Reduced ADAMTS13 in children with severe meningococcal sepsis is associated with severity and outcome

Thrombosis and Haemostasis

Volumn 103, Issue 6, 2010, Pages 1181-1187

  Bongers, Tamara N ^a   Emonts, Marieke ^b   De Maat, Moniek P M ^a   De Groot, Ronald ^c   Lisman, Ton ^d   Hazelzet, Jan A ^b   Leebeek, Frank W G ^a  

^a ERASMUS MEDICAL CENTER   (Netherlands)

^b SOPHIA CHILDREN S HOSPITAL   (Netherlands)

^c RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^d UNIVERSITY MEDICAL CENTER GRONINGEN   (Netherlands)

Author keywords

ADAMS ADAMTS 13; Disseminated intravascular coagulation (DIC); Fibrinolytic disorders; Paediatric haemostasis; Sepsis

Indexed keywords

COLLAGEN; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE; ADAM PROTEIN; ADAMTS13 PROTEIN, HUMAN;

ADOLESCENT; ARTICLE; CHILD; CORRELATION ANALYSIS; DISEASE SEVERITY; DISSEMINATED INTRAVASCULAR CLOTTING; ENZYME ACTIVATION; ENZYME LINKED IMMUNOSORBENT ASSAY; FEMALE; HEMOSTASIS; HUMAN; INFANT; INTENSIVE CARE; MAJOR CLINICAL STUDY; MALE; MENINGOCOCCEMIA; MORTALITY; PATHOPHYSIOLOGY; PRESCHOOL CHILD; PRIORITY JOURNAL; PROGNOSIS; PROTEIN BINDING; PROTEIN BLOOD LEVEL; PROTEIN EXPRESSION; SCHOOL CHILD; SEPSIS; BIOSYNTHESIS; BLOOD; DISEASE COURSE; GENETICS; IMMUNOLOGY; MENINGOCOCCOSIS; METABOLISM; NEISSERIA MENINGITIDIS; PATHOGENICITY; PROTEIN MULTIMERIZATION; SURVIVAL; TREATMENT OUTCOME;

ADAM PROTEINS; CHILD; CHILD, PRESCHOOL; DISEASE PROGRESSION; FEMALE; HUMANS; MALE; MENINGOCOCCAL INFECTIONS; NEISSERIA MENINGITIDIS; PROTEIN MULTIMERIZATION; SEPSIS; SURVIVAL ANALYSIS; TREATMENT OUTCOME; VON WILLEBRAND FACTOR;

EID: 77953143516     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1160/TH09-06-0376     Document Type: Article

References (28)

