局部節段性腎絲球硬化症之新進展 - tsim.org.tw

1 * .. (4 6 ) . 30 50% .. mycophenolate mofetil .. 20 50 .. focal segmental glomerulosclerosis . nephrotic syndrome). corticosteroid . end-stage renal disease).. Focal segmental Glomerulosclerosis FSGS .. corticosteroid . IgA . 1 FSGS 1957 Dr. Rich . 1. FSGS FSGS . FSGS . FSGS . 2. Mark Hass 1997 . FSGS membranous nephropathy . Minimal-change disease MCD 2 FSGS . FSGS .. end-stage renal disease ESRD 1 . FSGS 4 6 . FSGS .. 3 Cyclosporin A CsA . FSGS 3 FSGS. FSGS FSGS . FSGS . FSGS FSGS . FSGS . FSGS FSGS . FSGS . 1 . FSGS HIV heroin.

2 FSGS ESRD 2 . 1. 4 FSGS . mal-adaptation . agenesis dysplasia .. 2. 1 . FSGS FSGS. FSGS 1 FSGS . FSGS FSGS FSGS.. FSGS . FSGS FSGS . 7 15 15 20 10 40 . MCD FSGS . FSGS 2 . 1970 1980 . Mark Haas . 1997 2 20 . 1976 1979 1995 1997 2 1976. 1979 36% . 23% 15% 1970 1980 . 1995 1997 . 35% 33% . 15% 1976 1979. 9% 30%; 1995 1997. 25% 56% . FSGS FSGS . 45 67% FSGS 1976 1979. 1995 1997 FSGS FSGS . 2 . FSGS FSGS 55 60 . FSGS 21 15 . 3. 60% 40 FSGS 60 . 5 ( end-stage renal disease, ESRD ). 1-3. FSGS .. FSGS . 1, 3.. Hyalinosis .. 1, 3.

3 1, 3 .. IgM C1q C3 . IgG IgA 1, 3 . FSGS . 1, 4 .. 4.. 4 .. FSGS . adriamycin FSGS . 4. 1, 3 . FSGS MCD 20 FSGS. permeability factor 1, 3, 5 .. T (lymphokine) . 1, 5. FSGS.. 6 . 7 . FSGS FSGS. 2, 3. FSGS .. 1, 3 .. 68 88% 3gm 1, 3 . FSGS . 1, 3 . C3 IgG 1 C1q . 10 30 % . (immune complex) FSGS HLA-DR4 HLA-DR8 . 1 .. 1, 3, 7,. 8. 50 6 8 ESRD . 14gm 6 ESRD FSGS . 5. 3, 7,. 8. FSGS 2 3 ESRD . 1, 3. 10 80% . 1, 2 . ESRD 1- 3 Fuchshuber . FSGS ESRD . ESRD 10 . FSGS . FSGS .. 50% 10 ESRD 1 . ESRD . 1 . FSGS FSGS . FSGS FSGS . FSGS FSGS.

4 Angiotensin-converting enzyme inhibitor ACE inhibitor NSAID . FSGS 1, 3 . FSGS : . mycophenolate mofetil ACE inhibitor . 1 .. (sensitivity) (resistance) 1 . (equivalent dose) . 4 1 .. 1 . 6. FSGS . 4 6 . FSGS. FSGS . : ESRD 20 30% . ESRD 10 20% ESRD 50 . 70% FSGS 1 . 10 30 10 20 . 1, 3, 8, 1980 FSGS 20 1980 40 . 11-13.. 3, 12, 13. 8 1/3 6 . 3 4 . 6 . 3.. 30 50% 3 . 2mg 4 8 . 4 6 . FSGS .. 2 . cyclosporin A CsA FSGS . FSGS 1, 3, 14 CsA. FSGS CsA 3, 14, 15 . Korbet CsA . 3, 12. 69 . FSGS 17 CsA . 3 6 3, 14 CsA . Steroid-dependent CsA.

