Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
권호 표지

소아 IgA 신병증의 임상병리학적 양상과 예후

Clinicopathologic features and prognosis of childhood IgA nephropathy

  • 우성일 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 배근욱 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 이주훈 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 박영서 (울산대학교 의과대학 서울아산병원 소아과) ;
  • 조영미 (울산대학교 의과대학 서울아산병원 병리과)
  • Woo, Sung Il (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Bae, Keun Wook (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Joo Hoon (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park, Young Seo (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Cho, Yong Mee (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine)
  • 투고 : 2006.09.13
  • 심사 : 2006.12.15
  • 발행 : 2007.02.15
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

목 적: 소아 IgA 신병증을 발병시 임상 양상 및 병리학적으로 분류하여 이들의 임상 경과와 예후 인자를 조사하고자 한다. 방 법: 1991년부터 2005년까지 서울아산병원 소아과에서 신생검으로 IgA 신병증으로 진단되고 1년 이상 추적 관찰한 61명의 환아를 대상으로 후향적으로 조사하였다. 결 과: 환아들의 발병시 평균 연령은 9.3세였고, 평균 추적관찰 기간은 5.2년 이었다. 전체 61명의 환아 중 남아가 42명 여아가 19명이었다. 최종 추적 관찰시 24명(39.3%)는 정상 소견을 보였고, 30명(49.2%)은 혈뇨 또는 경도의 단백뇨(<$1g/m^2/d$), 5명(8.2%)은 중증도의 단백뇨(${\geq}1g/m^2/d$), 2명(3.3%)은 만성 신부전을 보였다. 추적 관찰 중 지속되는 고혈압의 여부는 임상 경과와 통계적으로 유의한 상관관계가 있었다(P<0.01). Haas 분류는 임상 경과와는 상관관계가 없었다. 그러나 20% 이상의 사구체에서 전경화나 분절 경화 또는 반월체 형성이 있었던 경우 60%에서 지속적인 중증도의 단백뇨와 만성 신부전의 소견을 보여 유의한 상관관계를 보였다(P<0.01). 결 론: 소아 IgA 신병증의 단기간의 추적 관찰 중 임상 경과 및 예후는 비교적 양호하다. 그러나 추적 관찰 중 지속적인 고혈압 및 신생검시 20% 이상의 사구체에서 전경화나 분절 경화 또는 반월체 형성이 있는 경우에서는 나쁜 예후 인자로서 유의한 상관 관계를 보였다. 앞으로 소아 IgA 신병증의 특징에 맞는 조직병리학적 분류가 필요하며, 보다 많은 소아 IgA 신병증 환아의 장기간 추적 관찰을 통한 연구가 이루어져야 할 것이다.

Purpose : Clinicopathological features were investigated to clarify the outcome and prognostic indicators for patients with IgA nephropathy in Korean children. Methods : We reviewed the outcomes of 61 patients in whom IgA nephropathy was diagnosed before the age of 15 years from 1991 to 2005 and followed-up at least for one year. All patients were confirmed by renal biopsy. Results : After mean follow-up of 5.2 years from onset, 24 patients of 61 (39.3%) were in clinical remission at the last examination. Thirty patients (49.2%) had hematuria or mild proteinuria (<$1g/m^2/d$), five (8.2%) had severe proteinuria (${\geq}1g/m^2/d$), and two (3.3%) had chronic renal failure. By univariate analysis, initial presentation at onset and Haas classification were less concordant with outcome. Hypertension during follow-up, rather than hypertension at presentation, was significantly correlated with outcomes (P<0.01). Sixty percent of patients who had more than 20% of glomerular sclerosis or crescent progressed to severe proteinuria or chronic renal failure, as compared with 7.1% of those who did not (P<0.01). Conclusion : Prognosis of childhood IgA nephropathy had a relatively benign course during a mean follow-up of 5.2 years. Persistent hypertension during follow-up and more than 20% of glomerular sclerosis or crescent were strong predictors of a progressive course of IgA nephropathy. A new histologic classification according to characteristics of childhood IgA nephropathy must be established to assess prognosis. Further efforts should be made to understand the prognosis of IgA nephropathy through long-term follow-up.

