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Том 10 №1 2008 год - Нефрология и диализ

Фокально-сегментарный гломерулосклероз (Обзор литературы)

Наушабаева А.Е. Канатбаева А.Б. Диканбаева С.А.

Аннотация: Фокально-сегментарный гломерулосклероз (ФСГС) представляет собой целую группу заболеваний с различной этиологией, патогенезом и клиническими проявлениями, среди которых основной - протеинурия [43]. ФСГС является первой причиной нефротического синдрома (НС) у взрослых [32] и первой причиной стероид-резистентного НС (СРНС) у детей. В последние десятилетия отмечается рост частоты ФСГС по всему миру. При ФСГС выявляется поражение подоцитов (II) различной этиологии, в связи с чем болезнь стала рассматриваться как «подоцитопатии».

Для цитирования: Наушабаева А.Е., Канатбаева А.Б., Диканбаева С.А. Фокально-сегментарный гломерулосклероз (Обзор литературы). Нефрология и диализ. 2008. 10(1):15-20. doi:

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Ключевые слова: фокально-сегментарный гломерулосклероз, нефротический синдром, фактор проницаемости, подоциты, белки щелевой диафрагмы, нефрин, подоцин

Список литературы:

1. Abeyagunawardena A.S., Sebire N.J., Risdon R.A. et al. Predictors of long-term outcome of children with idiopathic focal segmental glomerulosclerosis. Pediatr Nephrol 2007; 22 (2): 215-221.
2. Adler S., Chen X. Anti-Fx1A antibody recognizes a beta-1-integrin on glomerular epithelial cells and inhibits adhesion and growth. Am J Physiol 1992; 262: F770-F776.
3. Ahola H., Heikkila E., Astrom E. et al. A novel protein, densin, expressed by glomerular podocytes. J Am Soc Nephrol 2003; 14: 1731-1737.
4. Barisoni L. et al. The dysregulated podocyte phenotype: a novel concept in the pathogenesis of collapsing idiopathic focal segmental glomerulosclerosis and HIV-associated nephropathy. J Am Soc Nephrol 1999; 10: 51-61.
5. Beaman M., Howie A.J., Harwicke J. et al. The glomerular tip lesion: a steroid responsive nephrotic syndrome. Clin Nephrology 1987; 27: 217-221.
6. Becker-Cohen R., Broschi M., Rinat C. et al. Recurrent nephrotic syndrome in homozygous truncating NPHS2 mutation is not due to anti-podocin antibodies. Am J Transplant 2007; 7 (1): 256-260.
7. Bertani T. et al. Adriamycin-induced nephritic syndrome in rats: sequence of pathologic events. Lab Invest 1982; 46: 16-23.
8. Boerkel C.F. et al. Mutant chromatin remodeling protein SMARCAL1 causes Schimke immuno-osseous dysplasia. Nat Genet 2002; 30: 215-220.
9. Braden G.L. et al. Changing incidence of glomerular diseases in adults. Am J Kidney 2000; 35: 878-883.
10. Cho M.E., Hurley J.K., Kopp J.B. Sirolimus therapy of focal segmental glomerulosclerosis is associated with nephrotoxicity. Am J Kidney Dis 2007; 49 (2): 310-317.
11. Cho M.E., Smith D., Branton M. et al. Pirfenidone Slows Renal Function Decline in Patients with Focal Segmental Glomerulosclerosis. Clinical Journal of the American Society of Nephrology, published ahead of print on August 16, 2007.
12. Chun M.J., Korbet S.M., Schwartz M.M. et al. Focal segmental glomerulosclerosis in nephrotic adults: presentation, prognosis, and response to therapy of the histologic variants. I Am Soc Nephrol 2004; 8: 2169-2177.
13. Couloures K., Pepkowitz S.H., Goldfinger D. et al. Preventing recurrence of focal segmental glomerulosclerosis following renal transplantation: a case report. Pediatr Transplant 2006; 10 (8): 962-965.
14. Coward R.J. et al. Nephrotic plasma alters slit diaphragm dependent signaling and translocates nephrin, podocin and CD2 associated protein in cultured human podocytes. J Am Soc Nephrol 2005;16: 629-637.
15. D’Agati V., Fogo A., Bruijin J., Jennette J. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. American Journal of Kidney Diseases 2004; 43: 368-382.
16. D’Agati V., Valeri A., Barfisoni L. et al. Idiopathic collapsing focal segmental glomerulosclerosis: A clinicopathologic study. Kidney International 1996; 50: 1734-1746.
17. D’Agati V. Pathologic classification of focal segmental glomerulosclerosis. Semin Nephrol 2003; 23: 117-134.
