FGS prognosis

Of FSGS and poor prognosis, 25% to 30% and 30% to 40% 5 years and 10 years into the renal failure. 10% to 20% of the pediatric the OK dialysis or renal transplant recipients caused by the disease. The following clinical and laboratory indicators for estimating the prognosis of a certain reference value: (1) Clinical manifestations: non-selective proteinuria may be invalid prednisone severe persistent proteinuria and manifested as nephrotic syndrome is often the H-resistant creatinine level, acute renal failure and poor prognosis. (2) morphological changes: the lesions in the urinary pole that is, state-of-the-art disease, better prognosis, and prognosis of lesions around the vascular pole or the mixed type, the collapse associated with poor outcome. Associated with the development of interstitial fibrosis to the possibility of chronic renal failure. (3) The other accompanied circumstances: racial genetic backgrounds (Africa and Spain, children with poor prognosis, poor prognosis of a positive family history), adult patients, the prognosis is less favorable than those children, human immunodeficiency virus infection and heroin, cocaine, a drug abuser with poor prognosis.

High rates of renal transplantation recurrence of FSGS is generally believed that 25% to 40% recurrence after transplantation, 20% to 50% within five years, once again lose their kidney function, a higher relapse rate in OK kidney transplant again up to 80%. Related kidney transplantation than cadaveric renal higher relapse rate. Recurrence of FSGS often to glucocorticoid resistance, although the report cyclophosphamide (8 to 12 weeks, 1 ~ 2mg * kg-1 * d-1) allows long-term remission [15]. Early plasma exchange could prevent FSGS recurrence remains to be documented.

FSGS diagnosis

(1) how to reduce the rate of misdiagnosis. FSGS is a morphological diagnosis of an organization, in view of the hardening of the small ball as a focal distribution, found only in early disease nearly medullary renal unit, it is easy to misdiagnosis. In recent years, scholars have stressed the need for a sufficient number of biopsy specimens, and stressed that found a segmental sclerosis of glomerular enough to be diagnosed as of FSGS. According to probability estimates, 10 glomerular specimens missing rate of 35%, 20 glomerular enable the missed rate dropped to 12%. Authors suggest that, if the credibility of the FSGS diagnosis 95%, 99%, 99.5%, the minimum number of glomerular consecutive imprints on each cross-section need be for 7,8,9, but the slice cross sectiondistance of less than 27μm or 23μm [8]. Material restrictions not found in the glomerular segmental sclerosis, following 3:00 prompt FSGS have a certain reference value, namely (1) abnormalities of glomerular hypertrophy. A pediatric small lesions that later develop into FSGS the glomeruli than their peers in the control group and re-biopsy is still minimal change glomerular increased significantly. (2) with minimal change in the foot process fusion completely compared to that of FSGS foot process fusion is incomplete. (3) of FSGS is often accompanied by varying degrees of renal tubular damage, so check to see the small tube of focal atrophy with interstitial change is also a valuable reference.
Two different parts of the glomerular sclerosis. In recent years, noted that FSGS sclerosis lesions have a different distribution in the glomerulus, and its primary disease due and prognosis. Common change of five kinds: (1) hardening occurs in the glomerular urinary pole. (2) glomerular sclerosis occurs in blood vessels with a very transparent degeneration. (3) glomerular sclerosis in the capillary loop margin with parietal epithelial cell adhesion. (4) collapse of glomerular sclerosis. (5), diabetic nephropathy unique to tuberous sclerosis and hyalinization of the capillary arteries. Due to the cause, the variety of pathophysiological mechanisms of glomerulosclerosis area can see Schiff acid-positive cell-free material, but its composition may be different. In the glomerular vascular pole most of the renal atrophy and poor prognosis; located in the urinary pole, the so-called cutting-edge lesions, suggesting that the early lesions, the prognosis is good. The margin of lesions are most common in the pediatric, mixed lesions and vascular pole lesions is more pronounced in adults, but all the hardened forms can be found in all age groups. The morphological characteristics of illness thus different. Secondary to reflux nephropathy, often around the renal capsule fibrosis and Bowman's capsule, interstitial thickening and spotty scarring. Heroin kidney epithelial cells change, the onset of interstitial fibrosis and tubule damage bit more obvious. The human immunodeficiency virus (HIV) infection caused by focal scarring, severe renal damage, including cystic dilatation, as well as glomerular vascular atrophy and electron microscopy to see the network organization gathered on the endothelial cells.
3. Except of systemic disease or primary renal disease secondary of FSGS. : Confirm the primary or idiopathic FSGS, except a variety of secondary glomerular hypertrophy / hyperfiltration due to of FSGS (such as solitary kidney, reflux nephropathy, unilateral renal resection, a variety of reduced nephron caused by kidney disease, diabetes, hypertension, obesity, sickle cell disease, etc.) and due to renal scarring cause of FSGS (such as IgA nephropathy, polyarteritis, lupus nephritis, hereditary nephropathy, etc.). In addition, the need to except the human immunodeficiency virus infection and heroin, cocaine addict kidney changes.
Commonly used clinical indicators in glomerular diseases FSGS diagnosis and its severity predicted value. Of FSGS clinical: 97% had proteinuria, microscopic hematuria 91%, hyperlipidemia 83%, 68% of the urinary tube, hypertension 67%, 50% of nephrotic syndrome, renal dysfunction, 47%, family history of 11 %.
Due to the trauma of the kidney biopsy, kidney disease scientist trying to explore some of the features associated with the indicators to be diagnosed and prognosis. (1) urinary protein often clues of FSGS, but neither diagnosis is difficult to estimate the extent of of FSGS. Proteinuria is mainly caused by the non-hardening of the permeability of the glomerular capillary wall abnormalities, no direct correlation with FSGS. And extensive glomerular sclerosis, proteinuria but started to decrease. Furthermore proteinuria and glomerular capillary hemodynamic abnormalities. Therefore, to improve the hemodynamic drugs such as angiotensin converting enzyme inhibitors, although in the short term to reduce urinary protein but does not affect the morphology. (2) serum creatinine is also commonly used clinical indicators, FSGS can uniquely affect the glomerular filtration function activated, and some lesions of glomerular function decreased by other alive glomerular function enhanced to compensate, so the FSGSdegree and creatinine levels can be very different. (3) changes in glomerular filtration rate (GFR): FSGS is usually accompanied by a small tube between the different levels of quality change, the lack of the integrity of the tubule, GFR markers, such as creatinine, can reflux back into the blood circulation, rather than from the urine, leading to underestimate the level of GFR. And renal blood flow change can also affect the GFR.Some antihypertensive drugs (angiotensin converting enzyme inhibitors), effectively reduce glomerular capillary pressure, which led to the decline in GFR. Such a drastic decline in GFR is not caused due to deterioration in FSGS organizations learn.
To sum up, this feature of FSGS - the reason for the discrepancy in the structure can be summarized as: (1) focal segmental distribution of lesions; (2) loss of renal units; (3) of the complete glomerular compensatory ; (4) renal blood flow change; (5) plasma osmotic pressure changes and changes in glomerular local pressure; (6) tubulointerstitial changes.