키워드

참고문헌

  1. Berger J, Hinglais N. Intercapillary deposits of IgA-G. J Urol Nephrol 1968;74;694-5
  2. DAmico G. The commonest glomerulonephritis in the world: IgA nephropathy. Q J Med 1987;64:709-27
  3. DAmico G. Natural history of IgA nephropathy and factors predictive of disease outcome. Semin Nephrol, 2004;24:179-96 https://doi.org/10.1016/j.semnephrol.2004.01.001
  4. Coppo R, DAmico G. Factors predicting progression of IgA nephropathies. J Nephrol 2005;18:503-127
  5. Ronkainen J, Ala-Houhala M, Autio-Harmainen H, Jahnukainen T, Koskimies O, Merenmies J, et al. Long-term outcome 19 years after childhood IgA nephritis: a retrospective cohort study. Pediatr Nephrol 2006;21:1266-73 https://doi.org/10.1007/s00467-006-0163-x
  6. Kusumoto Y. Takebayashi S. Taguchi T. Harada T. Naito S. Long-term prognosis and prognostic indices of IgA nephropathy in juvenile and in adult Japanese. Clin Nephrol 1987;28:118-24
  7. Wyatt RJ, Kritchevsky SB, Woodford SY, Miller PM, Roy S 3rd, Holland NH, et al. IgA nephropathy: long-term prognosis for pediatric patients. J Pediatr 1995;127:913-9 https://doi.org/10.1016/S0022-3476(95)70027-7
  8. Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A. A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 1976;58:259-63
  9. Schwartz GJ, Brion LP, Spitzer A. The use of plasma creatinine concentration to estimate glomerular filtration rate in infancy, childhood, adolescence. Pediatr Clin North Am 1987:34;571-90 https://doi.org/10.1016/S0031-3955(16)36251-4
  10. Lee JK. Standard blood pressure of child and adolescent in 2005. Program and Abstract, 56th Annual Spring Meeting of The Korean Pediatric Society; 2006 Apr 28-29; Yong- Pyung, Seoul : The Korean Pediatric Society 2006:63-90
  11. Haas M. Histologic subclass of IgA nephropathy: a clinicopathologic study of 244 cases. Am J Kidney Dis 1997;29: 829-42 https://doi.org/10.1016/S0272-6386(97)90456-X
  12. Yosikawa N. Immunoglobulin A nephropathy. In : Qvener ED, Harmon WE, Niadet P. Pediatric nephrology. 5th ed. Philadelphia : LW & Wilkins Co 2004:615-28
  13. Utsunomiya Y, Kado T, Koda T, Okada S, Hayashi A. Fukuwaza A, et al. Features of IgA nephropathy in preschool children. Clin Nephrol 2000;54:443-8
  14. Yoshikawa N, Tanaka R, Iijima K. Pathophysiology and treatment of IgA nephropathy in children. Pediatr Nephrol 2001;16:446-57 https://doi.org/10.1007/s004670100582
  15. Park YH, Choi JY, Chung HS, Koo JW, Kim SY, Namgoong MK, et al. Hematuria and proteinuria in a mass school screening test. Pediatr Nephrol 2005;20:1126-30 https://doi.org/10.1007/s00467-005-1915-8
  16. Nozawa R, Suzuki J, Takahashi A, Isome M, Kawasaki Y, Suzuki S, et al. Clinicopathological features and the prognosis of IgA nephropathy in Japanese children on longterm observation. Clin Nephrol 2005:64;171-9 https://doi.org/10.5414/CNP64171
  17. Ariceta G, Gallego N, Lopez.Fernandez Y, Lopez Fernandez Y, Vallo A, Quintela MJ, el al. Long.term prognosis of childhood IgA nephropathy in adult life. Med Clin(Brac) 2001;116:361-4 https://doi.org/10.1016/S0025-7753(01)71831-3
  18. Yosikawa N, Iijima K, Ito H. IgA nephropathy in children. Nephron 1999;83:1-12 https://doi.org/10.1159/000045467
  19. Lee JS, Kwon JH, Choi EN, Park JM, Jeung HJ. A clinicopathologic study on the prognosis of IgA nephropathy in children. J Korean Soc Pediatr Nephrol 2003:7;23-9
  20. Andreoli SP, Yum MN, Bergstein JM. IgA nephropathy in children : significance of glomerular base membrane deposition of IgA. Am J Nephrol 1986;6:28-33 https://doi.org/10.1159/000167049
