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Monday, May 7, 2012
What are the early symptoms of focal segmental glomerulosclerosis?
The disease mostly occurs in children and adolescents, men slightly more than women. A small number of patients before the onset of upper respiratory tract infection or allergic reactions. Most common clinical symptom is the nephrotic syndrome, about 2/3 of patients with massive proteinuria and severe edema, urine protein 1 to 30g / d, more than about 50% of patients with hematuria, microscopic hematuria common and occasionally gross hematuria. 30% to 50% of the adult patients with mild persistent hypertension or manifestations of chronic nephritic syndrome, patients with 24h urinary protein <3.5g / d, obvious edema, often hematuria, hypertension and renal insufficiency, while more than 50% were kidney ensemble performance significantly "three high and one low clinical manifestations. A small number of patients with no obvious symptoms, and occasionally found in routine urinalysis proteinuria. This type of asymptomatic proteinuria sustainable a long time, a better prognosis. A small number of patients with this type of gradual development of end-stage renal failure. Proteinuria and the vast majority of non-selective, but early high or moderate selectivity. Serum C3 levels were normal, and IgG levels decline. Often proximal tubular function is impaired performance.The above symptoms of upper respiratory tract infections or allergies can make worse.
Slight differences in the different pathological types of clinical manifestations of the disease, typical FSGS and glomerular hypertrophy, urinary protein less; cell FSGS often massive proteinuria (> 10g / d), and prone to renal insufficiency. Reported that 60% of the cells in FSGS patients with serum creatinine> 2mg/dl, while only 10% of patients in a typical FSGS serum creatinine increased. Collapsing type of FSGS also significant proteinuria, often> 10g / d and renal insufficiency are more serious than other types, and hypertension is relatively small. This type of rapid onset, rapid progression, usually 1 to 2 years after the onset into the end-stage renal failure (ESRF).
The clinical manifestations of pediatric patients and adults, mostly with nephrotic syndrome, while the proportion of the occurrence of hypertension and renal insufficiency than in adults is low. The majority (40% ~ 60%) of FSGS was chronic progressive progress, eventually leading to kidney failure, a small number of patients (10% to 15%) rapid progress of the disease, earlier onset of renal failure.
The diagnosis clinically and there is no reliable indicators should rely on renal biopsy in the diagnosis of FSGS and pay attention to rule out all possible secondary factors, such as HIV infection and drug abuse. Asked in detail about the history, physical examination and laboratory tests are helpful in differential diagnosis. For example, the performance associated with proximal renal tubular dysfunction in patients with nephrotic syndrome, or simple proteinuria; persistent nephrotic syndrome with hypertension, microscopic hematuria, non-selective proteinuria; hormone-sensitive patients should be suspected of FSGS. Renal biopsy examination can help diagnose typical focal segmental glomerulosclerosis (FSGS) characteristics for focal damage, the local impact of a small number of glomerular (focal) and glomerular (segmental). Start at nearly the medulla of the glomerular involvement, mild cases involving only a few capillary loop, re-spread to most of the glomerular lesions were uniform without cells or minimal hyalinization substance (within the loop foam cells, transparent drops), and severe cases, the balloon adhesions, visceral epithelial cell proliferation to form a "cap-like" structure, or even the "umbilical" lesions. Another focal glomerulosclerosis. The involvement of tubular epithelial cells of the nephron often shrinking, the surrounding matrix, see cell infiltration, fibrosis. Electron microscope, most of the glomerular or glomerular foot process effacement, epithelial cells and their foot processes and basement membrane from the endothelial cells and mesangial electron dense deposits at. Immunofluorescence in the hardened area to see IgM and C3 were irregular lumps, nodular deposition. Glomerular lesions were negative or diffuse IgM, C3 deposition, IgA, IgG rare. The disease often misdiagnosed as minimal change nephropathy, and it requires a combination of clinical manifestations and renal histological findings and response to hormone therapy and presence of spontaneous remission or drug induced remission in fully taken into account.Help the differential diagnosis of FSGS and MCD. Glomerular focal segmental sclerosis seen in FSGS can also be found in the development of a variety of chronic kidney disease, such as obstructive nephropathy, reflux nephropathy, AIDS patients and diamorphine addicts; can even be found in obese persons. Therefore, a comprehensive analysis to make the right diagnosis.
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