clear evidence of any cause.
Treatment
There is no specific treatment for syphilis except for heavy metal poisoning. Patients with very izražeruim edema or infection needs to rest in bed. The infection should be treated quickly and effectively with appropriate antibiotics. If a patient receiving corticosteroids, it is desirable to hospitalization. Diet should contain the normal protein ratio (0.75 to 1.0 gm / kg per day) and an adequate amount of calories. Potassium intake should not be restricted.
Experience in the past ten years, has confirmed the value adrenokortikosteroidnih hormone in the treatment of nephrotic syndrome in children and adults whose basic disease causes minimal glomerular lesions (lipoic nephrosis) in the system lupuseritematozusa, glomerulonephritis, or idiosyncrasies of toxins and poisons. Corticosteroid therapy is less effective in the case of membrane disease, proliferative lesions, or mixed lesions in the glomerulus. This therapy has no value or is of little value in amyloidosis, renal vein thrombosis, is contraindicated in diabetic nephropathy.
The goal of therapy is to:
1 cause diuresis,
2 to create no urine protein,
3 to raise albumin levels to normal levels
4 to reduce lipidemija to normal values.
With increasing experience, there was a tendency that the treatment is for a long time and during periods of remission.
Although prednisone is widely used, can be used and other corticosteroids in equivalent doses.Prednisone is given 1-2 mg / kg per day for children or 80-120 mg per day for adults to underestimate the doses, orally for 10 days 3-4 weeks. If urine output is established early in the course of treatment, the dose of corticosteroids may be slightly reduced after a period of 3-4 weeks, trying to find the minimum dose that will achieve the therapeutic goal and maintain remission. When there is no response to corticosteroid therapy, rapid reduction in intake of corticosteroids may result in diuresis and improvement. In case you did not report improvement after stopping corticosteroid therapy should attempt to further treatment with high daily doses of this medication before a definite conclusion that the patient is refractory to corticosteroids.
When you establish a good diuresis and to reduce proteinuria and edema, corticosteroids may be administered intermittently every other day. This intermittent therapy can satisfactorily maintain disease remission. The total daily dose of continuous, daily allowances, given as a single dosage at breakfast every other day. This method of treatment usually does not lead to suppression of the adrenal gland, normal growth can be expected in children, and cushingoid changes and hypertension are rare. In some cases, the introduction of intermittent therapy, taking medication every 48 hours, yielded good results.
Another way to intermittent therapy, which are widely used, consists in giving prednisone 60 mg orally in divided doses for three consecutive days in a week, which follows četvorođnevna break.Unsuitability of this therapeutic schemes have a number of adverse effects of high doses of corticosteroids, with signs of adrenal suppression, which manifests itself in vrcme četvorodnevnc break.
For now, it is considered justified to intermittent therapy apply for one year, if the patient remains free of edema and proteinuria reduced to a negligible amount. If a minor exacerbations, therapy can be increased. Compensation potassium is preferred during corticosteroid therapy, although it may be unnecessary for administering corticosteroids every other day.
Diuretics are often ineffective. The most useful are derivatives of chlorothiazide, for example.hydrochlorothiazide, 50 mg - 100 mg every 12 hours. Other products chlorothiazide, chlorthalidone or other diuretics, can also be used in the respective effective doses. Aldosterone antagonists may help you. Together with the use of thiazides. Salt-free albumin, dextran, and other onkotični agents are of little help, and their effect in causing a transient diuresis.
Attention: Increase in serum potassium, the development of hypertension, a sudden strong increase in edema, a contraindication for further continuation of corticosteroid therapy. These complications usually occur during the first two weeks of continuous treatment.
Immunosuppressive drugs such as cyclophosphamide, mercaptopurine, azathioprine (Imuran) and others are currently attempting to treat nephrotic syndrome. The use of corticosteroids in the community with similar immunosuppressive agents used to prevent rejection in homotransplatata man. The experience is still scarce but the results of treatment of this type of therapy encouraging.This type of therapy leads to improvement in children and adults with proliferative, membranous, and mixed lesions with systemic lupus erythematosus. In patients with minimal lesions that are refractory to corticosteroids, giving, giving immune suppressive agents did not improve. Glomerular changes in renal function and in many cases have responded well to this treatment. The frequency of improvement was not found.
During the treatment, it is often plagued by serious side effects, which apply both to corticosteroids and cytotoxic agents. For now, this form of therapy should be used only in patients who are refractory to conventional treatment regimen, and by physicians who are experienced in the treatment of nephrotic syndrome.
Forecast
The course and prognosis depend on the underlying disease that is responsible for the occurrence of nephrotic syndrome in about 50% of nephrosis in childhood, the disease appears suddenly, and doing a benign course in the case of appropriate treatment and leaves a slight squeal. However, many patients are going to relentlessly terminal condition with renal insufficiency. Adult patients with nephrosis go lower, especially when the primary disease is glomerulonephritis, systemic lupus erythematosus, amyloidosis, renal vein thrombosis and diabetic nephropathy. In patients with minimal lesions or spontaneous remission or after corticosteroid therapy is common.
