2021-01-26 12:00 AM

Kimmelstien-Wilson syndrome stage damage to the mid-capillary nodular block with damage to the kidney arteries.



Glomerular disease is functional or physical damage that manifests itself in the glomeruli with clinical features of oedema, proteinuria, hypertension, chronic progression and often leading to chronic renal failure. The diagnosis of glomerular disease is mainly based on kidney biopsy. The prognosis of the disease depends on the cause and the histological damage on the biopsy.


There are many classifications of glomerulonephritis, including those of Hambuger 1980, Brenner 1985 and Cohn 1991. Below we refer to the simplest classification introduced by the association of kidney professors. In 2003, this classification divided the glomerular disease into two main groups, depending on the acute or chronic course of the disease:

Acute glomerular diseases:

Acute glomerulonephritis following streptococcal infection.

Glomerulonephritis progresses rapidly.

Glomerulonephritis caused by toxaemia in pregnancy.

Glomerular disease caused by Lupus.

Chronic glomerular diseases:

Glomerular disease with minimal damage (fatty kidney damage).

Fibromyalgia and segmental fibrosis.

Membranous glomerulonephritis (either on the membrane or the membrane).

Membranoproliferative glomerulonephritis.

IgA glomerulonephritis (Berger's disease).

Diabetic glomerulonephritis.

Amylose kidney

Alport syndrome.

Major forms of the disease

Acute glomerulonephritis

It is caused by group A haemolytic Beta haemolytic streptococci, type 12 is typical for a sore throat, type 49 is typical after purulent dermatitis. Clinical manifestations usually appear 10 - 21 days after pharyngitis suddenly appears lumbar pain in both sides, oliguria, swelling mainly of the eyelids, ankles and often accompanied by fever. Little urine, less than 500 ml / 24 hours, light red colour due to red blood cell urine, dark red in gross haematuria.

Both maximum and minimum high blood pressure are common in moderation.

Assay with ASLO increased over 400 units Todd, Protein ​​urine from 0.2 to 3 g / 24 hours, Addis residue has red blood cells from 100,000-500,000 / 1 minute, but leukocytes below 20,000 / 1 minute (also known as love erythrocytes - leukocytes in urine). Often see erythrocytes, granular casts.

Progression is usually good: healing is 75-95% in children and 50-70% in adults.

Acute glomerulonephritis can progress rapidly or malignantly

Clinical feature is usually severe, progressing rapidly to irreversible renal failure. Anatomically, there is a proliferation beyond the capillaries, invading the Bowman, strangling the microvascular. The disease accounts for about 1% of inflammatory glomerulonephritis, can be encountered at any age, can start after a sore throat or dermatitis.

The categories are classified according to the following types:

Type I: Basal membrane IgG deposit + 20% anti-membrane antibody.

Type II: Specific for immune system complex deposition with IgG and complement 40%.

Type III: There is no immune deposition on the glomerulus.

The main clinical sign is oliguria and then quickly leads to anuria with severe renal failure. Moderate oedema of normal or slightly elevated blood pressure sometimes increased blood pressure (globulin. The disease dies in a few weeks to months. pathological surgery found both inner and outer capillary lesions, glomeruli were completely destroyed in a few days, on immunofluorescence diffuse fibrin deposition on sickle lesions, IgG was deposited on the basal membrane corresponds to basal membrane antibodies.

Chronic glomerulonephritis

According to current conception, chronic glomerulonephritis after intermittent infection accounts for only 10%, the rest is secondary to another and idiopathic disease.

Primary chronic glomerulonephritis:

It is glomerulonephritis of unknown cause. Due to the lack of data on etiology, classification is based on clinical and anatomic studies.

A nephrotic syndrome due to minimal damage:

The result of the loss of proteinuria is mainly Albumin, which can be seen after completely different kidney damage. Commonly is a simple nephrotic syndrome appearing suddenly, proteinuria, swelling, decreased blood Protide, increased lipids, blood cholesterol. The disease does not appear to have a clear cause. It is common in children, men more than women, and in adults, the ratio of the two sexes is the same.

Minimal damage during kidney biopsy, normally seen on the optical microscope, an electron microscope into modified capillary wall spread to the base of the basal membrane of sickle cells, shows membrane fusion of cells with legs. Immunofluorescence did not detect any deposition, there was no immunoglobulin or complement deposited on the basal membrane.

