FSGS's treatment progress

Advances in the treatment of nephrotic syndrome, focal segmental glomerulosclerosis FSGS is one of the main reasons of the adult nephrotic syndrome, accounting for 15 to 20 percent of the adult nephrotic syndrome. FSGS is not an independent disease, but has many causes and pathogenic mechanisms and the type of tissue injury, a clinical and pathological syndrome. In 1957, Rich first FSGS pathology description, until the 1970s FSGS only as a clinical and pathological syndrome is listed. FSGS as one of the most common type of renal biopsy pathology, pathological type, further refinement is divided into five sub-categories. The differential diagnosis of primary and secondary FSGS, contribute to the treatment of FSGS. Past that FSGS hormone resistance, but recently with the improvement of the treatment, this situation has been improved. This article discusses the etiology, diagnosis, treatment and prognosis aspects of FSGS.
FSGS in the pathophysiology of FSGS is a descriptive diagnosis, not a disease of independence. Primary of FSGS, I do not know the cause of the secondary of FSGS (underlying cause) and pathogenesis are not fully understood, gradually profound secondary FSGS research, understanding the mechanism of primary FSGS. That the loss of secondary FSGS and renal units, high filtration and glomerular pressure and other cause renal decompensation response. Nephrectomized animal experiments to prove that the loss of podocytes is a key factor in FSGS renal decompensation response, and animal experiments confirmed the the FSGS existence of glomerular high perfusion, increased pressure within the phenomenon of hypertrophy or glomerular. That the secondary FSGS pathogenesis are as follows: Start the factors that damage podocytes, damaged podocyte detachment from the basement membrane and enter the capsule, because of foot cells can not regenerate, so that the basement membrane exposed capsule of the parietal epithelial cell adhesion on the bare basement membrane capillary climb and Bowman's capsule adhesion, and ultimately the formation of adhesion of the parts of the parietal epithelial cells and capillary climb the formation of cavities, if the adhesion of the capillary is still functional, the filtered fluid leakage into the cavities, these filtration the liquid leakage of the capillary, as fiber cells by filtrate stimulation, eventually leading to glomerular matrix fibrosis. If this leakage and loss of podocytes continued to exist, and finally to renal interstitial fibrosis, capillary collapse, adhesion, hyalinization and micro-thrombus gradually increased. According to the cause of secondary FSGS is divided into: 1). Family: ɑ-Actinin 4 defects; of nephrin defects; podocin in defects; WT-1 defects; carrying CD2AP and its defects, mitochondrial disease. 2) virus: HIV-related kidney disease, parvovirus B19 infection. 3) drugs: heroin nephropathy; interferon-ɑ; lithium; ammonia hydroxyl disodium phosphate, or alendronate. 4) high perfusion or hypertrophy lead to adaptive changes: congenital kidney to reduce single disease with compensatory hypertrophy; unilateral renal agenesis; renal cortical necrosis; reflux kidney disease; kidneys after surgical resection; chronic allograft nephropathy ; 5) non-specific scarring of the glomerular disease: focal proliferative glomerulonephritis, hereditary nephritis, membranous nephropathy, thrombotic microangiopathy. 6) lymphoma. 7) Other: diabetic nephropathy; hypertensive nephropathy; obesity; congenital cyanotic heart disease; anemia. The pathophysiology of primary FSGS is not clear, but there is evidence that circulating factors in the blood change the glomerular permeability [5].Previous studies have shown that the disease will relapse FSGS patients after renal transplantation, a few days after transplantation of patients in proteinuria can occur after plasma exchange can lead to proteinuria mitigation [6]. Literature that the increase in glomerular permeability may inhibit the permeability factor is missing or lost related.Animal and human studies results suggest that primary FSGS pathogenesis may be as follows: podocyte injury induced foot process effacement, proteinuria, and microvilli transformation, without loss or loss of podocytes, parietal epithelial cell injury, proliferation, and surrounded by the renal The ball capillary loop, and parietal epithelial cells secrete extracellular matrix lead to scar formation. However, the mechanism leading to the proliferation of parietal epithelial cells is unknown, the final activation or injury in podocytes and parietal epithelial cell activation or injury of the interaction lead to FSGS formation.