  21. Delos Santos NM, Ault BH, Gharavi AG, Kritchevsky SB, Quasney MW, Jackson EC, et al. Angiotensin-converting enzyme genotype and outcome in pediatric IgA nephropathy. Pediatr Nephrol 2002;17:496-502 https://doi.org/10.1007/s00467-002-0916-0
  22. Hogg RJ, Silva FG, Wyatt RJ, Reisch JS, Argyle JE, Savino DA. Prognostic indicators in children with IgA nephropathy: Report of the Southwest Pediatric Nephrology Study Group. Pediatr Nephrol 1994;8:15-20 https://doi.org/10.1007/BF00868251
  23. Delos Santos NM, Wyatt RJ. Pediatric IgA nephropathy: clinical aspects and therapeutic approaches. Semin Nephrol 2004:24;269-86 https://doi.org/10.1016/j.semnephrol.2004.01.007
  24. Yoshikawa N, Ito H, Yoshihara S, Nakagara C. Yoshiya K, Hasegawa O, et al. Clinical course of Immunoglobulin A nephropathy in children. J Pediatr 1987;110:555-60 https://doi.org/10.1016/S0022-3476(87)80547-4
  25. Holt RCL, Connell JE, Addison GM. Reference data for pediatric nephrology. In : Webb NJA, Postlethwaite RJ. Clinical pediatric nephrology. 3rd edition. Oxford : Oxford University Press 2003:493-509
  26. Yoshikawa N, Ito H, Nakamura H. Prognostic indicators in childhood IgA nephropathy. Nephron 1992;60:60-7 https://doi.org/10.1159/000186706
  27. Ikezumi Y, Suzuki T, Imai N, Ueno M, Narita I, Kawachi H, et al. Histologic difference in newly onset IgA nephropathy between children and adults. Nephrol Dial Transplant 2006:1-9
  28. Shigematsu H, Ito N, Ishigame H, Ehara T, Kato M, Washizawa K, et al. Age-related character of glomerular lesions in IgA nephritis(2). Histopathological peculiarity in childhood onset. Nippon Jinxo Gakkai Shi 1992;34:33-9
  29. DAmico G. Imbasciati E, Barbiano Di Belgioioso G. Bertoli S. Bertoli S, Fogazzi G, et al. Idiopathic IgA mesangial nephropathy. Clinical and histological study of 374 patients. Medicine(Baltimore) 1985;64:49-60 https://doi.org/10.1097/00005792-198501000-00004
  30. The Southwest Pediatric Nephrology Study Group. A multicenter study of IgA nephropathy in children. A report of the Southwest Pediatric Nephrology Study Group. Kidney Int 1982;22:643-52 https://doi.org/10.1038/ki.1982.224
  31. Levi M, Gonzalez-Buschard G, Broyer M, Dommergues J, Foulard M, Sorez J, et al. Bergers disease in children. Natural history and outcome. Medicine(Baltimore) 1985;64: 157-80
  32. Nolin L, Courteau M. Management of IgA nephropathy: evidence-based recommendation. Kidney Int 1999;70:s62-9
  33. Floege J. Evidence-based recommendation for immunosuppression in IgA nephropathy: handle with caution. Nephrol Dial Transplant 2003;18:241-5 https://doi.org/10.1093/ndt/18.2.241
  34. Maschio G, Gagnoli L, Claroni F, Fusaroli M, Ruqiu C, Sanna G, et al. ACE inhibition reduces proteinuira in normotensive patients with IgA nephropathy: A multicenter, randomizd, placebo-controlled study. Nephol Dial Transplant 1994;9:265-9
  35. Hebert LE, Wilmer WA, Falkenhain ME, Ladson-Wofford SE. Nahman NS Jr, Robin BH. Renoprotection: one or many therapies? Kidney Int 2001;59:1211-23 https://doi.org/10.1046/j.1523-1755.2001.0590041211.x
  36. Bhattacharjee R, Filler G. Additive antiproteinuric effect of ACE inhibitor and losartan in IgA nephropathy. Pediatr Nephrol 2002;17:302-4 https://doi.org/10.1007/s00467-002-0829-y
  37. Hogg RJ, Wyatt RJ. Scientific Planning Committee of the North American IgA Nephropathy Study. A randomized controlled trial of mycophenolate mofetil in patients with IgA nephropathy. BMC Nephrol 2004;5:3 https://doi.org/10.1186/1471-2369-5-3
  38. Coppo R. Chiesa M, Peruzzi L, Amore A. Treatment of IgA nephropathy with anigiotensin converting enzyme inhibiter: design of a prospective randomized multicenter trial. J Nephrol 2001:14;447-52