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Journal of the Korean Society of Pediatric Nephrology 2000;4(1): 17-24.
미세변화형 신증후군(MCNS)으로부터 국소성 분절성 사구체 경화증(FSGS)으로 이행된 환아의 임상양상
이지은, 육진원, 이의성, 김지홍, 김병길, 정현주
1연세대학교 의과대학 소아과학교실, 신장질환연구소
2연세대학교 의과대학 소아과학교실, 신장질환연구소
3연세대학교 의과대학 소아과학교실, 신장질환연구소
4연세대학교 의과대학 소아과학교실, 신장질환연구소
5연세대학교 의과대학 소아과학교실, 신장질환연구소
6연세대학교 의과대학 소아과학교실, 병리학교실
Clinical Analysis of Children with Transitory Minimal Change Nephrotic Syndrome (MCNS) to Focal Segmental Glomerulosclerosis (FSCS)
Ji Eun Lee, Jinwon Yook, Eui Seong Lee, Ji Hong Kim, Pyung-Kil Kim, Hyun Joo Chung
1Department of Pediatrics, The Institute of Kidney Disease, Yonei University, College of Medicine
2Department of Pediatrics, The Institute of Kidney Disease, Yonei University, College of Medicine
3Department of Pediatrics, The Institute of Kidney Disease, Yonei University, College of Medicine
4Department of Pediatrics, The Institute of Kidney Disease, Yonei University, College of Medicine
5Department of Pediatrics, The Institute of Kidney Disease, Yonei University, College of Medicine
6Department of Pathology, Yonsei University, College of Medicine
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ABSTRACT
Purpose: MCNS is found in approximately $85%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS.
Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy.
Results : The number of children was 296 cases comprising 241 cases($81.4%$) showing MCNS, 8 cases($2.7%$) transition group, 47 cases($15.9%$) FSGS. The mean onset age was $6.0{pm}2.6$years in MCNS, transition group $8.3{pm}2.3$years, FSGS $7.2{pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$%$ in MCNS. $12.5%$ in transition group, $29.6%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5%$ of MCNS, $100%$ in transition group, $80.8%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups.
Conclusion : 249 patients with MCNS have been followed and $3.2%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.
Key words: Minimal Change Nephrotic Syndrome | Focal Segmental Glomerulosclerosis
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