18. Daskalakis N., Winn M. Focal and segmental glomerulosclerosis: varying biologic mechanisms underline a final histopathologic end point. Semin Nephrol 2006; 26: 89-94.
19. Detwiler R.K., Falk R.J., Hogan S.L. et al. Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney International 1994; 45: 1416-1424.
20. Ehrich J.H.H., Geerlings C., Zivicnjak M. et al. Steroid-resistant idiopathic childhood nephrosis: overdiagnosed and undertreated. Nephrology Dialysis Transplantation 2007; 22 (8): 2183-2193.
21. Filler G. et al. Is there really an increase in non-minimal change nephritic syndrome in children? Am J Kidney Dis 2003; 42: 1107-1113.
22. Fine R.N. Recurrence of nephrotic syndrome/ focal segmental glomerulosclerosis following renal transplantation in children. Pediatr Nephrol 2007; 22 (4): 496-502.
23. Fujinaga S., Kaneko K., Muto T. et al. Independent risk factors for chronic cyclosporine induced nephropathy in children with nephrotic syndrome. Arch Dis Child 2006; 91 (8): 666-670.
24. Ghiggeri G.M., Aucella F., Caridi G. et al. Posttransplant recurrence of proteinuria in a case of focal segmental glomerulosclerosis assotiated with WT1 mutation. Am J Transplant 2006; 6 (9): 2208-2211.
25. Ghiggeri G.M. et al. Permeability plasma factors in nephritic syndrome: more than one factor, more than one inhibitor. Nephrology Dialysis Transplantation 2001; 16: 882-885.
26. Guttierez-Millet V., Nieto J., Praga M. et al. Focal glomerulosclerosis and proteinuria in patients with solitary kidneys. Arch Intern Med 1986; 146: 705-709.
27. Hall A.M., Unwin R.J. The not so «mighty chondrion»: emergence of renal diseases due to mitochtondrial dysfunction. Nephron Physio 2007; 105 (1): 1-10.
28. Howie A.J., Brewer D.B. The glomerular tip lesion: a previously undescribed type segmental glomerular abnormality. J Pathol 1984: 142.
29. Hristea D., Hadaya K., Marangon N. et al. Successful treatment of recurrent focal segmental glomerulosclerosis after kidney transplantation by plasmapheresis and rituximab. Transpl Int 2007; 20 (1): 102-105.
30. Huber T.B., Kottgen M., Schilling B. et al. Interaction with podocin facilitates nephrin signaling. J Biol Chem 2001; 276: 4153-4156.
31. Kambham N., Markowitz G.S., Valeri A.M. et al. Obesity-related glomerulopathy: an emerging epidemic. Kidney International 2001; 59: 1498-1509.
32. Kanjanabuch T., Lewsuwan S., Kitiyakara C. et al. Update in pathophysiology and histopathology of focal segmental glomerulosclerosis. J Med Assoc Thai 2006; 89, Suppl. 2: S262-79.
33. Kaplan J.M., Kim S.H., North K.N. et al. Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis. Nat Genet 2000; 24: 251-256.
34. Kerjaschki D. Caught flat-footed: podocyte damage and the molecular bases of focal glomerulosclerosis. J Clin Invest 2001; 108: 1583-1587.
35. Kojima K., Kerjaschki D. Is podocyte shape controlled by the dystroglycan complex? Nephroloy Dialysis Transplantation 2002; 17 (Suppl. 9): 23-24.
36. Korbet S.M. Treatment of primary focal segmental glomerulosclerosis. Semin Nephrol 2002; 62: 2301-2310.
37. Kris W., Le Hir M. Pathways to nephron loss starting from glomerular diseases - insights from animal models. Kidney International 2005; 67: 404-419.
38. Kuhn C., Kuhn A, Markau S. et al. Effect of immunoadsorbtion on refractory idiopathic focal and segmental glomerulosclerosis. J Clin Apher 2006; 21 (4): 266-270.
39. Li R.M. et al. Molecular identification of SV40 infection in human subjects and possible association with kidney disease. J Am Soc Nephrol 2002; 13: 2320-2330.
40. Markowitz G.S., Appel G.B., Fine P.L. et al. Collapsing focal segmental glomerulosclerosis following treatment with high-dose pamidronate. J Am Soc Nephrol 2001; 12: 1164-1172.
41. McCarthy et al. Sera from Patients with collapsing focal segmental glomerulosclerosis increase albumin permeability of isolated glomeruli. J Lab Clin Med 2004; 143; 225-229.
42. Meyrier A. E pluribus unum: the riddle of focal-segmental glomerulosclerosis. Semin Nephrol 2003; 23: 135-140.