Treatment is much more frequent failures or only leads to 'improvement when there are other glomerular lesions. Hypertension and nitrogen retention are poor prognostic signs.
Of the authors:
Dr. Henry Brainerd, professor of medicine
Dr. Marcus A. Krupp, Professor of Medicine
Dr. Milton J. Chatton, Professor of Medicine
Dr. Sheldon Margen, professor of medicine
- Generalized edema.
- Proteinuria greater than 3.5 gm per day.
- Hypoalbuminemia less than 3 gm/100 ml.
- Hyperlipidemia: Cholesterol greater than 300 mg/100 ml.
- Lipidurija: fat free, oval fat bodies, fatty casts.
General considerations
Renal tissue obtained by biopsy or necropsy, showing the changes that are characteristic of the underlying disease, for example. of disseminated lupus erythematosus, amyloidosis, diabetic nephropathy. However, in many cases, the development of nephrotic syndrome is not attributable to a specific disease. These idiopathic nephrosis are classified according to the nature of glomerular lesions.
1 Minimal glomerular lesions: (about 20% of cases of idiopathic nephrosis). An ordinary microscope can not see the pathological disorders. Electron microscope, the Mõigu videtd change glomerular basement membrane. These characters are swelling, vacuolization and loss of the prstastih extensions epitclnih cells called lipoic or pure nephrosis, disease Earleovih epithelial cells, disease prstastih (nežičastih) extensions.
2 Membranous type of the disease: (about 70% of cases of idiopathic nephrosis). Under an ordinary microscope shows thickening of the basement membrane. Under the electron microscope, in addition to thickening of the basement membrane shows distortion, blunt ost and connect prstastih (nežičastih) extensions of epithelial cells.
3 Proliferative lesions: (about 5% of cases of idiopathic nephrosis). An ordinary microscope can quickly identify the increase in the number of epithelial cells and the formation of polumesečastih Bowmanovoj thickening in the capsule, glomerular capillary turning into the scar tissue of different degrees.
4 Meat vite membranousand proliferative lesions: (make up about 5% of cases of idiopathic nephrosis).
Clinical Features
A. Symptoms and signs: edema can occur insidiously and slowly increasing. Often, however, appears suddenly and builds up quickly. If the fluid builds up in scroznim cavity, the abdomen is enlarged and the patient may have problems in terms of anorexia, and breathlessness. Other symptoms that are related to the mechanical effects of edema in serous cavities are not significant.
At physical examination revealed signs of generalized edema. There are often signs hiđrotoraksa and ascites. Edema emphasizes pale skin color, and stretch marks often appear on the skin of the extremities istegnutoj. Hypertension, changes in the retina and its blood vessels and cardiac and cerebral manifestations of hypertension are much more pronounced in the presence of collagen disease, diabetes mellitus or renal insufficiency.
B. Laboratory findings: urine contains a large amount of protein, 1-10 gm/24 hours or more. Sediment containing cylinders, including oily and waxy types, renal tubular cells, some of which contain fat droplets (oval fat bodies) and erythrocytes in varying numbers. The road is normochromic anemia light degree, but anemia can be much more difficult if the kidney damage higher. Nitrogen retention varies to the degree of renal function. Plasma is often lipemic and cholesterol in the blood is usually very high. Protein levels were really low. Albumin fraction may fall below 2 gm or even below 1 gm/100 ml. In pure nephrosis there is some reduction of gamma globulin, while on the contrary in systemic lupus erythematosus, they can be very elevated. Electrolyte concentration in the serum is often normal, although serum sodium can be easily reduced. Total kalcijuni levels may be low, according to the degree of hypoalbuminemia, which leads to a reduction in the amount of calcium bound to proteins. Sodium little is excreted in the urine, and urinary excretion of aldosterone is increased. If there is renal failure, blood tests and urine characterizes the change (see renal insufficiency). To confirm the diagnosis and prognosis of disease, renal biopsy is essential.
Differential Diagnosis
Nephrotic syndrome (nephrosis), may occur in different renal diseases, including glomerulonephritis, (membranous and proliferative), collagen diseases (disseminated lupus erythematosus, poliartervtis iLd.), Amyloidosis, renarnih vein thrombosis, diabetic nephropathy, syphilis, and reactions to toxins such as poisons, Rhus antigen drugs like trimetadion (Tridion), and heavy metals. In young children nephrosis can occur without clear evidence of any cause.
Treatment
There is no specific treatment for syphilis except for heavy metal poisoning. Patients with very izražeruim edema or infection needs to rest in bed. The infection should be treated quickly and effectively with appropriate antibiotics. If a patient receiving corticosteroids, it is desirable to hospitalization. Diet should contain the normal protein ratio (0.75 to 1.0 gm / kg per day) and an adequate amount of calories. Potassium intake should not be restricted.
