RGD Reference Report - Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. - Rat Genome Database

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Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure.

Authors: Ono, T  Mimuro, J  Madoiwa, S  Soejima, K  Kashiwakura, Y  Ishiwata, A  Takano, K  Ohmori, T  Sakata, Y 
Citation: Ono T, etal., Blood. 2006 Jan 15;107(2):528-34. Epub 2005 Sep 27.
RGD ID: 10449048
Pubmed: PMID:16189276   (View Abstract at PubMed)
DOI: DOI:10.1182/blood-2005-03-1087   (Journal Full-text)

Deficiency of ADAMTS13 is found in patients with thrombotic thrombocytopenic purpura (TTP), and the genetic defects in the ADAMTS13 gene or the autoantibody against ADAMTS13 is thought to be responsible for the development of TTP. The clinical correlation and mechanisms of secondary ADAMTS13 deficiency in other disease states were investigated. In addition to TTP, ADAMTS13 levels were severely decreased in patients with sepsis-induced disseminated intravascular coagulation (DIC). The incidence of acute renal failure and serum creatinine levels in patients with ADAMTS13 activity levels lower than 20% (incidence, 41.2%; creatinine, 160 +/- 150 microM [1.81 +/- 1.70 mg/dL]) (P < .05) were significantly higher than they were in patients with ADAMTS13 activity levels higher than 20% (incidence, 15.4%; creatinine, 84 +/- 67 microM [0.95 +/- 0.76 mg/dL]) (P < .01). Additionally, unusually large von Willebrand factor multimers were detected in 26 (51.0%) of 51 patients with ADAMTS13 activity levels lower than 20%. Lower molecular weight forms of ADAMTS13 were found in the plasma of patients with sepsis-induced DIC, suggesting that the deficiency of ADAMTS13 was partially caused by its cleavage by proteases in addition to decreased synthesis in the liver. These data suggested that severe secondary ADAMTS13 deficiency can be associated with sepsis-induced DIC and may contribute to the development of renal failure.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
disseminated intravascular coagulation  IEP 10449048protein:decreased expression and decreased activity:plasma (human)RGD 
disseminated intravascular coagulation  ISOADAMTS13 (Homo sapiens)10449048; 10449048protein:decreased expression and decreased activity:plasma (human)RGD 

Objects Annotated

Genes (Rattus norvegicus)
Adamts13  (ADAM metallopeptidase with thrombospondin type 1 motif, 13)

Genes (Mus musculus)
Adamts13  (ADAM metallopeptidase with thrombospondin type 1 motif 13)

Genes (Homo sapiens)
ADAMTS13  (ADAM metallopeptidase with thrombospondin type 1 motif 13)


Additional Information