Primary and secondary FSGS differential diagnosis of primary and secondary FSGS differential diagnosis contribute to their treatment options. In general, medical history, laboratory tests may provide clues in order to facilitate the differential diagnosis. The kidney comprehensive level of proteinuria, plasma protein is an important indicator to identify the primary and secondary FSGS. [10] prompted the plasma protein <30g / L, more inclined to the primary of FSGS,> 35g / L were more inclined to obesity, reflux nephropathy, or kidney was removed secondary of FSGS. Should be noted that, in the case of secondary FSGS virus-associated FSGS, drug-related FSGS and familial FSGS, plasma protein <30g / L,. A recent study [11] showed that primary and secondary FSGS patients with foot process fusion extent inconsistent, primary FSGS foot processes to the degree of integration than the secondary FSGS weight, and foot process width compared with secondary FSGS wide foot process width and type of FSGS is not dependent on the degree of proteinuria. For patients with familial FSGS, the podocyte-related protein gene can be detected defects, adult podocyte protein gene defects than children, its defects-positive rate of about 1.5 to 5.0%, available for detection of gene ɑ-Actinin of nephrin, podocin in WT-1 and CD2AP, the current study more ɑ-actinin4 and the podocin gene defects [12,13].
China Medical Nephrology credits 2010 Annual Conference seminar is a compilation of the thematic reports lead to the diversity of its clinical features and natural history of inconsistent diagnostic criteria of the previous FSGS. Secondary to hyperperfusion FSGS less renal biopsy, clinical research is less likely to be mentioned. This article focuses on the clinical and prognosis of primary FSGS. FSGS can occur at any age, but adults mainly aged 40 to 50-year-old, male to female ratio of 1:1. Primary FSGS, secondary to infection, drugs or genetic defects in FSGS manifests itself in nephrotic syndrome; hyperfiltration secondary FSGS clinical manifestations often relatively hidden, and even urine protein of more than 3 to 4g / d, but no significant hypoproteinemia or edema [14]. In addition to proteinuria, microscopic hematuria and hypertension, whether primary or secondary FSGS are very common.
On the other hand, a recent randomized controlled trials to prove that cyclosporine effectively. 75% with only 22% of the placebo patients into remission by disease mitigation. A year later, the cyclosporine group, 48% remained in remission and the placebo group, only 13% of the disease is still in remission. It is noteworthy that no patients discontinued because of adverse side effects. Similarly, randomized controlled trials also found that the abnormal renal function or worsening, membranous nephropathy, patients can still benefit, proteinuria will decline, deterioration of delay; with cyclosporine A year later, the efficacy of half of the patients able to maintain at least two years . However, cyclosporine is still inadequate: expensive and can cause kidney damage.
Experts: wet, hot (cold), drugs, and silt blocking the function of organs wasting leads to kidney cloud cult poison the block to proteinuria, occult blood, serum creatinine, blood urea nitrogen increased as the main performance, wasting Spleen kidney damage based. Spleen renal damage, proteinuria, occult blood, serum creatinine, blood urea nitrogen standard. Evil gather scattered the main aspects of conflict, Xie Sheng leading direct cause of renal failure aggravated, banishment of evil spirits for the basic treatment principle, from the immunological point of view is to promote retention in glomerular immune complexes while the discharge, and enhance the kidney function of a lid, to reach the repair of natural toxins (creatinine, blood urea nitrogen), urine protein, occult blood, tube gradually disappear. Between them there are complementary and interdependent dialectical relationship. Therefore, lowering cloud detoxification, Jianpiyishenfang, the principle of the entire course of treatment. Jiangzhuo restore therapy departure from the etiology and pathogenesis of kidney disease, uremia, the use of specimens to take into account the dialectical differentiation of ideas to improve a single therapy session of the Chinese medicine blood stasis, tonifying. Corrected dialysis Western session palliatives. Fang Akebia, rhubarb, Pinellia the Chinese medicine clinical practice prejudicial to the blood of the drug does not do prescription dialectical side certificate corresponding rule is compared with chronic renal failure and kidney yang, and advocated Onyang ; compared to the Western term dialysis therapy, are a large step forward.