Document Detail

Hemolytic uremic syndrome.
MedLine Citation:
PMID:  24526222     Owner:  NLM     Status:  Publisher    
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, nonimmune microangiopathic hemolytic anemia, and acute renal failure. The most frequent form is associated with infections by Shiga-like toxin-producing bacteria (STEC-HUS). Rarer cases are triggered by neuraminidase-producing Streptococcus pneumoniae (pneumococcal-HUS). The designation of aHUS is used to refer to those cases in which an infection by Shiga-like toxin-producing bacteria or S. pneumoniae can be excluded. Studies performed in the last two decades have documented that hyperactivation of the complement system is the pathogenetic effector mechanism leading to the endothelial damage and the microvascular thrombosis in aHUS. Recent data suggested the involvement of the complement system in the pathogenesis of STEC-HUS and pneumococcal-HUS as well. Clinical signs and symptoms may overlap among the different forms of HUS; however, pneumococcal-HUS and aHUS have a worse prognosis compared with STEC-HUS. Early diagnosis and identification of underlying pathogenetic mechanism allows instating specific support measures and therapies. In clinical trials in patients with aHUS, complement inhibition by eculizumab administration leads to a rapid and sustained normalization of hematological parameters with improvement in long-term renal function. This review summarizes current concepts about the epidemiological findings, the pathological and clinical aspects of STEC-HUS, pneumococcal-HUS, and aHUS, and their diagnosis and management.
Caterina Mele; Giuseppe Remuzzi; Marina Noris
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-2-14
Journal Detail:
Title:  Seminars in immunopathology     Volume:  -     ISSN:  1863-2300     ISO Abbreviation:  Semin Immunopathol     Publication Date:  2014 Feb 
Date Detail:
Created Date:  2014-2-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101308769     Medline TA:  Semin Immunopathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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