Open Access Research article

Deficiency of ADAMTS-13 in pediatric patients with severe sepsis and impact on in-hospital mortality

Farheen Karim1*, Salman Naseem Adil1, Bushra Afaq1 and Anwar ul Haq2

Author Affiliations

1 Section of Hematology, Department of Pathology and Microbiology, The Aga Khan University Hospital, Stadium Road, Karachi, 74800, Pakistan

2 Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan

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BMC Pediatrics 2013, 13:44  doi:10.1186/1471-2431-13-44

Published: 28 March 2013



The enzyme involved in regulating the size of vWF (von Willebrand factor) in plasma is ADAMTS-13 (A disintegrin and metalloprotease with thrombospondin type-1 motives). Deficient proteolysis of ULvWF (ultra large von Willebrand factor) due to reduced ADAMTS-13 activity results in disseminated platelet-rich thrombi in the microcirculation characteristic of thrombotic thrombocytopenic purpura. Reduced ADAMTS-13 has also been observed in severe sepsis and is associated with poor survival. We conducted this study to detect ADAMTS-13 deficiency and its impact on in-hospital mortality in pediatric patients with severe sepsis.


Pediatric patients diagnosed with severe sepsis were recruited for the study. Baseline clinical characteristics were noted. ADAMTS-13 antigen levels were assayed by ELISA. According to ADAMTS-13 levels, patients were grouped as deficient and non-deficient. Comparison was done with regard to some clinical and biological characteristics and in-hospital mortality between the two groups.


A total of 80 patients were enrolled in the study. The median age of the patients was 3.1 years (Range: 0.1-15 years). ADAMTS-13 deficiency with levels less than 350 ng/dl was found in 65% patients. In patients with ADAMTS-13 deficiency, 75.6% had low platelets of less than 150 × 109/L. In-hospital mortality was 42.3% and 35.7% in ADAMTS-13 deficient and non-deficient group, respectively.


Majority of the pediatric patients admitted to hospital with severe sepsis exhibit ADAMTS-13 deficiency. ADAMTS-13 deficiency might play a role in sepsis-induced thrombocytopenia. More studies are needed to evaluate the role of ADAMTS-13 deficiency on in-hospital mortality.

ADAMTS-13; Sepsis; Deficiency; Pediatric