الفهرس 
الملخص العربى
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Abstract
Focal segmental glomerulosclerosis (FSGS) is a leading cause of adult nephrotic syndrome. On microscopy, there is scarring (sclerosis) in some glomeruli (focal) that involve only a portion (segment) of the glomerulus. FSGS has a histologic pattern of injury rather than a discrete pathologic entity. It is classified as “primary” or “idiopathic” if no cause is known, or “secondary,” if attributed to an underlying condition (eg, human immunodeficiency virus [HIV], hyperfiltration, malignancy, or toxin exposure). Patients with the primary form often present with nephrotic syndrome, characterized by proteinuria, edema, hypoalbuminaemia, and hypercholesterolemia. Most individuals with FSGS progress to end-stage renal disease (ESRD) within 5 to 20 years.
FSGS comprises 7% to 20% of biopsy-proven glomerular disease in children and adults. Formerly considered the second leading cause of adult nephrotic syndrome worldwide, some medical centers have reported FSGS to be the most common idiopathic glomerular disease.
Primary FSGS is more likely to present with sudden-onset nephrotic syndrome, while secondary FSGS presents more insidiously with subnephrotic range proteinuria and renal insufficiency.
Traditionally, the majority of FSGS cases are considered idiopathic, and the etiologies and mechanisms involved in FSGS development remain to be elucidated. However, the genetic basis for this disease, along with other inherited diseases, has burgeoned as a result of the mapping of the human genome. Understanding the role that genetics plays in focal segmental glomerulosclerosis is still evolving. However, some interesting observations have been made.
While secondary FSGS can be seen in hyperfiltration states, such as renal agenesis, obesity, or sickle cell disease. It may also result from intravenous drug abuse and reflux disease. It has been reported that toxins, including lithium and pamidronate, are associated with the development of FSGS lesions.
The prognosis is much worse in blacks compared to whites. In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESRD.
Idiopathic FSGS is a difficult disease to treat because of its highly variable clinical course. The specific treatment approach is still empirical.
Corticosteroids remain the mainstay of treatment, but this applies to the nephrotic forms of FSGS. Mildly proteinuric FSGS is a different entity and it would be of no avail, and in most cases harmful, to treat it with steroids.
Approximately 30% of patients experience remission of proteinuria with subsequent long-term stabilization of renal function after treatment with corticosteroids, optimal therapy for FSGS remains to be defined. Identification of steroid-responsive patients remains difficult and questionable.