Author: Leo and Lucy
Key words: nephropathy | FSGS
Guidance
The definition, classification and pathogenesis of FSGS (focal segmental
glomerulosclerosis)
FSGS refers to a pathological form of the kidney, rather than a disease, manifested as glaucoma glaucoma focal, segmental sclerosis (mesangial matrix increased with capillary occlusion, sclerosis, vitreous changes, foam cells, segmental Scar formation, balloon adhesions).
figure 1
Figure 1: PAS staining, large arrows for the mesangial matrix increased, the lumen gradually occluded. Small arrows for segmental hardening parts of podocyte lesions, increased.
Classification of FSGS
① According to pathological classification:
(At least one glomerulus with segmental capillary hyperplasia), the portal type (≥50% of the segmental sclerosis at the door), except that the above 4 type was diagnosed as non-specific type The
FSGS recurrence after renal transplantation showed the same pathological type, suggesting that this classification there is a certain significance.
And the individual study observed the difference between the various types: the top and collapse of proteinuria more; top-end tubulointerstitial injury is the lightest, the best renal function, glucocorticoid treatment the best; collapse of the worst prognosis of the kidney.
At present, this classification has gradually been part of the pathologist to give up, that this type of clinical significance is not.
But the more special is the collapse of the current African-Americans found that the incidence of APOL1 gene mutation associated with a special clinical, pathogenesis and prognosis, and part of the collapse of glomerular disease without segmental sclerosis, so the current tendency to its Alone out of a disease: collapse of glomerular disease.
Figure 2: Collapse type.
Figure 3: Top type
② According to the pathogenesis, FSGS will be divided into four categories:
Primary (primary podocytosis), hereditary, adaptive, repairable
Adaptability and restorative FSGS "secondary FSGS". This is the relative comparison of the promotion of the classification.
The following describes the incidence of various FSGS mechanism.
The pathogenesis of FSGS
① primary:
At present, it is recognized that the pathogenesis of a cycle of a causative agent caused by podocyte injury. A circulatory factor that has been found to be suPAR (soluble urokinase plasminogen activator). SuPAR by podocyte αVβ3 integrin binding to affect the maturation of the foot, adhere to the GBM, part of the study found that the level of primary FSGS increased, nephrotic syndrome remission suPAR levels decreased, pre-transplant suPAR levels Higher recurrence, but some of the findings are contrary to the above.
Another pathogenesis in primary FSGS is microRNA-193a, which inhibits the translation of WT1 messenger RNA in podocytes, thereby inhibiting the expression of many genes that are controlled by WT1, such as nephrin.
② hereditary:
Mainly, the glomerular podocyte fissure membrane on the various molecules, due to genetic abnormalities, resulting in podocyte diffuse damage, fusion.
Clinical manifestations of congenital nephrotic syndrome or part of the hormone resistance nephrotic syndrome.
③ adaptability:
(GBM) is not covered by podocytes, GBM is exposed, and the localized glomerular basement membrane (GBM) is not covered by podocytes, and the localized glomerular basement membrane (GBM) Secondary FSGS-like lesions.
④ repair:
Such as vasculitis, IgA nephropathy or lupus caused by segmental glomerular necrosis, injury, local repair will form FSGS-like lesions.
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