FSGS that continued progress may be

(1) growth factors. The basis put forward this doctrine: ① pathological mild hypertrophy with a small ball with mild sclerosis often coexist. FSGS glomerular volume significantly larger than the minimal change disease or normal controls. ② inhibition of specific growth factors can effectively prevent the development of glomerular sclerosis.For example, an inhibitor of angiotensin Ⅱ by inhibiting platelet-associated growth factors and the transfer of growth factor β treatment of FSGS.③ the clinical lead to poor glomerular proliferation of abnormal factors, including hypoxia, hypertension and renal volume reduction, such as more than a kidney nephrectomy and unilateral renal dysplasia, often accompanied by the hair of FSGS. Molecular biology research from animal models and human biopsy data prompt The glomerulosclerosis each process is controlled by a major and several minor growth factor such as platelet-associated growth factors like early play an important role in hardening transfer growth factor beta hardening severity.
(2) hypertension. Glomerular pressure may impair kidney function and structure. On the contrary, reducing stress can improve and prevent hardening. Some people think that the pressure increased, the increase in capillary diameter, resulting in increased vascular wall tension caused by hardening. However, in the case of diabetic nephropathy and reflux nephropathy, glomerular increases than capillary diameter increased, but the vessel length extension and branch increase in the number; In addition, high blood pressure does not often appear in front of the glomerular sclerosis, as well as the The researchers found that only resection in nephron glomerular volume was significantly increased before the development of hardening. Therefore, high filtration, high perfusion pressure may not glomerular volume increase, or hardening of the only condition.
(3) the role and influence of proteinuria of FSGS progress: Some scholars believe that proteinuria itself may promote glomerular mesangial and epithelial cells or tubulointerstitial renal tissue scarring. Secondly, proteinuria can be induced by the inflammatory mediators, such as macrophages and TGFβ increased, leading to glomerular interstitial scarring.
(4) high cholesterol may affect the progress of FSGS. Foam cells, fat phagocytes with FSGS is more common. Some scholars believe that in the case of proteinuria, the filtered neutral fat matrix aggregation a specific pathogenic role [5,6]. Small lesions of lipids is also very significant and do not necessarily have hardened, it prompts the hyperlipidemia is only the cofactors of the hardening process.

Drug target for treatment of nephrotic

The scientists found a kidney disease can lead to kidney failure and other reasons, this finding may be able to bring a drug target for the treatment of chronic kidney disease.Focal segmental glomerulosclerosis (referred to as FSGS) is characterized by lesions and hardening of the tiny blood vessels in the kidneys, resulting in protein penetrate into the urine. There are no effective method for the treatment of FSGS, and its incidence is also increasing. By studying a large family of hereditary FSGS, Michelle Winn, and colleagues in the coding of a found a mutation in the gene of calcium into the cell protein.This ion channel proteins called transient receptor potential cation channel 6 or TRPC6.Because ion channels are usually susceptible to the influence of drugs, so this work will TRPC6 as a drug target for the treatment of chronic kidney disease a possibility.Previous studies have revealed that interrupt cytoskeletal and structural proteins of FSGS development process works.
Duke University Medical Center researchers have discovered a gene related to the type of chronic kidney disease. This disease called FSGS (familial focal segmental glomerulosclerosis) can cause complete renal failure, and affects 20% of dialysis patients. This finding will promote more effective treatment of this disease.
By the genetic composition of the detection of more than one multigenerational family with important types of FSGS, the researchers found something called TRPC6 (Transient Cycle Receptor Potential Cation Channel 6), a mutant form of the gene associated with the disease. Moreover, the function of this gene and related genes previously found in FSGS, these findings reveal a novel mechanism of renal injury. Targeting this ion channel drugs may effectively alleviate or prevent the scarring of the kidney. These channels are embedded in the membrane pore-like protein, able to control calcium flow. This gene represents the first with FSGS related ion channels. Winn et al. These findings are published in the May 5 online edition of Science magazine.
In the United States of FSGS incidence rate increases every year, and drug treatment of this disease is very limited and are non-specific drugs. So many patients to rely on dialysis to prolong life. Of FSGS etiology remains unclear, but previous findings suggest that the three other genes related with FSGS or class of FSGS disease. Previously identified genes responsible for the formation of the support membrane structural proteins. In 1999, the Duke research group identified in a New Zealand family with FSGS-related genomic regions.
In the latest study, researchers screened 106 individuals in seven generations and 600 members of the family, this narrowed it down to an exact gene - TRPC6. In this family, all FSGS members carry a TRPC6 gene mutation. Further study of variants of this gene in cultured kidney cells, this mutation responsible for the regulation of angiotensin II channel activity, angiotensin II can promote the occurrence of hypertension and kidney damage.
Although there are reports that TRPC6 mutations found in other hereditary FSGS family, but the findings on the role of this channel in kidney function problems bubbling to the surface. This channel may also be a new target for treatment of kidney disease.

