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Journal of the Korean Society of Pediatric Nephrology 2007;11(2): 178-184. doi: https://doi.org/10.3339/jkspn.2007.11.2.178
Methylprednisolone 충격 요법만 받은 국소성 분절성 사구체 경화증 환아의 예후
김정아, 박광식, 신재일, 정일천, 김지홍, 김병길, 정현주, 이재승
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
6관동대학교 의과대학 소아과학교실
7연세대학교 의과대학 병리학교실
8연세대학교 의과대학 소아과학교실, 신장질환 연구소
The Prognosis of Focal Segmental Glomerulosclerosis Patients with Methylprednisolone Pulse Therapy Alone
Joung-A Kim, Kwang-Sik Park, Jae-Il Shin, Il-Cheon Jeong, Ji-Hong Kim, Pyung-Kil Kim, Hyun-Joo Jeong, Jae-Seung Lee
1The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Severance Childrens Hospital
2The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Severance Childrens Hospital
3The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Severance Childrens Hospital
4The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Severance Childrens Hospital
5The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Severance Childrens Hospital
6Department of Pediatrics, Kwandong University College of Medicine
7Department of Pathology, Yonsei University College of Medicine
8The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Severance Childrens Hospital
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ABSTRACT
Purpose : Since the first report by Mendoza in 1990, there have been several studies reporting that long-term intravenous methylprednisolone(MP) pulse therapy combined with cyclosporin A(CsA) or cyclophosphamide might be beneficial for the treatment of steroid resistant focal segmental glometulosclerosis(FSGS). We investigated the therapeutic effect of long-term MP pulse therapy without CsA or cyclophosphamide on steroid resistant FSGS.
Methods : The medical records of the 10 steroid resistant FSGS patients who were treated with MP pulse therapy by the Mendoza protocol without CsA or cyclophosphamide in our hospital were retrospectively reviewed.
Results : The median age at onset was 2.6 years(range 1.1-10.6 years) and the median age at the initiation of therapy was 5.7 years(range 1.8-20 years). The median duration of follow-up was 35 months(range 4-132 months). At the end of therapy, 5 patients achieved complete remission(50%) and 2 partial remission(20%), one of whom relapsed after the therapy. Three patients did not respond to the therapy, two of whom progressed to end-stage renal failure during the therapy eventually requiring kidney transplantation.
Conclusion : Intravenous long-term MP pulse therapy without CsA or cyclophosphamide by the Mendoza protocol may be effective in a subset of patients with steroid-resistant FSGS.
Key words: Mendoza protocol | Methylprednisolone pulse therapy | Focal segmental glomerulosclerosis
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