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Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis
Jainn-Jim Lin1, Oi-Wa Chan, Hsiang-Ju Hsiao
1From the Division of Pediatric Critical Care and Pediatric Sepsis Study Group (J-JL, O-WC, S-HH), Department of Pediatrics, Chang Gung Memorial Hospital, Keelung; College of Medicine (H-JH), Chang Gung University, Taoyuan; Department of Pediatrics (YW), Chang Gung Memorial Hospital, Chiayi; Graduate Institute of Clinical Medical Sciences (J-JL), College of Medicine, Chang Gung University; Division of Pediatric Infection (C-HC, J-JL); and Molecular Infectious Disease Research Center (C-HC, J-JL), Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Abstract:
Decreased ADAMTS 13 activity has been reported in severe sepsis and in sepsis-induced disseminated intravascular coagulation. This study aimed to investigate the role of ADAMTS 13 in different pediatric sepsis syndromes and evaluate its relationship with disease severity and outcome. We prospectively collected cases of sepsis treated in a pediatric intensive care unit, between July 2012 and June 2014 in Chang Gung Children's Hospital in Taoyuan, Taiwan. Clinical characteristics and ADAMTS-13 activity were analyzed. All sepsis syndromes had decreased ADAMTS 13 activity on days 1 and 3 of admission compared to healthy controls. Patients with septic shock had significantly decreased ADAMTS 13 activity on days 1 and 3 compared to those with sepsis and severe sepsis. There was a significant negative correlation between ADAMTS 13 activity on day 1 and day 1 PRISM-II, PELOD, P-MOD, and DIC scores. Patients with mortality had significantly decreased ADAMTS 13 activity on day 1 than survivors, but not on day 3. Different pediatric sepsis syndromes have varying degrees of decreased ADAMTS 13 activity. ADAMTS 13 activity is strongly negatively correlated with disease severity of pediatric sepsis syndrome, whereas decreased ADAMTS 13 activity on day 1 is associated with increased risk of mortality.