On basic research in FSGS progress include

A plasma induced nephropathy factor previous evidence that the glomerular permeability factor is one of the reasons leading to FSGS, but has not clarified its essence. Infusion to rats with FSGS patient plasma, or serum extracts of staphylococcal protein A column can cause proteinuria. The extract of thermal instability, protease-sensitive, molecular weight of about 30 to 50 kDa, Podocytes in vitro incubation in plasma of patients with nephrotic syndrome enable podocytes of nephrin, podocin in and CD2AP molecules from the membrane translocation to the cytoplasm. Another study showed that normal human plasma in the plasma of patients with this role can be fully eliminated. Some scholars believe that there are protective factors in normal human plasma of FSGS patients lack the factor disease. The plasma in the end the existence of of FSGS protection molecules or pathogenic factor has not been fully elucidated.
The pathogenic role of podocytes in FSGS pathogenesis of podocytes gradually become the focus, the number of podocytes reduction is the main mechanism leading to glomerular sclerosis. The current positioning the podocyte hole membrane-associated molecules include: ① NPHS1 gene, the gene mutation is a major cause of nephrotic syndrome, Finnish type (CNF), and is mainly composed of two mutations (Fin major and Fin minor) due to massive proteinuria in NPHS1 gene knockout mice, their encoding nephrin molecules are also involved in enough cell signal transduction, including activation of the MAPK signaling pathway; ② The NPHS2 gene encoding podocin molecules can interact with nephrin and CD2AP may have a connection function of the gene mutation can lead to autosomal recessive inheritance of FSGS; ③ carrying CD2AP and its, it is located within the podocyte adapter molecules combine with the intracellular domain of the CD2 molecule, and nephrin anchored in the cytoskeleton, carrying CD2AP and its gene knockout mice manifested as nephrotic syndrome and renal insufficiency; ④ The ACTN4, which encodes the alpha-Actinin-4 is a muscle dynamic protein filaments cross-linked protein, is an important part of foot processes, to the maintenance of the skeleton of podocytes complete very important, ACTN4 gene mutations can cause autosomal dominant inheritance of FSGS, the ACTN4 knockout mice exhibit proteinuria and renal insufficiency; ⑤ The TRPC6, it encodes a calcium ion flow channel, TRPC6 gene mutations result in autosomal dominant genetic of FSGS.Found multiple mutations have contributed to the increase in calcium influx may therefore TRPC6 gene mutation as a functional (gain of function) pathogenic; ⑥ The PLCE1 gene, the gene mutation can lead to diffuse mesangial hyperplasia and FSGS lesions, thegene knockout zebrafish nephrotic syndrome.

Of FSGS treatment Introduction

What is of FSGS? Stands for focal segmental glomerulosclerosis, an onset of nephrotic syndrome in children and adolescents, but also result in adult renal failure.
How to the treatment of FSGS?
Why not promote the use of hormones? Proteinuria, a small ball hardening process of renal fibrosis due to FSGS appear, treatment, clinical medication Western conventional hormone immunosuppressive agents, such as hormones prednisone, to plug The betamethasone A strong nylon, etc.; commonly used immunosuppressants such as cyclophosphamide, tripterygium. These drugs can play an anti-inflammatory effects, but to control the disease is not only anti-inflammatory, but also expansion of the renal artery at all levels to solve the problem of renal hypoxia, but also anticoagulant, dilation of blood vessels after blood flow, but also degradation of glomerular sclerosis, to solve the problem of the filtration membrane, the above problem solving, as well as to impaired filtration membrane repair, only repair place, like proteinuria such external manifestations in order to completely disappear. For the treatment of FSGS, the hormone treatment not only side effects, the effect is often not ideal. So should resolve the issue will be a comprehensive treatment measures.
At the same time, the living diet should have a scientific system to develop programs to cope with the treatment of major principle with other nephropathy is a light diet, low salt, low fat, a small amount of high quality protein to avoid hot and spicy, the details mustbased on individual circumstances to the specific formulation.
The effect of micro-medicine treatment of FSGS?
Micro of traditional Chinese medicine treatment of FSGS with traditional treatment methods differ from traditional treatment methods tend to be directly aimed at the elimination of proteinuria to start, we are not at our hospital to treat the root cause for the cause of proteinuria. That is the focus of the treatment on the repair of the epithelial cells and mesangial cells.
Treatment measures is the integrated use of vasodilators, anti-inflammatory, anticoagulant, and measures of degradation of harmful substances. On the use of drugs, we are not simply use the single treatment of Chinese and Western medicines, but to maintain the foundation of Western medicine treatment to increase efforts to TCM treatment, and a new combination of methods, in addition to our treatment a major feature of our hospital in order to improve the efficacy of traditional Chinese medicines and the development of the hospital preparation, and that these agents played an important coordinating role in the process of TCM and Western medicine treatment. That treatment effects are very different.
We call this method: micro-based medicine blocking renal fibrosis infiltration method.Practice has proved that this treatment we can determine the exact effect of the repair of the glomerular capillary epithelial cells: damage to epithelial cells can be repaired, the condition will get better, "disease" is getting better, epithelial cell function will be restored, restore the function, proteinuria will naturally disappear. This function after the resumption of proteinuria disappeared, then the probability of recurrence will reduce.

New discovery of the new of familial FSGS susceptibility gene INF2 pathogenic mechanism

Recently, the international Biological Sciences Research top academic journals, "the U.S. National Academy of Sciences (PNAS), published online the latest achievements of the Shanghai Jiaotong University Affiliated Shanghai Children's Medical Center researchers. The new familial FSGS susceptibility gene the INF2 pathogenic mechanism for the new discovery "published article, the author is a child center pediatric into the Institute of Medicine, Department of Nephrology, the children's genetic disease task force member Dr. Sun Hua.
FSGS is a class performance for children and adults with kidney disease, chronic progressive glomerular diseases, the vast majority of clinical manifestations in patients with steroid resistant nephrotic syndrome. FSGS also contributed to the deterioration of renal function and chronic renal failure, the most important glomerular disease type, treatment antagonism exists, therefore, its pathogenesis and treatment strategies become hot and difficult in the field of diagnosis and treatment of kidney disease. The Shanghai Children's Medical Center is transformed into the Institute of Medicine Dr. Sun Hua group the use of cloning and mutation, as well as a series of protein interactions, the implementation of the pedigree linkage analysis was first discovered in the glomerular podocytes, INF2 negative adjustment of Rho / mDia was activated through interaction with the cell actin cytoskeleton regulatory protein mDia, cytoskeleton caused by heterogeneous and phenotypic changes of podocytes, thereby maintaining the normal function of podocytes, and confirmed that the INF2 pathogenic mutations interfere with this interactions lead to podocyte injury signal susceptibility, but also confirmed the new FSGS susceptibility gene INF2 mutations cause familial nephropathy. On this basis, the group further explore the pathogenic significance of these mutations, of FSGS susceptibility gene mutation is to affect or determine the clinical phenotype, disease progression, treatment response and prognosis, as well as important genetic factor for transplant relapse rate . The study comes from clinical cases of genetic data, from the molecular level interpretation of the INF2 mutations lead to the pathogenesis of FSGS, as well as the relationship of different mutations and different clinical phenotypes and prognosis. The study is also a very useful exploration of translational medicine and the practice of individualized treatment.
This research project has been the EPT of Shanghai Children's Medical Center of the Training Scheme funding; at the same time, Harvard Medical School hereditary kidney disease laboratory for this research project has also given strong support.

