Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis.
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.
Nguyen
Trung C
TC
Department of Pediatrics, Baylor College of Medicine, One Baylor Plaza, Houston, TX, USA.
Liu
Anne
A
Liu
Li
L
Ball
Chalmette
C
Choi
Hiuwan
H
May
William S
WS
Aboulfatova
Khatira
K
Bergeron
Angela L
AL
Dong
Jing-Fei
JF
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
D015850:Interleukin-6; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human