Buradasınız

Fokal Segmental Glomerüloskleroz

Focal Segmental Glomerulosclerosis

Journal Name:

Publication Year:

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.
147-151

REFERENCES

References: 

1. Appel GB, Pollak MR, D’Agati V: Focal segmental
glomerulosclerosis. In Feehally J, Floege J, Johnson
RJ, eds. Comprehensive Clinical Nephrology. 3 th ed.
Philadelphia, Mosby Elsevier, 2007; 217-230
2. Braun N, Schmutzler F, Lange C, Perna A, Remuzzi
G, Willis NS: Immunosuppressive treatment for focal
segmental glomerulosclerosis in adults. Cochrane Database
Syst Rev 2008; 16: CD003233
3. Crew RJ, Appel GB: Focal segmental glomerulosclerosis.
In Greenberg A, ed. The NKF Primer on Kidney Disease,
4th ed. Philadelphia, Elsevier, 2005; 178-182
4. Kitiyakara C, Eggers P, Kopp JB: Twenty-one year trend
in ESRD due to focal segmental glomerulosclerosis in the
United States. Am J Kidney Dis 2004; 44: 815- 825
5. Pollak MR: Focal segmental glomerulosclerosis: Recent
advances. Curr Opin Nephrol Hypertens 2008; 17: 138–
142
6. Huber TB, Schermer B, Benzing T: Podocin organizes
ion channel-lipid supercomplexes: Implications for
mechanosensation at the slit diaphragm. Nephron Exp
Nephrol 2007; 106: 27–31
7. Hinkes B, Wiggins RC, Gbadegesin R, Vlangos CN, Seelow
D, Nürnberg G, Garg P, Verma R, Chaib H, Hoskins BE,
Ashraf S, Becker C, Hennies HC, Goyal M, Wharram BL,
Schachter AD, Mudumana S, Drummond I, Kerjaschki D,
Waldherr R, Dietrich A, Ozaltin F, Bakkaloglu A, Cleper
R, Basel-Vanagaite L, Pohl M, Griebel M, Tsygin AN,
Soylu A, Müler D, Sorli CS, Bunney TD, Katan M, Liu J,
Attanasio M, O’toole JF, Hasselbacher K, Mucha B, Otto
EA, Airik R, Kispert A, Kelley GG, Smrcka AV, Gudermann
T, Holzman LB, Nürnberg P, Hildebrandt F: Positional
cloning uncovers mutations in PLCE1 responsible for a
nephrotic syndrome variant that may be reversible. Nat
Genet 2006; 38: 1397–1405
8. Fogo AB, Kashgarian M: Diagnostic atlas of renal
pathology. 1st ed. Spain: Elsevier-Saunders, 2005: 13-49
9. Stirling CM, Mathieson P, Boulton-Jones JM, Feehally
J, Jayne D, Murray HM, Adu D: Treatment and
outcome of adult patients with primary focal segmental
glomerulosclerosis in five UK renal units. Q J Med 2005;
98: 443-449
ŞENGÜL E ve ark: Fokal Segmental Glomerüloskleroz
Cilt/Vol. 18, No, 3, 2009 Sayfa/Page 147-151
150
Türk Nefroloji Diyaliz ve Transplantasyon Dergisi
Turkish Nephrology, Dialysis and Transplantation Journal
151
10. Chun MJ, Korbet SM, Schwartz MM, Lewis EJ: FSGS in
nephrotic adults: Presentation, prognosis, and response to
therapy of the histologic variants. J Am Soc Nephrol 2004;
15: 2169-2177
11. Pei Y, Cattran D, Delmore T, Katz A, Lang A, Rance P:
Evidence suggesting under- treatment in adults with
idiopathic focal segmental glomerulosclerosis. Regional
Glomerulonephritis Registry Study. Am J Med 1987; 82:
938-944
12. Ingulli E, Tejani A: Racial differences in the incidence
and renal outcome of idiopathic focal segmental
glomerulosclerosis in children. Pediatr Nephrol 1991; 5:
393- 397
13. Cameron JS: Focal segmental glomerulosclerosis in adults.
Nephrol Dial Transplant 2003; 18 [Suppl 6]: 45–51
14. Passerini P, Ponticelli C: Treatment of focal segmental
glomerulosclerosis. Curr Opin Nephrol Hypertens 2001;
10: 189-193
15. Malaton A, Valeri A, Appel GB: Treatment of focal
segmental glomerulosclerosis. Semin Nephrol 2000; 20:
309-317
16. Ponticelli C, Villa M, Banfi G, Cesana B, Pozzi C, Pani A,
Passerini P, Farina M, Grassi C, Baroli A: Can prolonged
treatment improve the prognosis in adults with focal
