In recent years, the morbidity of primary FSGS have an increasing tendency according to reports, the primary FSGS has an increase from 10% climbing to 25% among the adult primary glomerulonephritis. Focal Segmental Glomerulosclerosis is a common pathological type of Nephrotic Syndrome, and it is a description of pathology. Focal characteristics mean the disease lesion is not distributed diffusely and the suffering glomeruli are usually no more than 50%. Segment means that not the entire glomeruli is all suffered and the lesion part is usually no more than 50% of the entire glomeruli. And for the sclerosis, it means the glomerular segmental hyaline degneration or the formation of cicatrization.Back top
FSGS is a medical term used to describe the appearance of the kidney tissues on biopsy. Routine urine test and routine blood test help to found out how well the kidney functions, but fail to show the pathological changes. Renal biopsy is a gold standard to know kidney damages well, but disappointedly, it causes kidney damages while works. Therefore, renal biopsy usually is not recommended, especially for kidney failure patients.
In the diagnosis for FSGS, it should be identified with the traditional Minimal Change Disease(MCD). Paying attention to the following aspects is helpful for the diagnosis of FSGS. First, hypertension and renal function damage are nearly accompanied by the onset of the FSGS, which is more common seen in FSGS than in MCD. And this is more obvious in adult patients.
Second, the morbidity rate for microscopic haematuria in FSGS is also higher than that in MCD patients. In fact, about 2/3 FSGS patients are accompanied by microscopic hematuria.
Third, MCD patients commonly see the selective proteinuria while FSGS patients see more unselective proteinuria.
Fourth, renal tubules damage is often accompanied by with FSGS patients.
Fifth, the level of IgG in patient blood serum is obviously lower than that lost in the urine, which indicates the lowing blood IgG level is possibly related with the patients' abnormal immune function.
4.Anti-histone Antibody: Anti-histone Antibody can be tested in Lupus Nephritis and sometime in rheumatic arthritis and Sjoegren syndrome.
Sixth, FSGS patients have a more weaker sensitivity to the hormone treatment than the MCD.
Seventh, the size of glomeruli in patients with FSGS is larger than that in MCD patients.Back top
The segmental sclerosis of glomeruli reflects the result of damage reparation of local inflammation. The simple episodic sclerosis itself cannot reflect the feature of primary damage. Primary FSGS is often accompanied by the harms of glomrular epithelial cells. The epithelial cells of visceral layer, or you can call it podocyte, plays an important function in the onset of the primary FSGS.
Under the function of various factors such as toxins, inflammation harms, the epithelial cells happen with damage and podocyte happens with coalesce, and also, the glomerular filtration membrane permeability would increase, thus leading to the heavy proteinuria. Epithelial cells can produce various kinds of cell factor and growth factor, especially the TGF-B. TGF-B promotes the increase of synthesis of collagen from epithelial and mesangial cells and also lead to the decrease of degradation, therefore leading to the glomerular sclerosis.
Besides, the high filtration and high perfusion also play an important function in the formation of FSGS. High filtration and high perfusion irritate the renin-angiotensin system, thus leading to the increase of proliferation of mesangial cells and the ECM, so glomerular sclerosis is aggravated.Back top
Mostly. FSGS presents as a Nephrotic Syndrome. According to statistics, about two-thirds of patients have heavy proteinuria and serious swelling, and the urine protein usually is about 1-30g/d. Nearly 50% patients show hematuresis and in general, microscopic hematuresis is more common. About 30-50% patients have light degree of hypertension (high blood pressure) or presented as chronic nephritis syndrome.
Some patients have no obvious symptoms, and proteinuria is occasionally found during urine routine. And this kind of asymptomatic proteinuria could last for a long time and prognosis for this type is good. Some minority patients may gradually progress into end stage renal failure.
Children sufferers have a similar clinical symptoms with adults. And they are mainly presented with Nephrotic Syndrome. The morbidity rate for high blood pressure and renal insufficiency in children is lower than in adults. Most of FSGS(40-60%) progress with presentations of chronic feature and it may progress to kidney failure at last. Some minority patients(10-15%) progress quickly so patients could show renal failure at an early time.Back top
Due to the FSGS is a relatively more serious type of Nephrotic Syndrome type, so diet is another important aspect for the recovery of the disease.
Intake of Protein:Protein intake plays an important function in the diet plan for FSGS patients. Due to the loss of large amount of protein in FSGS, so extra protein should be supplemented timely so as to satisfy the body need. Enough protein intake can also make your body strong enough to resist infections or other diseases.