Progression of the disease is often difficult to determine, but it is found that 10% recover by itself, 80% recover completely (Protein ​​urine less than 0.10 g / 24 hours, other signs return to normal) thanks to treatment with corticosteroids after a few weeks or months but 40% of them relapse once in 6 months, 60% come back many times in 1 year and can depend on corticosteroids, which means stop the medicine from coming back again. If the disease is resistant to corticosteroids, the prognosis will easily lead to kidney failure.

Membranous (epidural and epidural) glomerulonephritis:

The clinical manifestation is a nephrotic syndrome with microscopic haematuria.

Pathological anatomical lesions consist of interrupted beads with a membrane form perpendicular to the capillary wall forming a brush pattern on a microscope. Immunofluorescence shows the deposition of IgG and complements C3. The disease is more common in adults than children, and this damage accounts for more than 50% of the primary nephrotic syndrome in American adults. The progression is relatively good, the disease can be reversed after a few months, the clinical signs are completely lost, on biopsy, the deposition is lost, others progress to kidney failure. Adults have a worse prognosis in children, about 66% of patients surviving 10 years of onset.

Membranoproliferative glomerulonephritis:

Uncommon form of chronic glomerulonephritis, clinically includes proteinuria, microscopic haematuria, sometimes with typical nephrotic syndrome with renal failure, hypertension, easily leading to end-stage chronic renal failure.

Pathological damage is the proliferation of stromal cells, the epithelial cells of the Bowman follicle (possibly endoderm cells) forming a helix pattern, proliferation with deposition in the basal membrane to create a 2-sided shape. called proliferation. On immunofluorescence, it is found to have deposition of complement C1 q, C4, C3, and IgG, sometimes IgM, and C3 antisera can be seen in the glomeruli, the complement concentration in the blood is always low compared to other forms. other.

A poor prognosis, often progressing to chronic kidney failure, is only about 20-50% to survive after 10 years.

Glomerulonephritis IgA deposition in the stroma:

The clinical manifestation is repeated major episodes of haematuria, moderate protein ​​urinary, not accompanied by nephrotic syndrome, rarely leading to renal failure. On immunofluorescence, IgA deposition sometimes even in the buffer zone can be detected through normal microscopy, some glomeruli see fragmentary lesions, superimposed capillary loops sticking to Bowman's cyst. The pathogenesis is not clear, often seen in young people. An increase in serum IgA could be seen but not constant. When a patient has a kidney transplant, the kidney transplant also has the same symptoms as the old kidney disease, so it is thought that it may be the cause outside the kidney.

Sclerosing and segmental glomerulonephritis:

It accounts for 5-10% of nephrotic syndrome in children, and 15-20% in nephrotic syndrome in adults, it is more common in men than in women. Patients often present with microscopic haematuria, hypertension, and chronic renal failure. Proteinuria in this form is always high and is not selective proteinuria. Anatomical lesions: Hyalin deposition on optical microscopy and fibrosis lesions of each cluster, segment, immunofluorescence microscopy show deposition of IgM and C3 of each segment.

The prognosis is often poor, leading to progressive renal failure in 25% of cases in children and in 70% of cases in adults. Usually leads to end-stage chronic kidney failure within 5 to 20 years

Secondary glomerulonephritis in a generalized disease

Acute glomerulonephritis in patients with lupus:

Glomerulonephritis accounts for about 70% of lupus erythematosus, common in young women with erythema, paraesthesia, with a butterfly-shaped rash on the face, weight loss, fever, joint pain ... Hargraves cells, especially antinuclear antibodies in the blood. Clinical manifestations include nephrotic syndrome without hypertension, nonspecific lesions with thick deposition of basal membrane forming iron ring, haematoxylin in the buffer zone. Prognosis is possible with renal failure and usually without hypertension.

Good Pasture syndrome or necrotic glomerulonephritis with haemorrhagic alveoli:

More common in men than women, starting with haemoptysis, blurred lung image of the sides is not clear, then damage to the kidneys, after a few months of causing kidney failure, high blood pressure leads to death. Haemorrhagic inflammation alveoli are filled with iron-pigmented macrophages, the kidneys form fibrin and then the chemical form. These lesions have been shown to coordinate with autoantibodies to act simultaneously against both the alveoli and the glomerular basement membrane.

Glomerulonephritis due to rheumatoid rash or Schonlein-Henoch rash:

Kidney damage accounts for 35% of this disease. Clinically often there is haematuria, glomerular damage, Immunofluorescence shows IgA deposition in the buffer and wall. The disease causes damage to most of the glomeruli, poor prognosis, progresses to kidney failure.

Renal Amylose Disease:

Common in visceral Amylose disease, secondary to chronic infections or infections such as osteomyelitis, tuberculosis, rheumatoid arthritis, leprosy, paralysis of the lower extremities. The disease can be primary. In Vietnam, no cases have been recorded, but in the Mediterranean region, most people from 50-60 years old can be found.

The clinical manifestation is nephrotic syndrome, which can only have protein ​​urine alone with hepatosplenomegaly, microscopic haematuria, a rare increase in blood pressure, leading to kidney failure. Digestion is manifested by diarrhoea, constipation, haemorrhage, enlarged tongue, enlarged liver, but normal liver function.

Anatomic pathology shows the deposition of amyloids that catch red to iridescent purple colour with a specific fibre structure on the electron microscope, which is essentially a protein, found outside the cell, impurity and city.

Glomerular disease caused by diabetes:

Diabetes-induced glomerular damage, diffuse sclerosis of the glomeruli, initially manifested by irregular thickening of the glomerular basal membrane with endodermal deposition. Clinical stage is the first stage with protein ​​urine, with or without the nephrotic syndrome, microscopic haematuria tends to turn to renal failure with hypertension.

Kimmelstien-Wilson syndrome stage damage to the mid-capillary nodular block with damage to the kidney arteries.

According to Mogensen divided glomerulonephritis caused by diabetes into 5 stages:

Stage 1: Hyperfunction and hypertrophy, which occurs as soon as diabetes is diagnosed, before insulin treatment. The size of the kidneys increases, the glomeruli are enlarged, but their structure is normal. Glomerular filtration rate increases, blood flow through the kidney increases.

Stage 2: Can persist for many years and last a lifetime. Glomerular filtration rate may increase. Urinary albumin is negative but can be positive under stress. The thickness of the glomerular basal membrane increases, the interstitial matrix increases more and these lesions are irreversible.

Stage 3: The initial stage of diabetic nephropathy, which occurs only in 40% of diabetic patients, lasts from 10 to 20 years, the most important manifestation in this stage is the appearance of Microalbuminuria. (Micro proteinuria, Micro proteinuria).

Stage 4: The established diabetic nephropathy stage with regular proteinuria over 0.5g / 24 hours, often with hypertension, nephrotic syndrome. Glomerular filtration rate decreased by an average of 1ml / month. Glomerular damage: diffuse glomerular fibrosis, including basal membrane thickening and increased intervascular stroma. In 10-20% of cases, nodules exist because PAS accumulates in the space around the glomeruli (called nodular glomerular fibrosis of Kimmelstiel and Wilson).

Stage 5: The stage of the patient with end-stage chronic renal failure.

Treatment of glomerulonephritis

Treatment of acute glomerulonephritis following streptococcal infection

Patients with minimal and anuria have increased urea, blood creatinine: 500-600ml water, salt 2g / 24h, protein 20g / day.

Minimize and anuria with increased blood pressure, urea, and creatinine in blood do not increase: salt 0.5 - 1g / 24h, protein 40g / day.

Absolute rest from 3 weeks to 1 month. Then return to activity slowly as soon as proteinuria remains and microscopic haematuria from 6 weeks to 2 months.


Only for signs of streptococcal infection.

Penicillin 1 million units/adult, 1/2 million units/child. If allergy to Penicillin, then use Erythromycin 0.2 x 5 tablets/day in adults or Tetracycline. Antibiotics for 10 - 12 days.

Treatment of complications:

Hypertension: Abstain from salt, rest, drugs such as Aldomet (0.25g x 2-4 tablets/day).

Pulmonary oedema: Lasix at high doses, intravenous uabaine, oxygen, morphine when needed.

Brain oedema: Hypertonic glucose infusion, Mannitol.

Anuria: Furosemide (Lasix) at high dose.

Best for both pulmonary oedema, cerebral, anuria is dialysis.

Other symptoms:

Increase blood urea, blood creatinine, limit the amount of Protide taken, but ensure enough energy from 1200-1600 calories to fight catabolism, Durabulin or Testosterone 25-50 mg/day.

Treatment of hyperkalaemia with glucose 20-30%, 300 - 500ml + 10-20vvv insulin intravenous infusion for 1 hour to 1g30 minutes or alkaline solution infusion.

Progression is usually good: healing is 75-95% in children and 50-70% in adults.

Treatment of acute glomerulonephritis is rapid progression or malignancy

Clinical condition is usually severe, progressing rapidly to irreversible renal failure.