Experience in the past ten years, has confirmed the value adrenokortikosteroidnih hormone in the treatment of nephrotic syndrome in children and adults whose basic disease causes minimal glomerular lesions (lipoic nephrosis) in the system lupuseritematozusa, glomerulonephritis, or idiosyncrasies of toxins and poisons. Corticosteroid therapy is less effective in the case of membrane disease, proliferative lesions, or mixed lesions in the glomerulus. This therapy has no value or is of little value in amyloidosis, renal vein thrombosis, is contraindicated in diabetic nephropathy.
The goal of therapy is to:
1 cause diuresis,
2 to create no urine protein,
3 to raise albumin levels to normal levels
4 to reduce lipidemija to normal values.
With increasing experience, there was a tendency that the treatment is for a long time and during periods of remission.
Although prednisone is widely used, can be used and other corticosteroids in equivalent doses.Prednisone is given 1-2 mg / kg per day for children or 80-120 mg per day for adults to underestimate the doses, orally for 10 days 3-4 weeks. If urine output is established early in the course of treatment, the dose of corticosteroids may be slightly reduced after a period of 3-4 weeks, trying to find the minimum dose that will achieve the therapeutic goal and maintain remission. When there is no response to corticosteroid therapy, rapid reduction in intake of corticosteroids may result in diuresis and improvement. In case you did not report improvement after stopping corticosteroid therapy should attempt to further treatment with high daily doses of this medication before a definite conclusion that the patient is refractory to corticosteroids.
When you establish a good diuresis and to reduce proteinuria and edema, corticosteroids may be administered intermittently every other day. This intermittent therapy can satisfactorily maintain disease remission. The total daily dose of continuous, daily allowances, given as a single dosage at breakfast every other day. This method of treatment usually does not lead to suppression of the adrenal gland, normal growth can be expected in children, and cushingoid changes and hypertension are rare. In some cases, the introduction of intermittent therapy, taking medication every 48 hours, yielded good results.
Another way to intermittent therapy, which are widely used, consists in giving prednisone 60 mg orally in divided doses for three consecutive days in a week, which follows četvorođnevna break.Unsuitability of this therapeutic schemes have a number of adverse effects of high doses of corticosteroids, with signs of adrenal suppression, which manifests itself in vrcme četvorodnevnc break.
For now, it is considered justified to intermittent therapy apply for one year, if the patient remains free of edema and proteinuria reduced to a negligible amount. If a minor exacerbations, therapy can be increased. Compensation potassium is preferred during corticosteroid therapy, although it may be unnecessary for administering corticosteroids every other day.
Diuretics are often ineffective. The most useful are derivatives of chlorothiazide, for example.hydrochlorothiazide, 50 mg - 100 mg every 12 hours. Other products chlorothiazide, chlorthalidone or other diuretics, can also be used in the respective effective doses. Aldosterone antagonists may help you. Together with the use of thiazides. Salt-free albumin, dextran, and other onkotični agents are of little help, and their effect in causing a transient diuresis.
Attention: Increase in serum potassium, the development of hypertension, a sudden strong increase in edema, a contraindication for further continuation of corticosteroid therapy. These complications usually occur during the first two weeks of continuous treatment.
Immunosuppressive drugs such as cyclophosphamide, mercaptopurine, azathioprine (Imuran) and others are currently attempting to treat nephrotic syndrome. The use of corticosteroids in the community with similar immunosuppressive agents used to prevent rejection in homotransplatata man. The experience is still scarce but the results of treatment of this type of therapy encouraging.This type of therapy leads to improvement in children and adults with proliferative, membranous, and mixed lesions with systemic lupus erythematosus. In patients with minimal lesions that are refractory to corticosteroids, giving, giving immune suppressive agents did not improve. Glomerular changes in renal function and in many cases have responded well to this treatment. The frequency of improvement was not found.
During the treatment, it is often plagued by serious side effects, which apply both to corticosteroids and cytotoxic agents. For now, this form of therapy should be used only in patients who are refractory to conventional treatment regimen, and by physicians who are experienced in the treatment of nephrotic syndrome.
Forecast
The course and prognosis depend on the underlying disease that is responsible for the occurrence of nephrotic syndrome in about 50% of nephrosis in childhood, the disease appears suddenly, and doing a benign course in the case of appropriate treatment and leaves a slight squeal. However, many patients are going to relentlessly terminal condition with renal insufficiency. Adult patients with nephrosis go lower, especially when the primary disease is glomerulonephritis, systemic lupus erythematosus, amyloidosis, renal vein thrombosis and diabetic nephropathy. In patients with minimal lesions or spontaneous remission or after corticosteroid therapy is common.
Treatment is much more frequent failures or only leads to 'improvement when there are other glomerular lesions. Hypertension and nitrogen retention are poor prognostic signs.
Of the authors:
Dr. Henry Brainerd, professor of medicine
Dr. Marcus A. Krupp, Professor of Medicine
Dr. Milton J. Chatton, Professor of Medicine
Dr. Sheldon Margen, professor of medicine
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