Progress in primary focal segmental glomerular sclerosis treatment

Focal segmental glomerulosclerosis (FSGS) is a group of similar pathological changes of chronic progressive glomerular disease, the characteristic pathological changes were part of the glomerular (focal, <50%, nearly marrow The glomerular easily sclerosis-like changes (matrix increase, capillary loop collapse) involved), part of the vascular loops (segmental vessels and vascular loops Yi involvement). FSGS was first described in 1957 by Rich in 1970 as an independent type of renal pathology. In recent years, renal biopsy data show that FSGS showed a trend of increasing year by year, and minimal change disease (MCD), mesangial proliferative glomerulonephritis (MsPGN) and FSGS can be transformed into each other. FSGS treatment response to glucocorticoids and cytotoxic drugs vary, their prognosis is directly related to the response to treatment, poor response to treatment are prone to chronic progressive renal damage, until the end stage renal disease (ESRD). The impact of FSGS is an important prognostic factor is the degree of proteinuria of FSGS's treatment objectives are: to reduce or eliminate proteinuria, prevent complications, slow down FSGS progress to ESRD. Are mainly elaborated primary FSGS treatment progress. 1 of FSGS glucocorticoid treatment.
The primary FSGS clinical performance as nephrotic syndrome, the majority reported that FSGS is not sensitive to hormone treatment, the response rate of eight weeks of the regular enough amount of hormone treatment of FSGS urinary protein less than 50% (average 20%). In recent years that, to extend the time of hormone therapy, may have more than 50% of FSGS in remission, glucocorticoid is still the first choice of FSGS treatment.
1 1 conventional method prednisone 1 5 2 0 mg / (kg • d), once every 4 to 8 weeks and achieved complete remission or partial remission (hormone-sensitive or sensitive part), prednisone can be gradually reduced the amount of maintenance treatment to more than 1 a or 1 a; sufficient quantities of prednisone treatment for four to eight weeks without remission (steroid-resistant), the joint use of immunosuppressive agents. 2 long course of hormone therapy program (1) Mendoza, program [4]: ​​① methylprednisolone intravenous pulse dose of 30 mg / (kg) (single maximum dose of 000mg): 1 - 2 weeks, every other day 1, 3 times / week; 3 - 10 weeks, 1 time / week; 11 - 18 weeks, every two weeks; 19 - 52 weeks, 1 times / month; 53 - 78 weeks, every 2 months to 1 year. ② prednisone oral dose of 2 mg / (kg) (single maximum dose of 60 mg): 1 - 2 weeks not 3 - 8 weeks, every other day, Dayton clothing, after the discretion to gradually decrease. The total course of treatment of men's-doza program, 5 a, the response rate and prognosis of urinary protein significantly improved compared with conventional hormone therapy program. (2) other long course of hormone therapy programs: the International Pediatric Nephrology Study Group (ISKDC) conventional treatment options recommended by the nephrotic syndrome did not distinguish between types of renal pathology, either MCD or of FSGS herein are prednisone 60 mg / (m * 2 d), in divided doses four weeks, followed by prednisone 40 mg / m 2, the next day orally for 4 weeks, tapering; the first 8 weeks of treatment of urinary protein-free remission are considered for steroid-resistant. Domestic pediatric prednisone 1 5 2 0 mg / (kg • d) After 8 weeks of treatment, remission, steroid-resistant criteria. However, the above criteria to judge the FSGS hormone resistance is not appropriate, and often lead to physicians reluctant to use hormone therapy, rather than FSGS real steroid-resistant, because the simple extension of hormone treatment can significantly improve urinary protein ease and prognosis. Experience in the treatment of adult FSGS: 8 weeks courses of sufficient quantities of hormones, sufficient quantities of hormone-induced FSGS urine protein remission time of 3 to 4 months of oral prednisone over 6 invalid before considering FSGS steroid-resistant.
But the lack of a long course of sufficient quantities of hormone treatment of FSGS samples and randomized controlled studies (RCT) data, its advantages and disadvantages to be subject to further evaluation, sufficient quantities of a long course of hormone therapy on children with adverse reactions (such as infection, growth developmental disorders, osteoporosis, high blood pressure and electrolyte imbalance, etc.), especially long-term effects can not be ignored.
2 combination therapy of FSGS
Of steroid resistance, dependence, frequency of recurrence of refractory primary of FSGS, in combination with other drugs to treat clinical inevitable choice.
2.1 cyclophosphamide (CTX) hormone the joint CTX treatment of FSGS common report, the CTX past as first-line drugs for the treatment of steroid-resistant FSGS. Rennert, etc. to the hormone oral and CTX intravenous pulse (at a dose of 500 mg / m * 2, the impact of 1 times / month) 6 months of treatment, results showed that 10 cases of steroid-resistant FSGS children, seven cases of complete remission, 1 cases partial remission (overall response rate 80%), two cases of no response. And CTX, Hari, etc. using the impact of hormone intravenous, oral treatment of 12 weeks, which, methylprednisolone 30 mg / kg or dexamethasone 5 mg / kg, every other day, six times every two weeks, four times, followed by a times / month, eight times, re-poured nylon oral tapering, were treated for 52 weeks. 59 cases of steroid-resistant FSGS patients with urinary protein / inosine values ​​from 10 0 to 0.75, serum albumin level rise by 19 g / L to 24 g / L; Among them, 17 cases of complete remission, eight cases of partial remission ; follow-up time of 3 of more than 34 cases, 22 cases (64.7%) with good prognosis, the majority of continuous complete remission. Gulati and so a prospective study: 1 / CTX shock, a dose of 500 to 750 mg / m * 2, while I poured nylon, four weeks before a dose of 60 mg / (m * 2 • d), then every other day taking splashed nylon (40 mg / m * 2) 4 weeks, decreasing the amount of 4 weeks is disabled. 20 cases of steroid-resistant FSGS patients, 13 cases of complete remission (65%), urine protein was negative CTX treatment time (12 ± 11.9) months follow-up time (21 2 ± 13 4) monthly urine protein continued negative. Bajpai and other every other day oral poured nylon and CTX intravenous pulse treatment of 24 cases of steroid-resistant FSGS patients, the CTX dose of 750 mg / m * 2, the impact of 1/6 months, only to find 7 cases of short-term (29. 2%) complete or partial remission, long-term follow-up only 5 cases (20.8%) remission, 17 cases of invalid (70.8%), and ease the untreated sensitive secondary steroid-resistant patients, suggesting that CTX on the original limited efficacy of drug resistance FSGS. Al Salloum et al reported 15 cases of steroid-resistant FSGS patients prednisone and CTX intravenous pulse therapy and were followed up for 4 years, prednisone 60 mg / (m * 2 • d) - 4 weeks, followed by every other day 40 mg / m * 2, 4 weeks, decreasing the amount of four weeks withdrawal; the CTX dose of 500 mg / m * 2, 1 / shock, 6 months. Were followed up for 4 years, 5 cases of primary resistant cases of treatment response (3 cases the development of chronic renal insufficiency), 10 cases of secondary resistance in patients with CTX pulse therapy is effective, but in the end are hormone-dependent, suggesting that hormone and CTX therapy for steroid-resistant FSGS, there is no significant effect, many studies also support the above conclusion.
Date, although some data show the efficacy of hormone and CTX treatment of drug-resistant FSGS, but still lack the RCT study, is generally believed that the subsequent secondary resistance in addition to the early governance hormone-sensitive FSGS patients have a certain effect, steroid and CTX treatment the efficacy of drug-resistant FSGS, there is no sufficient evidence. In addition, CTX adverse reactions (such as height, severe infections, hair loss, leukopenia, hemorrhagic cystitis, transient hypertension and drug injection, transient nausea, vomiting, etc.) must be given.
2.2 cyclosporine (CsA) of CsA is currently the treatment of FSGS have the exact effect of drugs, but usually require a longer course of treatment to maintain remission after renal toxic effects and withdrawal to recur is worthy of clinical attention. Mah-moud such as retrospective analysis of 106 cases to CsA treatment in patients with primary FSGS (45 cases of steroid-resistant, 61 cases of hormone dependent, of which 54 cases had received CTX treatment) of CsA starting dose of 6 mg / (kg • d), and gradually adjust the dose of CsA blood concentration maintained at 80 to 150 μg / L, treatment for 6 to 8 months after discontinuation of follow-up (6 ± 1 1 9) a. The rate of complete remission, partial remission rate and inefficiency were 71 7%, 7.5% and 20.8%. 31 cases but was discontinued in 91 cases of hormone relapse; 20 cases tried to stop the proteinuria disappeared CsA 16 cases immediately recurrence, and four cases, re-enable the CsA resistance. The results suggest that the significant effect of CsA on primary FSGS, but a higher relapse rate after stopping. Goumenos and other follow-up observation 5a CsA treatment, the efficacy of primary FSGS, with similar results. Recurrence of FSGS, Raafat large dose CsA treatment at a dose of 6 ~ 25 mg / (kg • d) of CsA by the low dose and gradually increase to remission or adverse renal effects until remission, CsA gradually reduction to the normal range, the results show a significant effect and can maintain for a long time in remission.
Long-term use of CsA adverse events (renal insufficiency, hypertension, gingival hyperplasia, hirsutism psychosis) is worthy of clinical concern. Chishti of a single small dose of CsA treatment of FSGS, the CsA dose (6 ± 0 8) mg / (kg • d), 1 / d, treatment time was 2 to 27 months without monitoring blood drug mass concentration. Results The total effective rate was 76% (16/21 cases), of which 52% (11/21 cases) in complete remission, partial remission 24% (5/21 cases), the onset time (2 ± 0 8 8 ) months; decreasing the amount of nine cases of complete remission of CsA withdrawal, including three cases of maintenance of remission (follow-up of 6 to 13 months), six cases of recurrence (follow-up 1.5 to 18. 7 months), recurrence of retreatment CsA or increased dose of CsA to sensitive. Treatment and follow-up period, CsA adverse reactions is small, suggesting that small-dose CsA treatment of FSGS is safe and effective. El - Husseini, 117 cases of children with primary FSGS to long-range small-dose CsA maintenance therapy of CsA starting dose is 5 mg / (kg • d), and gradually adjust the mass concentration of 1 to 2 months before the dose so that blood CsA Valley maintained at 100 ~ 150μg / L, after CsA plasma mass concentration maintained at 50 and with 100 μg / L, as long as proteinuria continue to ease of CsA blood concentration was maintained at 30 μg / L can also be low-dose CsA treatment time is 2 a (average 34 months), the results show a long time small dose CsA treatment of FSGS effective.
That CsA effectively reduce urinary protein and protection of renal function, used alone, especially with the hormone combination of CsA markedly hormone-sensitive FSGS, steroid-resistant and steroid-dependent children with primary FSGS have a certain effect. CsA as a steroid-dependent or steroid-resistant FSGS priority treatment options. CsA treatment should be emphasized that the individual differences of CsA dose can not be static, must adjust the dose according to blood concentration. It should be noted is easy to relapse after CsA withdrawal, and therefore adequate treatment, based on monitoring of adverse drug reactions (if necessary, repeat renal biopsy for renal tubulointerstitial injury), maintenance treatment is required more than 1 a.
2.3 other immunosuppressants new immunosuppressive agents try for the treatment of drug-resistant FSGS, including mycophenolate mofetil (MMF), he tacrolimus (FK506) and Manila neomycin, reported a lot, but Case, the efficacy of different, but its low toxicity and a more significant effect demonstrated its good prospects and look forward to the findings of the RCT. Joint and sequential use of immunosuppressive therapy for refractory of FSGS, is expected to achieve better results. Type of renal pathology, such as el - Reshaid, reported 21 cases of steroid-resistant MCD or FSGS patients with nephrotic syndrome treatment: the initial application of 12 weeks of CsA or of FK506, and then 3 months of MMF, and then 3 months MMF plus of CsA; if not complete remission in the MMF plus CsA on the basis of 1 / impact of CTX, three times in a row; complete remission plus complete remission after 3 times with 1 / CTX impact, every four months, decreasing the amount of MMF or CsA to the minimum dose to maintain urine protein negative; such as the above-mentioned program has not yet reached complete remission, repeated 1 time / month impact of CTX three times, plus methylprednisolone pulse therapy for 3 consecutive days. changed to oral prednisone and tapered. Immunosuppressants used in conjunction with the experience is not yet mature, but has a different mechanism of action combined with different immunosuppressive agents, and thus a synergistic therapeutic effect, will reduce the amount of immunosuppressants, and reducing the adverse effects of immunosuppressants advantage.
2.4 other ancillary drugs such as angiotensin-conversion enzyme inhibitors (ACEI) and (or) the use of angiotensin II receptor antagonist (ARB), as well as for high cholesterol lowering therapy, for a hypercoagulable state anti- coagulation therapy, have helped to reduce proteinuria and slow the deterioration of renal function and FSGS progress to ESRD can be used as adjuvant therapy for high blood pressure and antihypertensive treatment.
3 FSGS non-drug treatment Replacement of low-density lipoprotein (LDL), immunoadsorption and plasmapheresis can be used as adjuvant therapy for the treatment of refractory FSGS. In view of hyperlipidemia, especially high LDL effects on the kidneys, reported LDL replacement can make proteinuria reduced to improve the response rate and delaying the progress of renal pathology. Plasma replacement therapy can be used for the treatment of recurrent FSGS after kidney transplantation, plasma exchange before kidney transplantation, urine protein to reduce migration to maintain graft function have a certain effect; but the mechanism of humoral factors in FSGS is not yet clear. the efficacy of plasma exchange still need to be further determined.