1. Booy R, Habibi P, Nadel S, et al. Reduction in case fatality rate from meningococcal disease associated with improved healthcare delivery. Arch Dis Child 2001; 85: 386-390.
2. Maat M, Buysse CM, Emonts M, et al. Improved survival of children with sepsis and purpura: effects of age, gender, and era. Critical Care 2007; 11: R112.
3. O'Brien JM, Jr., Ali NA, Aberegg SK, et al. Sepsis. Am J Med 2007; 120: 1012-1022.
4. Blann AD. Plasma von Willebrand factor, thrombosis, and the endothelium: the first 30 years. Thromb Haemost 2006; 95: 49-55.
5. Thompson SG, Kienast J, Pyke SD, et al. Hemostatic factors and the risk of myocardial infarction or sudden death in patients with angina pectoris. European Concerted Action on Thrombosis and Disabilities Angina Pectoris Study Group. New Engl J Med 1995; 332: 635-641.
6. Folsom AR, Rosamond WD, Shahar E, et al. Prospective study of markers of hemostatic function with risk of ischemic stroke. The Atherosclerosis Risk in Communities (ARIC) Study Investigators. Circulation 1999; 100: 736-742. (Pubitemid 29383644)
7. Catto AJ, Carter AM, Barrett JH, et al. von Willebrand factor and factor VIII: C in acute cerebrovascular disease. Relationship to stroke subtype and mortality. Thromb Haemost 1997; 77: 1104-1108.
8. Bongers TN, de Maat MP, van Goor ML, et al. High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability. Stroke 2006; 37: 2672-2677.
9. Miura M, Kaikita K, Matsukawa M, et al. Prognostic value of plasma von Willebrand factor-cleaving protease (ADAMTS13) antigen levels in patients with coronary artery disease. Thromb Haemost 2010; 103: 623-629.
10. van Mourik JA, Boertjes R, Huisveld IA, et al. von Willebrand factor propeptide in vascular disorders: A tool to distinguish between acute and chronic endothelial cell perturbation. Blood 1999; 94: 179-185.
11. Claus RA, Bockmeyer CL, Budde U, et al. Variations in the ratio between von Willebrand factor and its cleaving protease during systemic inflammation and association with severity and prognosis of organ failure. Thromb Haemost 2009; 101: 239-247.
12. Martin K, Borgel D, Lerolle N, et al. Decreased ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin type 1 repeats) is associated with a poor prognosis in sepsis-induced organ failure. Crit Care Med 2007; 35: 2375-2382.
13. Kremer Hovinga JA, Zeerleder S, Kessler P, et al. ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock. J Thromb Haemost 2007; 5: 2284-2290.
14. Ono T, Mimuro J, Madoiwa S, et al. 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. Blood 2006; 107: 528-534.
15. Nguyen TC, Liu A, Liu L, et al. Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis. Haematologica 2007; 92: 121-124.
16. de Kleijn ED, de Groot R, Hack CE, et al. Activation of protein C following infusion of protein C concentrate in children with severe meningococcal sepsis and purpura fulminans: a randomized, double-blinded, placebo-controlled, dosefinding study. Crit Care Med 2003; 31: 1839-1847.
17. den Brinker M, Joosten KF, Visser TJ, et al. Euthyroid sick syndrome in meningococcal sepsis: the impact of peripheral thyroid hormone metabolism and binding proteins. J Clin Endocrinol Metab 2005; 90: 5613-5620.
18. Pollack MM, Ruttimann UE, Getson PR. Pediatric risk of mortality (PRISM) score. Crit Care Med 1988; 16: 1110-1116.
19. Kornelisse RF, Hazelzet JA, Hop WC, et al. Meningococcal septic shock in children: clinical and laboratory features, outcome, and development of a prognostic score. Clin Infect Dis 1997; 25: 640-646.
20. Taylor FB, Jr., Toh CH, Hoots WK, et al. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost 2001; 86: 1327-1330.
21. Borchiellini A, Fijnvandraat K, ten Cate JW, et al. Quantitative analysis of von Willebrand factor propeptide release in vivo: effect of experimental endotoxemia and administration of 1-deamino-8-D-arginine vasopressin in humans. Blood 1996; 88: 2951-2958.
22. den Brinker M, Joosten KF, Liem O, et al. Adrenal insufficiency in meningococcal sepsis: bioavailable cortisol levels and impact of interleukin-6 levels and intubation with etomidate on adrenal function and mortality. J Clin Endocrinol Metab 2005; 90: 5110-5117.
23. Pralong G, Calandra T, Glauser MP, et al. Plasminogen activator inhibitor 1: a new prognostic marker in septic shock. Thromb Haemost 1989; 61: 459-462. (Pubitemid 19168155)
24. Boermeester MA, van Leeuwen PA, Coyle SM, et al. Interleukin-1 blockade attenuates mediator release and dysregulation of the hemostatic mechanism during human sepsis. Arch Surg 1995; 130: 739-748.
25. Bockmeyer CL, Claus RA, Budde U, et al. Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor. Haematologica 2008; 93: 137-140.
26. Hazelzet JA, Risseeuw-Appel IM, Kornelisse RF, et al. Age-related differences in outcome and severity of DIC in children with septic shock and purpura. Thromb Haemost 1996; 76: 932-938.
27. Bernard GR, Vincent JL, Laterre PF, et al. Efficacy and safety of recombinant human activated protein C for severe sepsis. New Engl J Med 2001; 344: 699-709.
28. Chauhan AK, Motto DG, Lamb CB, et al. Systemic antithrombotic effects of ADAMTS13. J Exp Med 2006; 203: 767-776.