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Vol.59 (2013)  >

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Title: Henoch-schönlein purpura nephritis in childhood: pathogenesis, prognostic factors and treatment
Other Titles: HSPN in Childhood
Authors: Kawasaki, Yukihiko
Ono, Atsushi
Ohara, Shinichiro
Suzuki, Yuichi
Suyama, Kazuhide
Suzuki, Junzo
Hosoya, Mitsuaki
Affiliation: 小児科学講座
生命科学部門
Source title: Fukushima Journal of Medical Science
Volume: 59
Issue: 1
Start page: 15
End page: 26
Issue Date: 2013
Abstract: 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.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.
Publisher: The Fukushima Society of Medical Science
Publisher (Alternative foam): 福島医学会
language: eng
URI: http://ir.fmu.ac.jp/dspace/handle/123456789/360
Full text URL: http://ir.fmu.ac.jp/dspace/bitstream/123456789/360/1/FksmJMedSci_59_p15.pdf
ISSN: 0016-2590
2185-4610
DOI: 10.5387/fms.59.15
PubMed ID: 23842510
Related Page: http://dx.doi.org/10.5387/fms.59.15
Rights: © 2013 The Fukushima Society of Medical Science
Appears in Collections:Vol.59 (2013)

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