The FSGS type iga nephropathy How effective treatment?

The FSGS type iga nephropathy How effective treatment?
IgA nephropathy is now ring out acyl flushing and hormonal therapy, was discharged due to the protein drop does not go home medication two days ago went to read Chinesenow drink and herbs can be? Eat hormone methylprednisolone and antihypertensive drugs Diovan and enalapril? How is it treated?
"How effective type of FSGS iga nephropathy treated?" Response
You are suffering from iga nephropathy FSGS is focal stage of hardening, long-term oralmedicine side effects of the body can not be ignored, and traditional Chinese medicineoral onset is slower. The above mentioned drugs are immunosuppressants, usually can control the symptoms and indicators, side effects are more suitable for long-term use fortreatment of now the key is to repair the damage of the control of symptoms at the sametime repair the kidney pathology protein and red blood cells and thus prevent leakage.So that the protein continued to leak not only can cause hypoproteinemia, severe also affect renal function, it is best to nephropathy specialty hospitals, the standard treatment,this will be more targeted. The key to the treatment from the treatment of kidney start to reach good results. Infiltration therapy of micro-based traditional Chinese medicine can be considered depending on your situation, not only remove the immune complexdeposition in the glomerular filtration membrane and diseased tissue, but also to repairglomerular filtration membrane and improve its permeability through the repair proteinand occult blood disappear naturally, not easy to relapse. As for which treatment optionsbut also your own according to their own situation specific choice, as long to find the most appropriate for your illness, treatment options, together with your usual diet control and regular exercise, you a big step toward rehabilitation.

FSGS treatment

What is of FSGS? Stands for focal segmental glomerulosclerosis, an onset of nephrotic syndrome in children and adolescents, but also result in adult renal failure.
How to the treatment of FSGS? Why not promote the use of hormones? Proteinuria, a small ball hardening process of renal fibrosis due to FSGS appear, treatment, clinical medication Western conventional hormone immunosuppressive agents, such as hormones prednisone, to plug The betamethasone A strong nylon, etc.; commonly used immunosuppressants such as cyclophosphamide, tripterygium. These drugs can play an anti-inflammatory effects, but to control the disease is not only anti-inflammatory, but also expansion of the renal artery at all levels to solve the problem of renal hypoxia, but also anticoagulant, dilation of blood vessels after blood flow, but also degradation of glomerular sclerosis, to solve the problem of the filtration membrane, the above problem solving, as well as to impaired filtration membrane repair, only repair place, like proteinuria such external manifestations in order to completely disappear. For the treatment of FSGS, the hormone treatment not only side effects, the effect is often not ideal. So should resolve the issue will be a comprehensive treatment measures.
At the same time, the living diet should have a scientific system to develop programs to cope with the treatment of major principle with other nephropathy is a light diet, low salt, low fat, a small amount of high quality protein to avoid hot and spicy, the details mustbased on individual circumstances to the specific formulation.
The effect of micro-medicine treatment of FSGS? Micro-based medicine treatment and traditional treatment methods differ from traditional treatment methods tend to be directly aimed at the elimination of proteinuria to start, we are not at our hospital to treat the root cause for the cause of proteinuria. That is the focus of the treatment on the repair of the epithelial cells and mesangial cells.
Treatment measures is the integrated use of vasodilators, anti-inflammatory, anticoagulant, and measures of degradation of harmful substances. On the use of drugs, we are not simply use the single treatment of Chinese and Western medicines, but to maintain the foundation of Western medicine treatment to increase efforts to TCM treatment, and a new combination of methods, in addition to our treatment a major feature of our hospital in order to improve the efficacy of traditional Chinese medicines and the development of the hospital preparation, and that these agents played an important coordinating role in the process of TCM and Western medicine treatment. That treatment effects are very different.
We call this method: micro-based medicine blocking renal fibrosis infiltration method.Practice has proved that this treatment we can determine the exact effect of the repair of the glomerular capillary epithelial cells: damage to epithelial cells can be repaired, the condition will get better, "disease" is getting better, epithelial cell function will be restored, restore the function, proteinuria will naturally disappear. This function after the resumption of proteinuria disappeared, then the probability of recurrence will reduce.