Type I: Prednisolone, 5 mg tablet, 2-3 mg / Kg / 24 hours, combined with Cyclophosphamide (Endoxan) 50 mg tablet, 1-3 mg / kg / 24 hours or Chlorambucil (Chloraminophene) 2 mg capsule, dose 0.15-0.2 mg / Kg / 24 hours. The duration of immunotherapy is from 2 to 4 months.

Combined with 2 - 4 liter plasma filter.

Type II: Methylprednisolone 30 mg / Kg / 24 hours intravenously x 3 times for 2 days.

Then maintain Prednisolone 1-3 mg / Kg/day for many months.

Type III: Treatment as type II + combined with Heparin, cyclophosphamide (Endoxan).

Also, coordinate with Heparin 5000 - 10,000 units in 4-8 weeks.

Treatment of primary chronic glomerulonephritis

Nephrotic syndrome:

Treatment includes: Prednisolone 1 mg / Kg / 24 hours in adults 1.5 - 2 mg / Kg / 24 hours in children.

If the response is good, reduce the dose on a ladder every 5 days, reduce 10 mg and then maintain the disruption every 20-30 mg / 2 days, orally once in 10 mg / 2 days.

When there is a phenomenon of steroid resistance or dependence, cyclophosphamide 2 - 3 mg / Kg / 24 hours, or chlorambucil 0.2 - 0.3 mg / Kg / 24 hours for at least 2 months, must be monitored for complications. about blood and other complications.

Membranous (epidural and epidural) glomerulonephritis:

According to the British school, the US appoints Prednisolone with a high dose of 1.5-2 mg / Kg / 48 hours orally once in the morning for 8 weeks to see good results, people can give another 4 weeks or a combination with 1g methylprednisolone for 3 consecutive days, then 0.5mg prednisolone/kg/day/month then 0.2 mg/kg/day chlorambucil for 6 months. This treatment is better than a steroid alone. When it relapses, use the same, immunosuppressants, after a few days.

Treatment of glomerulonephritis membrane proliferation

The prognosis is bad, progressing to kidney failure, only 20-50% after 10 years.

Combination treatment of corticosteroids and immunosuppressants with anticoagulants or anti-platelet aggregation.

Treatment of glomerulonephritis IgA deposition in the stroma

In patients with moderate histological damage, with proteinuria more than 3 g / 24 hours, corticoids treatment within 4 to 6 months. When proteinuria does not improve, immunosuppressive therapy can be used as in nephrotic syndrome.

In Western countries, people use Omega 3 fatty acid (fish oil) in the case of advanced disease, to limit the production and activity of Cytokines caused by the deposition of IgA in the glomeruli.

In the presence of hypertension, ACE inhibitors are preferred to reduce proteinuria and slow the progression of renal failure.

Other drugs used by some authors in IgA-deposition glomerulonephritis are anti-platelet aggregation agents, Urokinase, or high dose intravenous immunoglobulins that have not been found to be effective. definitely in this disease.

Sclerosing and segmental glomerulonephritis

The prognosis of life over 15 years accounts for 50%.

Corticoid treatment as low dose prednisolone 1/3 - 1/2 mg/kg / 24 hours for 1-2 months, or used as in the membrane.

When there is a relapse can be treated with immunosuppressive drugs.

Secondary glomerulonephritis in a generalized disease

Acute glomerulonephritis in patients with lupus:

Treatment: Prednisolone high dose 2-3 mg / kg / 24 hours.

The immunosuppressant Chlorambucil 0.2 - 0.3 mg / Kg / 24 hours for 4-8 weeks have a clear effect.

Good Pasture syndrome or necrotic glomerulonephritis with haemorrhagic alveoli:

Treatment is also with corticoid 2-3 mg / Kg / 24 hours for 4-6 weeks, but the clinical response is poor.

Glomerulonephritis due to rheumatoid rash or Schonlein-Henoch rash:

The disease causes damage to most of the glomeruli, poor prognosis, progresses to kidney failure. Treatment with corticosteroids and immunosuppression.

Kimmelstiel-wilson syndrome:

Diabetes treatment: very difficult, because insulin sensitivity is not stable. Treatment of kidney disease is only symptomatic treatment, not corticosteroids. In the presence of end-stage chronic kidney failure due to diabetes, replacement treatment for kidney failure has some difficulties, especially the embolism and infection complications of dialysis.

Kidney - pancreas transplant is indicated in this case, although the results are very positive in both terms of diabetes and kidney failure, in practice is rarely done because of technical difficulties and donors.