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신생검을 받은 소아 신증후군에서 조직병리 소견과 치료 성과

Histopathologic Diagnosis and Outcome of Renal Biopsied Pediatric Nephrotic Syndrome

김성도|조병수|정혜전|윤석
대한소아신장학회 (The Korean Society Of Pediatric Nephrology)|2005.10|Childhood Kidney Diseases (Childhood Kidney diseases)|제9권 제2호|pp. 149-158

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참고문헌

주제분류소아과학

초록

목 적 : 소아 신증후군 환아 중 신생검을 시행한 경우 그 임상적, 검사적 특징과 조직학적 소견, 그리고 치료 결과를 보기 위해 연구를 시행하였다.방 법 : 1984년부터 2004년까지 21년 동안 경희의료원 소아과에서 신생검을 시행 받은 169명(남아 130명, 여아 39명)에 대해 그 임상적 특징, 검사 소견, 병리 소견들과 치료 결과를 후향적으로 조사하였다. 신생검의 적응증으로는 입원 당시 비특이적 소견을 보이거나 빈발재발형, 스테로이드 의존성, 스테로이드 저항성 등의 소견으로 세포 독성 약제 치료가 필요했던 경우였다.결 과 : 약 반수의 환아(52.1%)에서만 미세변화형을 보였고, 그 다음으로는 미만성 메산지움증식형 33.1%, 초점 분절사구체신염 5.3%, 막증식사구체신염 2.4%, 막성신병증 2.4%, Ig A 신염 1.8% 등이었다. 미세변화형의 경우보다 미세변화형이 아닌 진단명인 경우 혈뇨와 스테로이드에 저항성을 보일 확률이 높았다. 총 37명의 스테로이드 저항성 환아 중 장기간에 걸쳐 스테로이드 치료를 시행한 결과 30명(81.0%)에서 마침내 완해를 보였다.

Abstract

Purpose : To determine the histological findings and treatment outcome in cases of childhood nephrotic syndrome which required renal biopsy. Methods : We retrospectively reviewed the clinical, laboratory, pathologic findings and therapeutic outcomes of 169 nephrotic children who received a renal biopsy at the Department of Pediatrics, Kyunghee Medical University Hospital, Seoul from 1984 to 2004 over a period of 21 years. The renal biopsy was performed in nephrotic children who showed atypical features at presentation, or needed cytotoxic therapy because of frequent-relapsing, steroid-dependent, or steroid-resistant nephrotic syndrome(SRNS). Results : Minimal change disease(MCD) was found in 52.1% of the patients, followed by diffuse mesangial proliferation(33.1%), focal segmental gomerulosclerosis(5.3%), membranoproliferative glomerulonephritis(2.4%), membranous nephropathy(2.4%), and IgA nephropathy(1.8 %). In MCD children, 14.8% had hematuria, 22.7% had hypertension, 5.7% showed decreased renal function, and no patient was found to have an abnormal complement level. Among patients diagnosed with diseases other than MCD, 43.2% had hematuria, 21.0% was found to be hypertensive, 7.4% of children showed decreased renal function and only 3(3.7%) had decreased complement level; the rates of hematuria and SRNS were found to be significantly higher than MCD patients. Among 37 SRNS patients, 30(81.0%) showed a final remission state with long-term steroid therapy, including methylprednisolone pulse therapy, over 4 months, with or without cytotoxic therapy. Conclusion : Almost half of the cases of childhood nephrotic syndrome requiring renal biopsy were not diagnosed with MCD. Among atypical features, hematuria and steroid-resistance would be the most probable indicators for a diagnosis other than MCD. Even in patients with SRNS, long-term methylprednisolone pulse therapy may result in a good remission rate.

