caa a22 4500 445883448 CHVBK 20180317145552.0 cr unu---uuuuu 170323e20111001xx s 000 0 eng 10.1007/s10157-011-0478-1 doi (NATIONALLICENCE)springer-10.1007/s10157-011-0478-1 Kawasaki Yukihiko Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikariga-oka, 960-1295, Fukushima, Fukushima, Japan aut The pathogenesis and treatment of pediatric Henoch-Schönlein purpura nephritis [Elektronische Daten] [Yukihiko Kawasaki] 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 of Henoch-Schönlein purpura nephritis (HSPN) and to identify the most appropriate treatment. We herein review the pathogenesis and treatment of HSPN. As to the pathogenesis, several studies suggest that galactose-deficient IgA1 is recognized by anti-glycan antibodies, leading to the formation of circulating immune complexes and their mesangial deposition, thereby inducing renal injury. Aggressive therapies for the treatment of severe HSPN, including multiple drug combination therapy and plasmapheresis, have been shown to be effective in ameliorating proteinuria and histological severity. Nevertheless, detailed investigations of the pathogenesis of HSPN and double-blind randomized control studies on children with HSPN are still necessary. Japanese Society of Nephrology, 2011 HSP nationallicence HSPN nationallicence Pathogenesis nationallicence Treatment nationallicence Child nationallicence Mizoribine nationallicence Cyclophosphamide nationallicence Clinical and Experimental Nephrology Springer Japan 15/5(2011-10-01), 648-657 1342-1751 15:5<648 2011 15 10157 https://doi.org/10.1007/s10157-011-0478-1 text/html Onlinezugriff via DOI 1 review-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10157-011-0478-1 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Kawasaki Yukihiko Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikariga-oka, 960-1295, Fukushima, Fukushima, Japan aut NATIONALLICENCE 773 0- Clinical and Experimental Nephrology Springer Japan 15/5(2011-10-01), 648-657 1342-1751 15:5<648 2011 15 10157 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer