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Rapidly progressive, pauci-immune diffuse crescentic glomerulonephritis in an infant

  • Glomerular Disease
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Pediatric Nephrology Aims and scope Submit manuscript

Abstract.

A Japanese male infant presented with nephrotic syndrome at 41 days. His renal function progressively deteriorated, and he died at 4 months of the age. An open renal biopsy revealed diffuse crescentic glomerulonephritis (CrGN) without immune complex deposition, which is not characteristic of the congenital nephrotic syndrome (CNS). Examination for nephrin antigen using rabbit anti-nephrin extra- and intracellular site antibodies was positive. These clinical observations suggest that the patient had a unique histological variant of CNS. This is the first report of rapidly progressive, pauci-immune diffuse CrGN in infancy.

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Authors and Affiliations

  1. Department of Pediatrics, Hirosaki University, School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562 Japan, , , , ,

    Hiroshi Tanaka, Shinobu Waga, Koichi Suzuki, Tohru Nakahata & Etsuro Ito

  2. Department of Cell Biology, Institute of Nephrology, Niigata University School of Medicine, Niigata, Japan, , , , ,

    Hiroshi Kawachi & Fujio Shimizu

Authors
  1. Hiroshi Tanaka
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  2. Shinobu Waga
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  3. Koichi Suzuki
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  4. Tohru Nakahata
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  5. Hiroshi Kawachi
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  6. Fujio Shimizu
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  7. Etsuro Ito
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Tanaka, H., Waga, S., Suzuki, K. et al. Rapidly progressive, pauci-immune diffuse crescentic glomerulonephritis in an infant. Pediatr Nephrol 17, 730–732 (2002). https://doi.org/10.1007/s00467-002-0938-7

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  • DOI: https://doi.org/10.1007/s00467-002-0938-7

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