segmental glomerulosclerosis? Am J Kidney Dis 1999; 34:
618-624
17. Alexopoulos E, Stangou M, Papagianni A, Pantzaki A,
Papadimitriou M: Factors influencing the course and
the response to treatment in primary focal segmental
glomerulosclerosis. Nephrol Dial Transplant 2000; 15:
1348-1356
18. Ruf RG, Lichtenberger A, Karle SM, Haas JP, Anacleto
FE, Schultheiss M, Zalewski I, Imm A, Ruf EM, Mucha
B, Bagga A, Neuhaus T, Fuchshuber A, Bakkaloglu A,
Hildebrandt F: Patients with mutations in NPHS2 (podocin)
do not respond to standart steroid treatment of nephrotic
syndrome. J Am Soc Nephrol 2004: 15; 722-732
19. Mendoza SA, Reznik VM, Griswold WR, Krensky
AM, Yorgin PD, Tune BM: Treatment of steroid
resistant focal segmental glomerulosclerosis with pulse
methylprednisolone and alkylating agents. Pediatr Nephrol
1990; 4: 303-307
20. Tune BM, Kirpekar R, Sibley RK, Reznik VM, Griswold
WR, Mendoza SA: Intravenous methylprednisolone and
oral alkylating agent therapy of prednisone- resistant
pediatric focal segmental glomerulosclerosis: A long-term
follow-up. Clin Nephrol 1995; 43: 84-88
21. Heering P, Braun N, Mullejans R, Ivens K, Zauner I,
Fünfstück R, Keller F, Kramer BK, Schollmeyer P, Risler
T, Grabensee B: Cyclosporine A and chlorambucil in the
treatment of idiopathic focal segmental glomerulosclerosis.
Am J Kidney Dis 2004; 43: 10-18
22. Ponticelli C, Passerini P: The place of cyclosporin in the
management of primary nephrotic syndrome. BioDrugs
1999; 12: 327-341
23. Ponticelli C, Rizzoni G, Edefonti A, Altieri P, Rivolta E,
Rinaldi S, Ghio L, Lusvarghi E, Gusmano R, Locatelli F,
Pasquali S, Castellani A, Casa-Alberighi OD: A randomized
trial of cyclosporine in steroid-resistant idiopathic nephrotic
syndrome. Kidney Int 1993; 43: 1377-1384
24. Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl
MA, Hoy WE, Maxwell DR, Kunis C: A randomized trial
of cyclosporine in patients with steroid-resistant focal
segmental glomerulosclerosis. Kidney Int 1999; 56: 2220-
2226
25. Meyrier A, Noel LH, Auriche P, Callard P: Long-term renal
tolerance of cyclosporine A treatment in adult idiopathic
nephrotic syndrome. Kidney Int 1994; 45: 1446-1456
26. Mendizabal S, Zamora I, Berbel O, Sanahuja MJ, Fuentes J,
Simon J: Mycophenolate mofetil in steroid/cyclosporinedependent/
resistant nephrotic syndrome. Pediatr Nephrol
2005; 20: 914–919
27. Sahin GM, Sahin S, Kantarci G, Ergin H: Mycophenolate
mofetil treatment for therapy-resistant glomerulopathies.
Nephrology (Carlton) 2007; 12: 285–288
28. Tumlin JA, Miller D, Near M, Selvaraj S, Hennigar R,
Guasc A: A prospective, open-label trial of sirolimus in
the treatment of focal segmental glomerulosclerosis. Clin
J Am Soc Nephrol 2006; 1: 109–116
29. Tahzib M, Frank R, Gauthier B, Valderrama E, Trachtman H:
Vitamin E treatment of focal segmental glomerulosclerosis:
Results of an open-label study. Pediatr Nephrol 1999; 13:
649-652
30. Sharma RK, Sahu KM, Gulati S, Gupta A: Pefloxacin
in steroid dependent and resistant idiopathic nephrotic
syndrome. J Nephrol 2000; 13: 271-274
31. Matalon A, Markowitz GS, Joseph RE, Cohen DJ, Saal
SD, Kaplan B, D’Agati VD, Apel GB: Plasmapheresis
treatment of recurrent FSGS in adult transplant recipients.
Clin Nephrol 2001; 56: 271-278
32. Muso E, Mune M, Fujii Y, Imai E, Ueda N, Hatta K,
Imada A, Miki S, Kuwahara T, Takamitsu Y, Takemura T,
Tsubakihara Y: Low density lipoprotein apheresis therapy
for steroid-resistant nephrotic syndrome. Kansai-FGSApheresis
Treatment (KFLAT) Study Group. Kidney Int
1999; 7 (Suppl.): 122-125

Thank you for copying data from http://www.arastirmax.com