Atypical Hemolytic Uremic Syndrome
Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulator...
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W.B. Saunders
2013
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| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3863953/ |
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pubmed-38639532013-12-17 Atypical Hemolytic Uremic Syndrome Kavanagh, David Goodship, Tim H. Richards, Anna Article Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described. Incomplete penetrance of mutations in all predisposing genes is reported, suggesting that a precipitating event or trigger is required to unmask the complement regulatory deficiency. The underlying genetic defect predicts the prognosis both in native kidneys and after renal transplantation. The successful trials of the complement inhibitor eculizumab in the treatment of atypical HUS will revolutionize disease management. W.B. Saunders 2013-11 /pmc/articles/PMC3863953/ /pubmed/24161037 http://dx.doi.org/10.1016/j.semnephrol.2013.08.003 Text en © 2013 Elsevier Inc. This document may be redistributed and reused, subject to certain conditions (http://www.elsevier.com/wps/find/authorsview.authors/supplementalterms1.0) . |
| repository_type |
Open Access Journal |
| institution_category |
Foreign Institution |
| institution |
US National Center for Biotechnology Information |
| building |
NCBI PubMed |
| collection |
Online Access |
| language |
English |
| format |
Online |
| author |
Kavanagh, David Goodship, Tim H. Richards, Anna |
| spellingShingle |
Kavanagh, David Goodship, Tim H. Richards, Anna Atypical Hemolytic Uremic Syndrome |
| author_facet |
Kavanagh, David Goodship, Tim H. Richards, Anna |
| author_sort |
Kavanagh, David |
| title |
Atypical Hemolytic Uremic Syndrome |
| title_short |
Atypical Hemolytic Uremic Syndrome |
| title_full |
Atypical Hemolytic Uremic Syndrome |
| title_fullStr |
Atypical Hemolytic Uremic Syndrome |
| title_full_unstemmed |
Atypical Hemolytic Uremic Syndrome |
| title_sort |
atypical hemolytic uremic syndrome |
| description |
Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described. Incomplete penetrance of mutations in all predisposing genes is reported, suggesting that a precipitating event or trigger is required to unmask the complement regulatory deficiency. The underlying genetic defect predicts the prognosis both in native kidneys and after renal transplantation. The successful trials of the complement inhibitor eculizumab in the treatment of atypical HUS will revolutionize disease management. |
| publisher |
W.B. Saunders |
| publishDate |
2013 |
| url |
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3863953/ |
| _version_ |
1612038615959863296 |