Brief for FSGS

FSGS refers to the glomerular capillary loops focal segmental sclerosis or hyaline degeneration, no significant cell proliferation of the glomerular capillary. May as the Department of
Mesangial proliferation, mesangial IgM deposition, and focal glomerulosclerosis, but minimal change nephropathy resistant to steroids, the consequences of recurrent chronic progress. There are also hormone invalid primary nephrotic syndrome of early renal biopsy is the focal glomerular sclerosis. Therefore, whether the disease as an independent glomerular disease is still controversial. However, representatives of other kidney disease type of clinical pathology, or as an independent disease, more common, and there is a growing trend.
(A) the primary focal glomerulosclerosis of unknown etiology.
(B) secondary focal glomerulosclerosis
1, glomerular diseases, heroin-associated nephropathy, tumor-associated nephropathy, diabetes, AIDS, hereditary nephritis, IgA nephropathy, preeclampsia and Hodgkin's disease.
2, tubular, interstitial and vascular disease, reflux nephropathy, radiation nephritis, analgesic nephropathy, and sickle cell disease.
3, other renal hypoplasia, obesity and old age and so on.
Not yet clear. Majority view that glomerular hemodynamic changes or basement membrane damage causes the ball mesangial overload intake the macromolecules caused by glomerular sclerosis. Human embryonic near medullary nephron occur early, large size, high filtration rate, capillary high-pressure, high filtration eventually lead to structural damage, the disease nearly medullary nephron damage early and severe.Segmental glomerular epithelial cell damage, the basement membrane anionic electrical barrier damage, chronic proteinuria overload, sustained high filtration, high perfusion will eventually lead to glomerulosclerosis. Glomerular hypertrophy and foam cell generation is important in the formation and development of the disease. 5/6 nephrectomy animal model, the glomerular capillary plasma flow and pressure, glomerular epithelial cells was significantly impaired in residual nephron hyperthyroidism, leading to hyalinization. Fogo primary focal glomerulosclerosis pathophysiology and clinical phase, it was found that the average glomerular area of ​​adult and children patients was significantly greater than the minimal change of the same age. Repeat renal biopsy also confirmed that some of the disease, expressed initially as small lesions, glomerular hyperplasia. Be seen in many patients with primary focal glomerulosclerosis, glomerular foam cells, it has the characteristics of the macrophage group, can be transformed by circulating monocytes or mesangial cells. Some cytokines and growth factors such as IL-1 alpha-TNF, IL-6 may play a role in the lead to glomerulosclerosis. There are animal studies found that serum cholesterol levels are related with the degree of hardening.
Immune damage is also involved in the occurrence and development of the disease, the immune pathological the glomerulosclerosis area visible IgM and C3 granular deposits. Electron microscopy showed sclerosis lesions have a large number of electron dense deposits. And the disease to recur in kidney transplantation.
(A) general treatment performance for massive proteinuria, edema, given the low-salt diet, the proper use of diuretics. Hypoalbuminemia obvious, appropriate use of albumin.High blood pressure significantly, sodium restriction, diuretic invalid, can be added, such as angiotensin converting enzyme inhibitors, calcium antagonists and other antihypertensive drugs.
(B) of hormones and other immunosuppressants
1, the hormone to nephrotic syndrome as the main performers, especially the original biopsy for small lesions, the development of focal segmental glomerular sclerosis, is still the preferred hormone, mostly favorable response, adult dosage, prednisone 0. 5 ~ 1mg / (kg · d), 6 to 8 weeks, then gradually reducing over to every other day therapy, the total course in one year or more. Pei reports prednisone for treatment of primary focal glomerular sclerosis, the complete remission rate of up to 47% of these patients 5-year kidney health survival rates significantly higher than the responders (96% vs 55%).Although there the data hormone plus cytotoxic effect is not better than a single hormone.However, most scholars advocate invalid, on the hormone-dependent and recurrent episodes should combination therapy. Cytotoxic drugs can significantly reduce the relapse rate and extend remission. And reduce the amount of hormones, and reduce its side effects. More choice of cyclophosphamide intermittent intravenous injection, total dose of <150mg/kg. Also oral chlorambucil In recent years, also with cyclosporine A treatment of this disease, recently some efficacy in the reduction or withdrawal process to recur. Expensive and potentially nephrotoxic, it is not appropriate for the drug of choice.
(C) treatment of angiotensin-converting enzyme inhibitor not only lower blood pressure, and can reduce urinary protein may be beneficial to delay renal failure. In addition, the disease associated with nephrotic syndrome is not only high-clotting disorder, there intrarenal coagulation, balloon adhesion, should be the anticoagulant therapy, such as: dipyridamole 25 to 75mg / d, China Flynn 2,5 mg / d can reduce protein urine, improve renal function.

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.

Do not want to reuse hormone, how to treat?

For the treatment of FSGS kidney disease, current clinical is still a lack of effective measures. Western medicine clinical application of hormone combination therapies for treatment. Corticosteroid therapy, a small number of patients with FSGS kidney disease (30% - 40%) of the condition can be eased. However, long-term use of hormone, there will be a lot of side effects attendant. Indeed, a large number of side effects of hormone produced by kidney patients on the treatment of their disease has concerns about the mental. Especially after the application of hormones or immunosuppressive agents in kidney patients discontinued the course of medication in the face of the predisposing factors (such as colds, fever, fatigue, allergies, etc.), kidney disease condition is very easy to relapse and increase.
Typically, FSGS kidney patients face hormone inevitably there are concerns about the psychological. Therefore, in search of better treatment methods, to avoid the side effects of hormones, become the common aspiration of the FSGS kidney patients. In fact, the micro therapy for the penetration of traditional Chinese medicine to break this traditional treatment model. Why do you say? Infiltration therapy of micro-based traditional Chinese medicine can effectively block the fibrosis of the kidney lesions, activation of cells in lesions of the kidney metabolic function, and mentioned the necessary nutrients as well as the rebuilt repair and reconstruction of damaged kidneys. Kidney area skin penetration into the body of the micro-penetration therapy of Chinese medicine to improve the repair environment can damage the kidneys, the gradual recovery of kidney disease, glomerular effective filtration, enhanced compensatory ability of the kidneys of healthy cells. In this way, the root causes of nephropathy proceed slowly to restore renal function, fundamentally eliminate the patient's urine protein.
However, for Miss Jiang is now applied hormone therapy, hormone medication principle limit, not the temerity to disable the hormone drugs. Because the hormone withdrawal there is a pattern, sudden withdrawal can cause hormone arrest reaction, exacerbate kidney disease. Therefore, you want to really get rid of the hormone, wants to fundamentally cure kidney disease, should be gradual infiltration therapy in the application of micro-based traditional Chinese medicine treatment of kidney disease during hormone dosage reduction, and ultimately achieve the purpose of treating kidney disease and completely get rid of the hormone.

What is the FSGS kidney disease? What are clinical manifestations of FSGS kidney disease?

Glomerular capillary nephrotic FSGS kidney disease called focal segmental glomerulosclerosis whole, refers to the glomerular capillary loops with focal segmental glomerular sclerosis or hyaline degeneration, no significant cell proliferation. FSGS kidney disease is a common pathological type of primary nephrotic syndrome, the pathological type 50% of patients with nephrotic syndrome FSGS kidney disease, renal biopsy can be clear of the disease.
The disease mostly occurs in children and young people, more men than women, a small number of patients with FSGS kidney disease before the onset of upper respiratory tract infection or allergic reaction history. In addition, the occurrence of FSGS nephrotic familial tendency FSGS kidney disease is relatively common in the atopic population, the main clinical manifestations and characteristics:
(1) The first clinical symptoms of nephrotic syndrome, microscopic to common hematuria, and occasionally gross hematuria in adults is about 2/3 of patients had mild persistent hypertension.
(2) urine routine examination, a small number of patients with FSGS kidney disease is asymptomatic proteinuria. Proteinuria and the vast majority of non-selective, but early high or moderate selectivity.
(3) blood test serum C3 levels were normal, IgG levels decline.
(4) of FSGS kidney patients more than the performance of the proximal renal tubular dysfunction, progressive decline of glomerular filtration rate, upper respiratory tract infection or allergic allows a variety of symptoms get worse.

Focal segmental glomerulosclerosis should do what?

A urine routine examination, microscopic hematuria, proteinuria, and often aseptic white blood cells in urine, grape diabetes. Impaired renal tubular function, urinary amino acids and phosphate in urine, its high incidence of other types of NS.
Blood tests have significantly lower serum albumin, serum albumin is usually less than 25g / L, and a few up to 10g / L below. Decline in glomerular filtration rate (GFR). BUN, creatinine increase. The majority of patients with hyperlipidemia. Serum C3 is usually normal IgG level decreased, C1q is mostly normal. 10% ~ 30% of patients positive for circulating immune complexes. Hypovolemia can cause the increase in hematocrit.Normal white blood cells and classification. Platelets slightly elevated. Water retention will result in lower sodium concentration, the long period of sodium or acquired adrenal insufficiency, can lead to lower sodium concentration. Hyperlipidemia can cause pseudo-hyponatremia, and platelets in vitro release of potassium ions, thrombocytosis can also cause pseudo hyperkalemia.
A renal biopsy light microscopy typical FSGS lesions characteristic focal segmental glomerular damage, lesions involving a small number of glomeruli and glomerular some segments of hyaline sclerosis. The lesions are often deep from the cortex or nearly medullary parts of the glomerulus began, and gradually extended to the renal cortex.Glomerular lesions showed segmental glomerular sclerosis, uninvolved normal of glomerular mesangial matrix increase. Hyaline material deposited in the damaged capillary loop endothelial cells, hardened area with occasional foam cell formation, proliferation of epithelial cells of the common limitations. Early lesions may only local epithelial cells and basement membrane from the epithelial cell swelling, vacuolar degeneration, basophilic cytoplasm. Hardening of the capillary loop wall adhesions with Bowman. Each segmental glomerular damage of a different range of disease progression may contribute to global sclerosis. Fully developed cases of lesions, easily mistaken for the "non-specific chronic sclerosing glomerulonephritis, and through immunofluorescence differential diagnosis. Renal tubular damage often appears as a focal thickening of the basement membrane and atrophy. Coexist, such as focal tubular damage and mild glomerular changes should be suspected of FSGS. Renal tissues of focal, global glomerulosclerosis FSGS often late performance, associated with severe tubulointerstitial lesions in pediatric patients up to 30%. The typical adult hormone-sensitive minimal change can be seen a small number of global sclerosis of glomeruli, with FSGS phase difference. In addition to primary FSGS, many diseases of the kidney tissue can be seen the similar FSGS change. FSGS may also overlap with primary glomerular diseases.
Electron microscopy a large number of proteinuria cases most or all of the glomerulus shows diffuse or segmental foot process change. Early visible in the capillary wall and (or) mesangial foam cells, mesangial matrix increase and part of the capillary collapse.Endothelial cells and mesangial area corresponding to the electron dense deposits, mesangial cell proliferation, large electron-dense material under the light microscope hyalinization and immunofluorescence IgM and C3 deposition. Ball collateral membrane area and endothelial cells can also be found fine granular electron dense deposits.
3 immunofluorescence sclerosis or necrosis can be found in the C3 or IgM and C1q was irregular, granular or nodular distribution. Glomerular lesions were negative. Occasionally mesangial have IgM and C3 distribution and IgG, IgA, rare.