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CEN Case Rep. 2013 May;2(1):117-122. doi: 10.1007/s13730-012-0056-8. Epub 2013 Jan 12.

Methylprednisolone pulse therapy and intravenous cyclophosphamide therapy combined with cocktail therapy in severe pediatric Henoch-Schönlein purpura nephritis patient.

CEN case reports

Hiroaki Kanai, Anna Kobayashi, Kyoko Matsushita, Emi Sawanobori, Kanji Sugita, Kosuke Higashida

Affiliations

  1. Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Shimokato 1110, Chuo-city, Yamanashi, 409-3898, Japan. [email protected].
  2. Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Shimokato 1110, Chuo-city, Yamanashi, 409-3898, Japan.

PMID: 28509230 PMCID: PMC5411523 DOI: 10.1007/s13730-012-0056-8

Abstract

Henoch-Schönlein purpura (HSP) is a common self-limited vasculitis in children. The long-term prognosis depends on renal involvement. In severe Henoch-Schönlein purpura nephritis (HSPN) patients, >50 % have crescent formation and nephrotic syndrome that are important predicted outcomes. Therefore, for such patients, an aggressive immunosuppressive therapy is needed to prevent the progression. However, there is no consensus for an appropriate therapeutic regimen for severe pediatric HSPN patients. In this paper, we have reported on a 6-year-old boy who presented with HSPN with nephrotic syndrome and severe histopathological abnormalities; he was diagnosed with International Study of Kidney Disease in Children (ISKDC) grade IVb. Despite treatment with methylprednisolone pulse therapy, followed by oral prednisolone and dipyridamole; the nephrotic syndrome persisted. Subsequently, intravenous cyclophosphamide therapy (IVCY) (500-1,000 mg m

Keywords: Henoch-Schönlein purpura nephritis; Intravenous cyclophosphamide therapy; Methylprednisolone pulse therapy; Nephrotic syndrome

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