The pathogenesis of focal segmental sclerosis

The pathogenesis of focal segmental sclerosis

The pathogenesis of focal segmental sclerosis 

1. The genetic background The incidence of the disease in different ethnic groups have significant differences. Especially the black American high incidence, prognosis is poor, tip genetic background play an important role in its pathogenesis. Now find genetic FSGS have autosomal recessive and dominant inheritance 

Two ways. The former is relatively common, more code located within the chromosome lq25-31 by foot podocln - NPHS2 mutations on the cell membrane. Podocin is composed of 383 amino acids, is located in the foot on the membrane of cells near the fracture, and the foot on the membranes of the burst 

Nephrin. And nephrin is the most important function of membrane proteins of joints, the disease can cause kidney disease levels of proteinuria, its genetic NPHSl mutations in congenital nephrotic syndrome of the Finnish type. Thus infer that podocin lesions may be affected by work of nephrin 

To cause disease. 

2. Circulation factor, people call it the earliest vessels appear a factor, put forward the ideas to benefit from some very valuable clinical observation. Patients with FSGS can relapse after renal transplantation surgery. Typical cases of postoperative occurred within 24 h proteinuria, 

If 1-4 go in kidney transplant renal biopsy, the vast majority of patients with renal histopathological basic normal, only a foot process fusion was observed under electron microscope. If you don't relieve postoperative patients with proteinuria continued, 2-11 months after surgery again renal biopsy, glomerular lesions showed FSGS samples, one of them 

Some patients show the collapse type FSGS. After renal transplantation patients with recurrence of FSGS serum injected into mice can induce proteinuria, suggests the role of circulating factors on the pathogenesis of FSGS. 

3. The formation of FSGS Under the influence of pathogenic factors, various inherent cells within the glomeruli are subject to different levels of stimulation, to produce a large number of cytokines mediated inherent cell activation, causes the extracellular matrix has increased, the plasma leakage, thus make the capillary loops 

Stuck, blocked, hardening gradually formed. Layer in the process, the glomerulus epithelial cells, sertoli cell, is mainly involved in the cell. Classic FSGS line into process may be the pathological changes, after podocyte damage, appear degeneration and glomerular basement membrane (off) 

Sample change, causes the expansion of capillary loops and micro hemangioma, bare glomerular basement membrane and baumann capsule and adhesion, and filtered plasma composition here directly into the wall layer between epithelial cells and baumann thecal sac, tear through further, extending, makes the disease 

Change to the glomerulus and its continuous progress of renal tubule; At the same time, the local lesion, extracellular matrix produce growing. Pressure capillary loops, eventually leads to the formation of a segmental glomerular sclerosis, may be accompanied by foot cells, endothelial cell hyperplasia (cells). 

4. The progress of FSGS, worse, to end-stage renal disease This includes two aspects of content: (1) a single nephron segmental sclerosis in progress; How to progress from segmental sclerosis for global sclerosis and how to from the development of glomerular lesions to multifocal renal tubular atrophy and renal interstitial fine 

D; (2) what are the external factors accelerated the end-stage kidney disease. Authorities, after the formation of focal segmental sclerosis in continuously under the action of pathogenic factors, will gradually progress to diffuse the ball sclerosis (i.e., end-stage renal disease. In the same glomerulus with two kinds of pathological evolution process is 

Common: (1) increasing segmental sclerosis, expand, bring about sclerosis of the ball, (2) the balloon of adhesion is still can continue to filter the plasma components are no longer as a normal condition to enter into the lumen of the baumann, but directly into the wall layer between epithelial cells and baumann capsule wall, the capsule wall beam 

Bound, the filtered liquid stripped lining epithelial cells further until the blood vessels, and by again into the glomerular mesangial area part hasn't been hardened, hardened. These two kinds of evolution can appear at the same time. In the latter case, when the filter liquid along the baumann sac dissection to the kidney 

Ball, when urine very fluid filtration can by spinning off renal tubular epithelial cells and renal tubular basement membrane, downstream along the renal tubule nephron invasion, lead to multifocal renal tubular atrophy, well stimulation surrounding renal interstitial fibrosis. This may be why in the pathologic specimens of patients with FSGS often easily 

See multifocal renal tubular atrophy and renal interstitial fibrosis. And in the same large amounts of albuminuria in patients with MCD is difficult to meet one of the main reasons, become so both identify important clues. In addition, besides the above mechanism, also often exist in patients to accelerate pathological changes 

Progress of other factors, such as; Overworked, excessive salt intake, hypertension, hyperlipidemia, woods nephron high dynamic state, etc. 

In everyday life we want to avoid the above factors. 

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