5 3, 14 CsA . 7. 1, 14.. FSGS CsA . 30 40 20 30 CsA . CsA 3, 14 CsA 4 5mg . 3, 14. 6 7mg CsA . CsA . 3 .. 3 . Cyclophosphamide, chlorambucil, azathioprine . 47 . 11. 59 22 . 19 . 55 . 11.. : Rennert cyclophosphamide . 16 . 4 mycophe ycophenolate mofetil FSGS . 17. CsA . 5 NSAID. NSAID . NSAID . NSAID NSAID . 1, 3, 18. NSAID . NSAID NSAID.. 6 ACE inhibitor 8. ACE inhibitor . (angiotensin receptor type 1 antagonists) . ACE inhibitor ESRD ACE inhibitor . FSGS ACE inhibitor . 24 19, 20 . ACE inhibitor NSAID 77. 19, 20. 27 1 mmol.

6 19 . 7 . FSGS Artero M FSGS . 20 50 FSGS 7 FSGS . 7. FSGS .. 3, 5, 15 FSGS . 15 . 3, 15 FSGS . 3, 7, 15 . FSGS . 7, 15.. CsA FSGS . CsA FSGS , 7, 15 . CsA . FSGS . FSGS collapsing variant .. 9. detachment . 4, 21 . ( 10gm) ESRD .. 4, 21 . 10 80 . 50 . 10 ESRD 1, 3 . 1 . 1, 2 . FSGS 15 ESRD . 20 . 60% 20 40% 1 . 300mg/ 5 15 . 1, 3. ESRD 80 ESRD . 36 11 . 3. 80 15 70 . 1 . : .. FSGS .. 10. 2 20 ( 4--6. ) 30 50% . 47 59 . 19 55 .. ACE inhibitor .. CsA FSGS .. 1. Glassock RJ, Chen AH, Alder SG: Primary glomerular disease: Focal glomerular sclerosis.

7 In: Brenner BM, Rector FC Jr, ets. The Kidney. 3 rd ed. Piladelphia: WB Saunders, 1996;. 14:46-52. 2. Mark Hass, SM. Meehan, TG. Karrison, and BH. Spargo: Changing etiology of unexplained adult nephrotic syndrome: A comparison of Renal Biopsy Findings from 1976-1979 and 1995-1997. Am J Kidney Dis. 1997; 30 (5), 621-631. 3. Sung-Feng WEN: Primary focal segmental glomerulosclerosis: Prognosis and therapeutic approaches. Acta Nephrol 1998;12: 49-60. 4. Itaru Kihara, Satoko Tsuchida, ad Eishin Yaoita: Podocyte detachment and epithelial cell reaction in focal segmental glomerulosclerosis wit cellular varients.

8 Kidney Int. 1997; 52, , 171-176. 11. 5. Savin VJ, Sharma R, Sharma M, et al: Circulating factor associated with increased permeability to albumin in recurrent focal segmental glomerulosclerosis. N Engl J Med 1996; 334:878-83. 6. Dantal J, Bigot E, Rogers W, et al: Effect of plasma protein adsorption on protein excretion in kidney transplant recipients with recurrent nephrotic syndrome. N Engl J Med 1994; 330:7 14. 7. Artero M, Biava C, Amend W, Tomiandovich S, Vincenti F: Recurrent focal glomerulosclerosis: Natural history and response to therapy.

9 Am J Med. 1992; 92:375-83. 8. Korbet SM, Schwartz MM, Lewis EJ: The prognosis of focal segmental glomerulosclerosis of adulthood. Medicine 1986; 65: 304-11. 9. Velosa JA, Holley KE, Torres VE, Offord KP: Significance of proteinuria on the outcome of renal function in patients with focal segmental glomerulosclerosis. Mayo Clin Proc 1983;. 58:568-77. 10. Fuchshuber A, Jean G, Gribouvalo, et al: Mapping a gene (SRN1) to chromosome 1q25-q31 in idiopathic nephrotic syndrome confirms a distinct entity of autosomal recessive nephrosis.

10 Hum Mol Genet 1995; 4: 2155-8. 11. Banfi G, Moriggi M, Sabadini E, Fellin GD Amico G, Ponticelli C: The impact of prolonged immunosupression on the outcome of idiopathic focal-segmental glomerulosclerosis with nephrotic syndrome in adults. A collaborrative retrospective study. Clin Nephrol. 1991;36:53-9. 12. Korbet SM, Schwartz MM, Lewis EJ: Primary focal segmental glomerular sclerosis; Clinical course and response to therapy. Am J Kidney Dis. 1994; 23; 773-83 . 13. Rydel JJ, Korbet SM, Borok RZ, Schwartz MM: Focal segmental glomerular sclerosis in adults.