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Fokal Segmental Glomerüloskleroz

Focal Segmental Glomerulosclerosis

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Abstract (2. Language): 
Focal segmental glomerulosclerosis (FSGS) is a common histological aspect of glomerular injury due to diverse etiologies. It is the most common cause of end stage renal disease (ESRD) in primary glomerulonephritis. The incidence of ESRD due to FSGS has increased over several decades. Most patients with FSGS present with asymptomatic proteinuria or nephrotic syndrome. In primary FSGS, macroscopic hematuria is very rare, but microscopic hematuria usual. At the diagnosis time, glomerular filtration rate is reduced in 20 to 30 %of patients. In series, half of patients has been demonstrated to develop ESRD within 5 years. It has been suggested that several epidemiologic, clinic, and initial histological findings may be useful to indicate the long term course of FSGS. Treatment with angiotensin converting enzyme (ACE) inhibitors and/or angiotensin II type 1 receptor (ARB) blockers instead of aggressive immunosupressive treatment is recommended for patients with non nephrotic proteinuria and mild renal biopsy findings. On the other hand, patients with nephrotic proteinuria and poor prognostic factors should be treated with corticosteroids for at least 6 months. Cyclosporine, cyclophosphamide, and mycophenolate mophetil are suggested as the alternative therapeutic drugs for patients with steroid resistant FSGS.
Abstract (Original Language): 
Fokal segmental glomerüloskleroz (FSGS), çeşitli nedenlere bağlı olarak gelişen glomerül hasarının ortak histolojik bir bulgusudur. Primer glomerülonefritler içinde son dönem böbrek yetmezliğinin (SDBY) en sık nedenidir. FSGS’ye bağlı SDBY insidansı son dekatlarda artmıştır. Hastaların çoğu asemptomatik proteinüri veya nefrotik sendrom ile ortaya çıkmaktadır. Primer FSGS’de makroskopik hematüri çok nadirdir, fakat mikroskopik hematüri sıklıkla saptanır. Tanı anında, hastaların %20-30’unda glomerül filtrasyon hızı azalmıştır. Serilerde, hastaların yarısında 5 yıl içinde SDBY geliştiği gösterilmiştir. Bazı epidemiyolojik, klinik ve başlangıçtaki böbrek biyopsi bulgularının FSGS’nin uzun dönemdeki seyrini belirlemede faydalı olabileceği belirtilmektedir. Nefrotik düzeyde olmayan proteinüri ve böbrek biyopsi bulguları hafif olan hastalarda agresif immünosüpresif tedavi yerine anjiotensin dönüştürücü enzim (ACE) inhibitörleri ve/veya anjiotensin II tip 1 reseptör (ARB) blokerleri ile tedavi önerilmektedir. Diğer taraftan, nefrotik düzeyde proteinürili ve kötü prognostik belirtileri olan hastaların en az 6 ay süre kortikosteroidler ile tedavisi önerilmektedir. Siklosporin, siklofosfamid ve mikofenolat mofetil steroid direnci olan hastalar için alternatif tedavi edici ilaçlar olarak önerilmektedir.
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