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.