43. Meyrier A. Mechanisms of disease: focal segmental glomerulosclerosis. Nat Clin Pract Nephrol 2005; 1: 44-45.
44. Meyrier A. Nephrotic focal segmental glomerulosclerosis in 2004: an update. Nephrology Dialysis Transplantation 2004; 10: 2437-2444.
45. Moudgil A. et al. Association of parvovirus B19 infection with idiopathic collapsing glomerulopathy. Kidney International 2001; 59: 2126-2133.
46. Mundel P., Heid H.W., Mundel T.M. et al. Synaptopodin: an actin-associated protein in telencephalic dendrites and renal podocytes. J Cell Biol 1997; 139: 193-204.
47. Nair R. Focal segmental glomerulosclerosis: cellular variant and beyond. Kidney International 2006; 70: 1676-1678.
48. Paik K.H., Lee B.H., Cho H.Y. et al. Primary focal segmental glomerulosclerosis in children: clinical course and prognosis. Pediatr Nephrol 2007; 22 (3): 389-395.
49. Pavenstadt H. et al. Cell biology of the glomerular podocyte. Physiol Rev 2003; 83: 253-307.
50. Pescovitz M.D., Book B.K., Sidner R.A. Resolution of recurrent focal segmental glomerulosclerosis proteinuria after rituximab treatment. N Engl J Med 2006; 354 (18): 1961-1963.
51. Praga M., Morales E., Herrero J.C. et al. Absence of hypoalbuminemia despite massive proteinuria in focal segmental glomerulosclerosis secondary to hyperfiltration. Am J Kidney Dis 1999; 33: 52-58.
52. Reusz G., Szabo A., Fekete A. Nephrotic syndrome in childhood. Orv Hetil 2006; 147 (47): 2251-2260.
53. Sanna-Cherchi S., Somenzi D., Carnevali M.L. et al. Recurrent autosomal-dominant focal segmental glomerulosclerosis. Kidney International 2006; 70 (9): 1664-1665.
54. Saxen L. Organogenesis in the Kidney. Cambridge University Press: Cambridge, 1997.
55. Schwartz M.M. et al. Focal segmental glomerulosclerosis: prognostic implications of the cellular lesion. J Am Soc Nephrol 1999; 10: 1900-1907.
56. Shankland S.J., Al-Douahji M. Cell cycle regulatory proteins in glomerular disease. Exp Nephrol 1999; 7: 207-211.
57. Shankland S.J. The podocyte’s response to injury: Role in proteinuria and glomerulosclerosis. Kidney International 2006; 69: 2131-2147.
58. Shatat I.F., Schoeneman M., Flynn J.T. et al. Association of steroid and cyclosporine resistance in focal segmental glomerulosclerosis. Pediatr Nephrol 2007; 22 (6): 834-839.
59. Shih N.Y., Li J., Cotran R. et al. CD2AP localizes to the slit diaphragm and binds to nephrin via a novel C-terminal domain. Am J Pathol 2001; 159: 2303-2308.
60. Smeets B., Steenbergen M.L., Dijkman H.B. et al. Angiotensin converting enzyme inhibition prevents development of collapsing focal segmental glomerulosclerosis in Thy-1.1 transgenic mice. Nephrol Dial Transplant 2006; 21 (11): 3087-3097.
61. Stokes M.B., Markowitz G.S., D’Agati V.D. Cellular focal segmental glomerulosclerosis: Clinical and pathologic features. Kidney International 2006; 70: 1783-1792.
62. Stokes M.B., Markowitz G.S., Lin J. et al. Glomerular tip lesion: a distinct entity within the minimal change disease/ focal segmental glomerulosclerosis spectrum. Kidney International 2004; 65: 1690-1702.
63. Tanawattanachaoren S. et al. Parvovirus B19 DNA in kidney tissue of patients with focal segmental glomerulosclerosis. Am J Kidney Dis 2000; 35: 1166-1174.
64. Thomas D.B., Franceschini N., Hogan S.L. et al. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney International 2006; 69: 920-926.
65. Tomlinson L. et al. Acute cytomegalovirus infection complicated by collapsing glomerulopathy. Nephrology Dialyis Transplantation 2003; 18: 187-189.
66. Valeri A., Barisoni L., Appel G.B. et al. Idiopathic collapsing focal segmental glomerulosclerosis: a clinicopathologic study. Kidney International 1996; 50: 1734-1746.
67. Varghese S.A., Powell T.B., Budisaljevic M.N. et al. Urine biomarkers predict the cause of glomerular disease. J Am Soc Nephrol 2007; 18 (3): 913-922.
68. Woroniecki R.P., Shatat I.F., Supe K. et al. Urinary Cytokines and Steroid Responsiveness in Idiopathic Nephrotic Syndrome of Childhood. Am J Nephrol 2007; 28 (1): 83-90.

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