Document Detail


Pathogenesis of thrombotic microangiopathies.
MedLine Citation:
PMID:  18215115     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Profound thrombocytopenia and microangiopathic hemolytic anemia characterize thrombotic microangiopathy, which includes two major disorders: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). TTP has at least three types: congenital or familial, idiopathic, and nonidiopathic. The congenital and idiopathic TTP syndromes are caused primarily by deficiency of ADAMTS13, owing to mutations in the ADAMTS13 gene or autoantibodies that inhibit ADAMTS13 activity. HUS is similar to TTP, but is associated with acute renal failure. Diarrhea-associated HUS accounts for more than 90% of cases and is usually caused by infection with Shiga-toxin-producing Escherichia coli (O157:H7). Diarrhea-negative HUS is associated with complement dysregulation in up to 50% of cases, caused by mutations in complement factor H, membrane cofactor protein, factor I or factor B, or by autoantibodies against factor H. The incomplete penetrance of mutations in either ADAMTS13 or complement regulatory genes suggests that precipitating events or triggers may be required to cause thrombotic microangiopathy in many patients.
Authors:
X Long Zheng; J Evan Sadler
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Annual review of pathology     Volume:  3     ISSN:  1553-4006     ISO Abbreviation:  Annu Rev Pathol     Publication Date:  2008  
Date Detail:
Created Date:  2008-03-20     Completed Date:  2008-05-13     Revised Date:  2014-09-18    
Medline Journal Info:
Nlm Unique ID:  101275111     Medline TA:  Annu Rev Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  249-77     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
ADAM Proteins / deficiency,  genetics,  metabolism
Acute Kidney Injury / etiology,  metabolism,  pathology
Diarrhea / metabolism,  microbiology,  pathology
Hemolytic-Uremic Syndrome / etiology*,  metabolism,  pathology
Humans
Microcirculation / pathology
Point Mutation
Purpura, Thrombotic Thrombocytopenic / etiology*,  metabolism,  pathology
von Willebrand Factor / metabolism
Grant Support
ID/Acronym/Agency:
HL079027/HL/NHLBI NIH HHS; HL72917/HL/NHLBI NIH HHS; HL89746/HL/NHLBI NIH HHS; P50HL081012/HL/NHLBI NIH HHS; R01 HL079027/HL/NHLBI NIH HHS; R01 HL079027-03/HL/NHLBI NIH HHS; R01 HL079027-04/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/von Willebrand Factor; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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