Association of ADAMTS-13 Activity with Clinical Outcomes in Hospitalized Children with Infections
2Department of Clinical Skills and Medical Technology, Riga Stradins University
3Department of Human Physiology and Biochemistry, Riga Stradins University
4Clinic of Children’s Diseases, Children’s Clinical University Hospital
Background: Deficient proteolysis of ultra-large von Willebrand factor multimere caused by decreased activity of ADAMTS-13 may result in thrombotic microangiopathy, which is one of the organ damage mechanisms in septic patients.
Objective: The aim of this work was to analyze ADAMTS-13 blood concentration in association with clinical disease characteristics and outcomes in hospitalized children.
Methods: The study was conducted in a tertiary children’s hospital in Latvia. Children aged from 1 day to 18 years hospitalized with infections of various localization were enrolled prospectively during the period from February 2015 to January 2017. Complete blood count, C-reactive protein (CRP), interleukin-6 (IL-6), and procalcitonin (PCT) were performed using standardized methods. ADAMTS-13 was determined by Quantikine ELISA Human ADAMTS-13 Immunoassay test.
Results: During the study period, 77 patients were enrolled – 31 (40.3%) girls and 46 (59.7%) boys with average age of 52 months (interquartile range 16.5-87.5). Median ADAMTS-13 concentration was 693.38 ng/ml ( interquartile range 584.75-770.37). Diagnosis of sepsis was established in 51 (66.2%) children and the mean ADAMTS-13 concentration was significantly lower in these patients (mean difference 110.08 ng/ml; 95% CI 51.77–168.37; p<0.001). Sepsis was defined as severe infection with life-threatening organ dysfunction. Decreased ADAMTS-13 concentrations correlated with increased CRP (r = -0.323; p=0.004), IL-6 (r=-0.226; p=0.049), length of stay in hospital (r=-0.425; p<0.001) and in paediatric intensive care unit (r=-0.238; p=0.037), and a tendency to longer mechanical ventilation in intubated patients (r=-0.217; p=0.058). The mean ADAMTS-13 concentration was significantly lower in paediatric intensive care unit patients, compared to patients hospitalized on general paediatric wards (mean difference 159.04 ng/ml; 95% CI 69.29–248.8; p=0.001).
Conclusion: Decreased ADAMTS-13 concentration was associated with more pronounced inflammation and sepsis, poorer clinical outcomes in hospitalized children with infection. It makes this protein interesting as a potential diagnostic marker of the organ damage.
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