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Journal of the Korean Society of Pediatric Nephrology 2001;5(2): 117-124.
일차성 및 이차성 신증후군에서 Mendoza Protocol에 의한 Intravenous Methylprednisolone Pulse Therapy의 효과
이경재, 한재혁, 이영목, 김지홍, 김병길
1연세대학교 의과대학 소아과학교실, 신장질환 연구소
2연세대학교 의과대학 소아과학교실, 신장질환 연구소
3연세대학교 의과대학 소아과학교실, 신장질환 연구소
4연세대학교 의과대학 소아과학교실, 신장질환 연구소
5연세대학교 의과대학 소아과학교실, 신장질환 연구소
The Effects of Intravenous Methylprednisolone Pulse Therapy by Mendoza Protocol in Primary and Secondary Nephrotic Syndrome
Kyoung-Jae Lee, Jae-Hyuk Han, Young-Mock Lee, Ji-Hong Kim, Pyung-Kil Kim
1Department of Pediatrics, The institute of Kidney Disease Yonsei University College of Medicine
2Department of Pediatrics, The institute of Kidney Disease Yonsei University College of Medicine
3Department of Pediatrics, The institute of Kidney Disease Yonsei University College of Medicine
4Department of Pediatrics, The institute of Kidney Disease Yonsei University College of Medicine
5Department of Pediatrics, The institute of Kidney Disease Yonsei University College of Medicine
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ABSTRACT
Purpose : Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulosclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid- dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective.
Methods : We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy Period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methylprednisolone, often in combination with oral cyclophosphamide for 82 weeks.
Results : In all the 10 children with SD-MCNS, complete remission was visible on average of $18{pm}9$ days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was $2.1{pm}1.0$ relpases/year, which was reduced to $1.4{pm}0.9$ relapses/year during MP therapy(P>0.05) and rose to $2.7{pm}1.0$ relapse/year after MP therapy. Of the 6 children with FSGS, 4 children($67%$) showed complete remission, of whom 3 children($50%$) remained in the remission status during the follow up period, $1.2{pm}0.7$ years, after the end of MP therapy. 2 children($33%$) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, $1.7{pm}0.6$ years The only side effect of MP therapy was transient hypertension in 10 children of ail subjects during the intravenous infusion of methylprednisolone.
Conclusion : We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 117-24)
Key words: Mendoza protocol | Methylprednisolone pulse therapy | Nephrotic syndrome
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