Focal segmental glomerulosclerosis is one pathological type of nephrotic syndrome which mainly involve the renal glomeruli. It can occur to any age, but is more common among children and adolescents and there are more male patients than female patients.
Children FSGS patients are mainly minimal change disease and adult patients are mainly focal segmental nephritis, membraneous nephropathy and minimal change disease.
The first clinical symptom of FSGS is nephrotic syndrome. And there are different manifestations and prognosis for different types of FSGS.
About 2/3 patients have massive proteinuria and severe edema and daily urinary protein vary from 1-30g; more than 50% patients will have hematuria which in most cases can only be seen with microscope;
There are 30%-50% adult patients have mild but persistent hypertension or chronic nephritis with 24-h urinary protein less than 3.5g, slight edema, hematuria, high blood pressure and kidney failure;
More than 50% patients are manifested with nephrotic syndrome with obvious “three highs and one low” symptoms;
Some patients do not have obvious symptoms and proteinuria can be found by accident in urine urine tests. In this condition, proteinuria can last for long time and the prognosis is relatively better. However, some patients can develop into end stage renal failure. In most cases, the proteinuria is non-selective, but in the early stage it can be highly selective.
Typical FSGS with glomerular hypertrophy has small amount of urinary protein and there are only 10% will have elevated serum creatinine;
Cellular type FSGS often has large amount of proteinuria (>10g/d) and is more susceptible to progressing into renal failure. It has been reported that there are 60% cellular FSGS have serum creatinine higher than 2mg/dl.
Compressed FSGS has obvious proteinuria (often more than 10g/d) and renal failure in this type is more severe than other types. This type FSGS has rapid onset and progression and usually will enter into end stage renal failure within 1-2 years.
Children FSGS patients have similar clinical symptoms as adults. Most casees have slow progression and in the end will develop into renal failure, but about 10%-15% patients have rapid illness progression and develop renal failure earlier.
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