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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