ISSN: 1305-385X
Hakkında: Özel sayılar şeklinde yayınlanır.
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Focal Segmental Glomerulosclerosis
Dr. Saime PAYDAŞa
aNefroloji BD, Çukurova Üniversitesi Tıp Fakültesi, ADANA
FSGS is a glomerular disease seen both in childhood and adulthood and its incidence is increasing in recent years. The most important property of the disease is the absence of mesangial immune deposition and all the glomerules are not affected, disease is focal. Besides it involves some capillary loop. it is segmantary. It may be primary, familial or may be associated with HIV infection or pamidronate using. In addition, it has been defined some secondary FSGS types accounting to some medical conditions Collapsing glomerulopathy is a subtype of FSGS. Here, primary FSGS will be presented. Although the pathogenesis is not known, circulating permeability factors have been determined. In addition some podocin and cell-cell adhesion molecule gene mutations have been defined. The risks of recurrence and nephron loss after transplantation are high. The most common presenting manifestations are proteinuria and nephrotic syndrome. It may resemble minimal change disease at the first presentation. The presence of renal failure, tubular atrophy and tubulointerstitiel fibrosis is bad prognostic indicators. The response to treatment is not good. High dose steroid and immunosuppressives (cyclophosphamide, cyclosporine and others) are recommended. The most important therapeutic methods are strict control of blood pressure and the use of angiotensin converting enzyme inhibitors and/or angiotensin receptor blockers. Steroids should not be used in familial and secondary FSGS cases and in cases without heavy proteinuria,Keywords: Focal segmental glomerulosclerosis, proteinuriaTurkiye Klinikleri J Int Med Sci 2006, 2(21):15-24
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