HENOCH-SCH^|^Ouml;NLEIN PURPURA NEPHRITIS IN CHILDHOOD: PATHOGENESIS, PROGNOSTIC FACTORS AND TREATMENT

• KAWASAKI YUKIHIKO

  Department of Pediatrics, Fukushima Medical University School of Medicine

• ONO ATSUSHI

  Department of Pediatrics, Fukushima Medical University School of Medicine

• OHARA SHINICHIRO

  Department of Pediatrics, Fukushima Medical University School of Medicine

• SUZUKI YUICHI

  Department of Pediatrics, Fukushima Medical University School of Medicine

• SUYAMA KAZUHIDE

  Department of Pediatrics, Fukushima Medical University School of Medicine

• SUZUKI JUNZO

  Department of Pediatrics, Fukushima Medical University School of Medicine

• HOSOYA MITSUAKI

  Department of Pediatrics, Fukushima Medical University School of Medicine

書誌事項

タイトル別名

• HENOCH-SCHÖNLEIN PURPURA NEPHRITIS IN CHILDHOOD: PATHOGENESIS, PROGNOSTIC FACTORS AND TREATMENT

抄録

Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. Thus, it is important to clarify the onset mechanism as well as the prognostic factors of Henoch-Schönlein purpura nephritis (HSPN) and to identify the most appropriate treatment. We herein review the pathogenesis, the prognostic factors and treatment of HSPN. As to the pathogenesis, several studies suggest that galactose-deficient IgA1 (Gd-IgA1) is recognized by anti-glycan antibodies, leading to the formation of circulating immune complexes and their mesangial deposition, thereby inducing renal injury. With regard to the prognostic factors, a number of factors have been suggested including nephrotic syndrome, decreased factor XIII activity, hypertension, severe renal injury, high renal accumulation of activated macrophage, alpha-smooth muscle actin, and high serum myeloid-related protein levels.<br>For the treatment of severe HSPN, aggressive therapies including multiple drug combination therapy and plasmapheresis have been shown to be effective in ameliorating proteinuria and histological severity. Nevertheless, detailed investigation into the pathogenesis of HSPN and double-blind randomized control studies on children with HSPN are still necessary.

収録刊行物

• 福島医学会

  福島医学会 59 (1), 15-26, 2013

  福島医学会

キーワード

• HSPN
• Pathogenesis
• urokinase pulse therapy
• cyclophosphamide
• mizoribine

詳細情報

• CRID: 1390001206304201600
• NII論文ID: 130003376082
• NII書誌ID: AA0065246X
• DOI: 10.5387/fms.59.15
• COI: 1:STN:280:DC%2BC3sjpvVejug%3D%3D
• ISSN: 21854610
• ISSN: 00162590
• PubMed: 23842510
• Web Site: http://ir.fmu.ac.jp/dspace/handle/123456789/360
• Web Site: https://search.jamas.or.jp/link/ui/2014059596
• 本文言語コード: en
• データソース種別:
  • JaLC
  • IRDB
  • Crossref
  • PubMed
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可