Histological types and clinical features of primary nephrotic syndrome

1, MCNS minimal change nephropathy (minimal change nephropathy) occurs in children (accounting for about 80% of children with nephrotic syndrome), the major clinical manifestations of sudden massive proteinuria and hypoproteinemia may be associated with high-fat acidosis and edema. Hematuria and hypertension are rare. , Hypertension and renal dysfunction is common in patients over the age of 60.
Light microscope, glomerular lesions, visible fatty degeneration of proximal tubular epithelial cells. Immunofluorescence was negative. Electron microscope, the characteristics of changes in glomerular visceral epithelial cell foot process fusion.
2, mesangial proliferative glomerulonephritis with mesangial proliferative glomerulonephritis (mesangial proliferative glomerulongphritis) is a common pathological type of primary nephrotic syndrome, accounting for about 30%, significantly higher than that of Europe and the United States (about 10%). The disease occurs in young people, male predominance. The majority of patients before the onset of prodromal symptoms of infection of upper respiratory tract infections, some patients with insidious onset. The main clinical manifestations were proteinuria and (or) hematuria, approximately 30% of nephrotic syndrome.
The pathological characteristics of the light microscope, the mesangial cells and extracellular matrix diffuse hyperplasia can be divided into mild, moderate to severe. According to the immunofluorescence results can be divided into IgA nephropathy (pure IgA or IgA deposition-based) and non-IgA mesangial proliferative glomerulonephritis (IgG or IgM, the deposition-based), often accompanied by C3 deposition in the kidney ball mesangial area or along the capillary wall granular deposition. Electron microscope, mesangial electron dense deposits.
3, focal segmental glomerular sclerosis focal segmental glomerulosclerosis (focal segmental glomerulosclerosis) is more common in young people, more men than women. Onset are more hidden, the major clinical manifestations of massive proteinuria or nephrotic syndrome. The majority of patients with hematuria, and some patients had gross hematuria; mild also can be expressed as asymptomatic proteinuria and (or) hematuria. Upper respiratory tract infection or other predisposing factors can make the clinical symptoms. The majority of patients to establish the diagnosis is often accompanied by hypertension and renal damage, and aggravated as the disease progresses. In some cases by the shift from minimal change nephropathy.
Pathological features of the light microscope, the glomerular lesions were focal, segmental distribution, increased mesangial matrix and plasma protein deposition and balloon adhesion as the main performance, may be associated with a small amount of mesangial cell proliferation, accompanied by the same nephron tubular atrophy and interstitial fibrosis in the kidney. The immunopathological visible lumps deposition of IgM and C3 in the glomerular lesions. Electron microscope, mesangial matrix increase, electron dense precipitation of the lesion, glomerular epithelial cells wide range of foot process fusion.
4, membranous nephropathy, membranous nephropathy (membranous nephropathy) occurs in the elderly, male predominance, the peak age of onset is 50 to 60 years old. Europe and the United States adult common pathological type of nephrotic syndrome, while China is not common. Membranous nephropathy onset more hiding, no history of prodromal infection. 70% to 80% of patients with nephrotic syndrome. Early in the disease without hypertension. Most patients with normal renal function or mild impairment. The higher the incidence of arterial and venous thrombosis, especially renal vein thrombosis is the most common (about 10% ~ 40%). The 10-year kidney survival rate is about 65%.
Membranous nephropathy under the light microscope the characteristic of the diffuse thickening of the glomerular basement membrane. The immunopathological characterized immunoglobulin and complement around the capillary wall diffuse granular deposition of IgG strength can also be the deposition of IgA and IgM. Electron microscope, the basement membrane subcutaneous or dispersed or rules for the distribution of electron dense precipitate within the basement membrane, epithelial cells wide range of foot process fusion. Part of the patients with membranous nephropathy have a tendency natural ease.
5, the mesangial capillary glomerulonephritis, also known as mesangial proliferative glomerulonephritis (membranoproliferative glomerulonephritis). The disease occurs in young people, men and women roughly equal proportion. Half of the patients have a precursor of the upper respiratory tract infection history. 50% of patients with nephrotic syndrome, 30% of the patients had asymptomatic proteinuria, often accompanied by recurrent episodes of microscopic hematuria or gross hematuria. 20% to 30% of the patients manifested as acute nephritic syndrome. Hypertension, anemia and renal dysfunction is common, often ongoing development. 75% of patients with persistent hypocomplementemia, is an important feature of this disease.
Of the disease pathology is characterized by light microscopy showed mesangial cells and mesangial matrix diffuse severe hyperplasia, widely inserted into the glomerular basement membrane and endothelial cells, glomerular basement membrane was thickened layering, capillary Ban was "double-track sign. Immune pathological examination showed IgG, C3 granular precipitation along the basement membrane and mesangial areas. Electron microscope, visible electron dense precipitate in the mesangial area and subendothelial.
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