title: Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis.
abstract: We studied the state of ultra-large von Willebrand factor (ULVWF) proteolysis in 21 pediatric patients with severe sepsis and found that the overall group of patients had moderately reduced ADAMTS-13 activity, but 31% had severe enzymatic deficiency. The severe deficiency correlated with greater adhesion activity of von Willebrand factor, severity of thrombocytopenia and plasma levels of interleukin-6. It also correlated clinically with severity of illness and organ dysfunction. These results suggest that ULVWF proteolysis is insufficient in septic patients and severely deficient in a subgroup of patients. The deficiency may contribute to the development of thrombocytopenia and ischemic organ failure associated with sepsis.
authors.affiliation: Department of Pediatrics, Baylor College of Medicine, One Baylor Plaza, Houston, TX, USA.
authors.forename: Trung C
authors.initials: TC
authors.lastname: Nguyen
authors.forename: Anne
authors.initials: A
authors.lastname: Liu
authors.forename: Li
authors.initials: L
authors.lastname: Liu
authors.forename: Chalmette
authors.initials: C
authors.lastname: Ball
authors.forename: Hiuwan
authors.initials: H
authors.lastname: Choi
authors.forename: William S
authors.initials: WS
authors.lastname: May
authors.forename: Khatira
authors.initials: K
authors.lastname: Aboulfatova
authors.forename: Angela L
authors.initials: AL
authors.lastname: Bergeron
authors.forename: Jing-Fei
authors.initials: JF
authors.lastname: Dong
mesh_terms: D051722:ADAM Proteins / Q000096:biosynthesis / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005786:Gene Expression Regulation; D006801:Humans; D015850:Interleukin-6 / Q000097:blood; D008297:Male; D018805:Sepsis / Q000097:blood*; D013921:Thrombocytopenia / Q000097:blood / Q000209:etiology; D014841:von Willebrand Factor / Q000096:biosynthesis
chemical_list: D015850:Interleukin-6; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human