참고문헌 (31)

  1. Eddy AA, "Nephrotic syndrome in childhood", 362, pp.629~39 (2003)
  2. Srivastava T, "High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood", 13, pp.13~8 (1999)
  3. No authors listed, "Nephrotic syndrome in children:prediction of histopathology from clinical and laboratory characteristics at time of diagnosis-a report of the International Study of Kidney Disease in Children", 13, pp.159~65 (1978)
  4. Simon P, "Epidemiology of primary glomerular diseases in a French region", 46, pp.1192~8 (1994)
  5. Haas M, "Increasing incidence of focal-segmental glomerulosclerosis among adult nephropathies:a 20-year renal biopsy study", 26, pp.740~50 (1995)
  6. Gulati S, "Changing trends of histopathology in childhood nephrotic syndrome", 34, pp.646~50 (1999)
  7. Bonilla-Felix M, "Changing patterns in the histopathology of idiopathic nephrotic syndrome in children", 55, pp.1885~90 (1999)
  8. Braden GL, "Changing incidence of glomerular diseases in adults", 35, pp.878~83 (2000)
  9. Choi IJ, "An analysis of 4,514 cases of renal biopsy in Korea", 42, pp.247~54 (2001)
  10. Kari JA, "Changing trends of histopathology in childhood nephrotic syndrome in western Saudi Arabia", 23, pp.317~21 (2002)
  11. Bhimma R, "Nephrotic syndrome in South African children:changing perspectives over 20 years", 11, pp.429~34 (1997)
  12. Task force on blood pressure control in children, "Report of the second task force on blood pressure control in children-1987", 79, pp.1~25 (1987)
  13. No authors listed, "controlled trial of cyclophosphamide therapy in children with nephrotic syndrome Report of the International study of Kidney Disease in Children", pp.423~7
  14. No authors listed, "The primary nephrotic syndrome in children Identification of patients with minimal change nephrotic syndrome from initial response to prednisone A report of the International Study of Kidney Disease in Children", pp.561~4
  15. Lee SJ, "Evaluation of the indications of renal biopsy in children with primary nephrotic syndrome", 39, pp.1736~44 (1996)
  16. Ko KW, "Childhood renal diseases in Korea", 1, pp.664~9 (1987)
  17. Gulati S, "Spectrum of adolescent-onset nephrotic syndrome in Indian children", 16, pp.1045~8 (2001)
  18. Stadermann MB, "Is biopsy required prior to cyclophosphamide in steroid-sensitive nephrotic syndrome", 60, pp.315~7 (2003)
  19. Gulati S, "Do current recommendations for kidney biopsy in nephrotic syndrome need modifications", 17, pp.404~8 (2002)
  20. Nolasco F, "Adult-onset minimal change nephrotic syndrome:a long-term follow-up", 29, pp.1215~23 (1986)
  21. Siegel NJ, "Long-term follow-up of children with steroid-responsive nephrotic syndrome", 81, pp.251~8 (1972)
  22. Niaudet P, "Steroid resistant idiopathic nephrotic syndrome. In : Barratt TM, Avner ED, Harmon WE. editors. Pediatric Nephrologyz", pp.749~64 (1999)
  23. Rose GM, "The treatment of severe glomerulopathies in children using high dose intravenous methylprednisolone pulses", 1, pp.148~56 (1981)
  24. Murnaghan K, "Pulse methylprednisolone therapy in severe idiopathic childhood nephrotic syndrome", 73, pp.733~9 (1984)
  25. Griswold WR, "Treatment of childhood prednisone-resistant nephrotic syndrome and focal segmental glomerulosclerosis with intravenous methylprednisolone and oral alkylating agents", 46, pp.73~7 (1987)
  26. Mendoza SA, "Treatment of steroid-resistant focal segmental glomerulosclerosis with pulse methylprednisolone and alkylating agents", 4, pp.303~7 (1990)
  27. Waldo FB, "Methylprednisolone treatment of patients with steroid-resistant nephrotic syndrome", 6, pp.503~5 (1992)
  28. Tune BM, "Intravenous methylprednisolone and oral alkylating agent therapy of prednisone-resistant pediatric focal segmental glomerulosclerosis:a long-term follow-up", 43, pp.84~8 (1995)
  29. Yorgin PD, "Pulse methylprednisolone treatment of idiopathic steroid-resistant nephrotic syndrome", 16, pp.245~50 (2001)
  30. Bajpai A, "Intravenous cyclophosphamide in steroid-resistant nephrotic syndrome", 18, pp.351~6 (2003)
  31. Mori K, "Efficacy of methylprednisolone pulse therapy in steroid- resistant nephrotic syndrome", 19, pp.1232~6 (2004)

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