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Pathological types and clinical features of primary nephroti

Dec 26, 2016
Microscopically, glomerular microscopically was normal in glomeruli with minimal change nephropathy, and fat degeneration was observed in proximal tubule epithelial cells. Immune pathological examination negative. The main diagnostic basis of the systemic change and the disease is the extensive fusion of the glomerular epithelium foot foot under the electron microscope.
Minimal change nephropathy accounts for 80%-90% of primary nephrotic syndrome in children, 20%-25% in adults with primary nephrotic syndrome. The disease of male than female, good hair in children, adult incidence is low, but the incidence of the elderly is increased. Typical clinical manifestations of nephrotic syndrome, only about 15% of patients with microscopic hematuria, generally no persistent hypertension and renal dysfunction. Can cause transient hypertension due to severe sodium and water retention, usually disappear after diuresis.
Approximately 30%-40% cases of this disease may spontaneously relieve within months after onset. 90% cases were sensitive to steroid treatment, about two weeks after beginning diuresis, urine protein can be in a few weeks to quickly reduce the negative, plasma albumin gradually returned to normal level, to reach clinical complete remission. However, the recurrence rate of this disease is as high as 60%. If the recurrent or long-term proteinuria is not controlled, the disease may be transformed into mesangial proliferative glomerulonephritis, and then focal segmental glomerulosclerosis. It is generally believed that the remission rate and remission rate of adult are lower than that of children.
Two. Mesangial proliferative glomerulonephritis showed diffuse proliferation of mesangial cells and mesangial stroma under light microscope. The pathological changes could be pided into IgA nephropathy and non IgA mesangial proliferative glomerulonephritis. The former is mainly IgA deposition, the latter with IgG (more common in China) or IgM deposition, are often accompanied by C3 in the glomerular mesangial area, or mesangial area and capillary wall particle deposition. Electron dense matter can be seen in the film zone under electron microscope.
The incidence of this disease in China is very high, accounting for about 30% in primary nephrotic syndrome, significantly higher than in Western countries. The disease more men than women, predilection in adolescents. About 50% of patients have prodromal infection, can be acute after upper respiratory infection, and even acute nephritic syndrome. Some patients with concealed onset. In this group of diseases, non IgA mesangial proliferative glomerulonephritis of about 50% patients showed nephrotic syndrome, about 70% patients with hematuria; and IgA nephropathy almost hematuria, about 15% nephrotic syndrome. With the severity of renal disease from mild to severe, the incidence of renal insufficiency and hypertension gradually increased.
This is a group of diseases of nephrotic syndrome, treatment response and pathological types in the severity of glucocorticoid and cytotoxic drugs, it has good curative effect, or poor effect.
Three, a common pathological mesangiocapillary glomerulonephritis under light microscope changes of glomerular mesangial cells and mesangial matrix with severe hyperplasia, can be inserted between the glomerular basement membrane and endothelial cells, the capillary loops showed "double track sign". Immunohistochemical examination showed that IgG and C3 were granular in the mesangial area and capillary wall deposition. Electron dense deposits in the mesangial area and under the electron microscope.
This pathological type accounts for about 10% of primary nephrotic syndrome in China. The disease of men than women, good hair in young adults. About 1/4-1/3 patients often in the incidence of upper respiratory tract infection, such as acute nephritis syndrome; about 50%-60% patients with nephrotic syndrome, almost all patients with hematuria, for which a few episodes of gross hematuria; the remaining few patients showed asymptomatic hematuria and proteinuria. Renal function damage, hypertension and anemia appear early, the disease continues to progress. 50%-70% cases of serum C3 continued to reduce, suggesting significant significance for this disease.
Treatment of nephrotic syndrome caused by this disease is difficult, glucocorticoid and cytotoxic drug therapy may only be effective for some children, poor adult efficacy. Lesions progress rapidly, 10 years after the onset of about 50% of cases will progress to chronic renal failure.
Four, membranous nephropathy can be seen under light microscope diffuse glomerular lesions, early in the glomerular basement membrane epithelial side of the majority of neat red particles; and then nail formation, the basement membrane gradually thickened. Immuno pathology showed that IgG and C3 were finely granular along the glomerular capillary wall. Under electron microscope, the electron dense deposits were arranged on the epithelial side of the basement membrane, which was often accompanied by extensive foot fusion.
The disease more men than women, good hair in the elderly. Usually insidious onset, about 80% showed nephrotic syndrome, about 30% may be accompanied by microscopic hematuria, generally no gross hematuria. Renal dysfunction often occurs after 5-10 years of onset. The disease is prone to thrombosis, embolism complications, renal vein thrombosis can be as high as 40%-50%.
This disease accounts for about 25%-30% of primary nephrotic syndrome in China. Slow progression of disease. Clinical manifestations of approximately 20%-35% patients can spontaneously relieve. Approximately 60%-70% of patients with early membranous nephropathy (without nail spikes) can be clinically relieved after treatment with corticosteroids and cytotoxic drugs. But with the gradual progress of the disease, pathological changes, poor therapeutic efficacy, often difficult to reduce urinary protein.
Five, focal segmental glomerulosclerosis light microscopy showed lesions were focal, segmental distribution, mainly for the involvement of segmental sclerosis (mesangial matrix increased, capillary occlusion, balloon adhesion, etc.). Corresponding renal tubular atrophy and renal interstitial fibrosis. Immunohistochemical examination showed that IgM and C3 showed mass deposition in the glomerular segments. Electron microscopy revealed extensive fusion of podocytes in glomerular epithelium.
This pathological type accounts for about 5%-10% of primary nephrotic syndrome in China. The disease occurs in young men, mostly occult onset, some cases can be transformed from small form of nephrosis. Clinical manifestations of nephrotic syndrome, of which about 3/4 patients with hematuria, about 20% visible gross hematuria. About half of patients diagnosed with this disease have hypertension and 30% have renal dysfunction, some patients may be accompanied by renal diabetes, amino acid urine and phosphate urine and other proximal tubule disorder.
The treatment of glucocorticoid and cytotoxic drugs is slow, more than half of patients with poor efficacy, gradually developed to renal failure. But about 30%-50% patients after treatment may get clinical remission, the condition can be more stable.

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