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溶血尿毒综合征三例报告
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作者 曹敏 季清锐 《实用儿科临床杂志》 CAS CSCD 1989年第2期111-112,共2页
溶血尿毒综合征是婴儿和儿童急性肾功能衰竭的主要病因之一,其临床特点为微血管病性溶血性贫血。
关键词 肾衰竭 溶血尿毒征 病因学 儿童
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Update on hemolytic uremic syndrome:Diagnostic and therapeutic recommendations 被引量:13
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作者 Maurizio Salvadori Elisabetta Bertoni 《World Journal of Nephrology》 2013年第3期56-76,共21页
Hemolytic uremic syndrome (HUS) is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in ... Hemolytic uremic syndrome (HUS) is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in particular, mutations of genes that encode the complement-regulatory proteins have improved our understanding of atypical HUS. Similarly, the comple-ment proteins are clearly involved in all types of thrombotic microangiopathy: typical HUS, atypical HUS and thrombotic thrombocytopenic purpura (TTP). Fur-thermore, several secondary HUS appear to be related to abnormalities in complement genes in predisposed patients. The authors highlight the therapeutic as-pects of this rare disease, examining both “traditional therapy” (including plasma therapy, kidney and kidney-liver transplantation) and “new therapies”. The latter include anti-Shiga-toxin antibodies and anti-C5 mono-clonal antibody “eculizumab”. Eculizumab has been recently launched for the treatment of the atypical HUS, but it appears to be effective in the treatment of typical HUS and in TTP. Future therapies are in phases Ⅰ and Ⅱ. They include anti-C5 antibodies, which are more purifed, less immunogenic and absorbed orally and, anti-C3 antibodies, which are more powerful, but potentially less safe. Additionally, infusions of recombinant complement-regulatory proteins are a potential future therapy. 展开更多
关键词 Enterohemorrhagic Escherichia coli Diarrhea Shiga toxin (Stx) 1 and Stx 2 Atypical hemolytic uremic syndrome Complement factors Thrombotic thrombocytopenic purpura Secondary thrombotic microangiopathy Plasma therapy ECULIZUMAB
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