Intake of Water:Water intake should be made a balance between the body need as well as the kidneys' ability in removal of it.
Intake of Sodium:In order to avoid aggravating the conditions of high blood pressure and swelling in patients, water and sodium intake should be both restricted. You'd better say "no" to salty foods, such as salted drinking, pickles, walnuts, and salted cashew nuts, etc. Low-sodium foods include rice, cereals, plain breads, and so on.
Intake of Fat and Cholesterol:If you do not pay attention to the control of the intake of fat and cholesterol, you may experience the complications. You should limit the intake of eggs for 2g per week but you need to take lean meat, poultry, fish. Animal oil should replace the healthy oil and the saturated fats.
In a conclusion, FSGS diet plays an important part in the recovery of the FSGS, therefore, patients should pay attention to these dietary details and ask for your doctor or dietitians for more specific diet details so as to make you better recovery.Back top
FSGS can not be cured totally, but many treatments can be applied to stop FSGS effectively.
Conventional Treatments for FSGS
Nowadays you can use the glucocorticoid or other immune suppressants to reduce the glomerular filtration membrane permeability so as to achieve the purpose of reducing proteinuria.
For those patients with FSGS who are unable to achieve a bettSines to improve the effect.
There is a report that FSGS progresses to ESRD very fast after the use of hemodialysis together with high-dosage of prednison, in the later, after the use of plasmaphoresis along with the low-dosage of cyclophosphamide, the renal function restores obviously, and the following two years follow up proved that the patient still had a stable renal function and during that time, hemodialysis is never undergone during that time.
Hormone and cytotoxicity drugs are the currently common used drugs for the treatment of FSGS. And the new medicine of mycophenolic acid is also praised for its treatment for FSGS. Compared with the traditional treatment plan for FSGS, mycophenolate mofetil(MMF) along with prednisolone for 2-3 months can quickly induce the relieve of the clinical manifestations, reduce the using time of hormones, and reduce the untoward effect of hormones.
And studies show that ACEI could effectively reduce the proteinuria, and along with the extend of treatment, the function of ACEI becomes more and more obvious in reduction of the proteinuria. There are reports that when the traditional treatment is ineffective in treatment for FSGS, the joint application with MMF and ARB could effectively alleviate the clinical symptoms of FSGS.
As for the relapse FSGS after kidney transplant, the effect of blood purification treatment is relatively satisfactory. Especially the double filtration plasmapheresis(DFPP), it could reduce the level of proteinuria as well as extend the survival rate of transplanted kidney.
Immunotherapy- newest treatment for FSGS
Aside from the above methods, there is another therapy which halts FSGS progress to kidney failure, and that is Immunotherapy.
Immunotherapy is a relatively new therapy which combines immunosuppressive agents and Chinese medicines. It is widely known that long-term application of hormone medicines will give rise to various side effects, so it is necessary to avoid long-term application of hormone medicines. Although Immunotherapy can not cure FSGS, but compared with hormone therapy, Immunotherapy is more advisable, as it is free of side effects. Besides this, another reason for Immunotherapy to be advantageous among FSGS patients is that there is no relapse of FSGS after Immunotherapy.
In the process of treating FSGS, Immunotherapy also involves hormone medicines, but differently with conventional treatment, patients with Immunotherapy need not to rely on hormen medicines. They stop after a short time usage of these medicines, thereby, there is no worries about any side effects.
In Immunotherapy, Chinese medicines are used to regulate patient's immune system after application of Immunosuppressive agents. FSGS is a medical term that describe kidney damages in the angle of pathological changes. Kidney damages occurs because of immoderate inflammatory response which occurs only when there is antigens, immune complexes or other harmful substances in kidneys. With function of extension of blood vessels, anti-inflammation, anti-coagulation and degradation of extracellular matrixes, FSGS patient's immunity and self-curative ability can be strengthened greatly. Under such a condition, no antigen invade patient's body again and kidney damages are remitted as well, as a result of which, kidney function increases and progression of FSGS is stopped fundamentally.
Dialysis and Kidney Transplantation
Generally, dialysis and kidney transplantation are adopted when FSGS develops to kidney failure. However, not everyone with kidney failure will certainly suffer from dialysis and kidney transplant. According to study, only these whose kidneys have been damaged totally and with no urine for at least 2 years need to live their rest life by dialysis or kidney transplant.Back top
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