Once-daily low-dose cyclosporine A treatment for steroid-resistant nephrotic syndrome in a patient with Galloway-Mowat syndrome: A case report

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  • Nakamizo Tomoya
    Department of Pediatric, Tsuchiura Kyodo General Hospital
  • Tada Norimasa
    Department of Pediatric, Tsuchiura Kyodo General Hospital
  • Udagawa Tomohiro
    Department of Pediatric, Tsuchiura Kyodo General Hospital Department of Pediatric, Tokyo Medical and Dental University Hospital
  • Kikuchi Eriko
    Department of Pediatric, Nerima Hikarigaoka Hospital
  • Kamei Koichi
    Division of Nephrology and Rheumatology, National Center for Child Health and Development
  • Mori Takayasu
    Department of Nephrology, Tokyo Medical and Dental University Hospital
  • Sohara Eisei
    Department of Nephrology, Tokyo Medical and Dental University Hospital
  • Matsuoka Kentaro
    Department of Pathology, Tokyo Metropolitan Children’s Medical Center
  • Shirai Kentaro
    Department of Pediatric, Tsuchiura Kyodo General Hospital
  • Watanabe Akimitsu
    Department of Pediatric, Tsuchiura Kyodo General Hospital

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  • シクロスポリン投与によりステロイド抵抗性ネフローゼ症候群の長期寛解を維持しているGalloway-Mowat症候群の1例

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Abstract

<p>Galloway-Mowat syndrome (GAMOS) is a disorder characterized by mental retardation with microcephaly and steroid-resistant nephrotic syndrome (SRNS). According to previous reports, SRNS defines the prognosis in patients with GAMOS because of its resistance to treatment. We herein describe a patient with GAMOS who achieved long-term remission on cyclosporine. A 1-year-old girl was noted to have mental retardation and microcephaly at a health checkup. She developed proteinuria at 2 years of age; she thereafter met the criteria for SRNS at 5 years of age and was diagnosed with GAMOS based on clinical symptoms. After the introduction of cyclosporine A for SRNS, the proteinuria gradually decreased, and nephrotic remission was achieved at 7 years of age. When the cyclosporine was discontinued after remission, the proteinuria worsened again, suggesting that the cyclosporine had contributed to the decrease in proteinuria. To reduce nephrotoxicity, the cyclosporine was changed to once-daily administration at 8 years of age. Renal biopsy at 14 years of age showed no obvious nephrotoxicity, and remission was maintained. Once-daily administration of cyclosporine may be effective in reducing nephrotoxicity and improving the prognosis in patients with hereditary SRNS such as GAMOS nephropathy.</p>

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