Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis | Zendy

Tom C. Nguyen | Zendy; An Liu | Zendy; L. Liu | Zendy; Chalmette Ball | Zendy; Hannah Choi | Zendy; W. Stratford May | Zendy; Khatira Aboulfatova | Zendy; Angila Bergeron | Zendy; Jing Dong | Zendy

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Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis

Author(s) -

Tom C. Nguyen,

An Liu,

L. Liu,

Chalmette Ball,

Hannah Choi,

W. Stratford May,

Khatira Aboulfatova,

Angila Bergeron,

Jing Dong

Publication year - 2007

Publication title -

haematologica

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.782

H-Index - 142

eISSN - 1592-8721

pISSN - 0390-6078

DOI - 10.3324/haematol.10262

Subject(s) - von willebrand factor , sepsis , medicine , adamts , proteolysis , gastroenterology , severe sepsis , immunology , metalloproteinase , platelet , biology , septic shock , enzyme , matrix metalloproteinase , biochemistry , thrombospondin

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.

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