title: Deficiency of ADAMTS-13 in pediatric patients with severe sepsis and impact on in-hospital mortality.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.forename: Farheen
authors.initials: F
authors.lastname: Karim
authors.forename: Salman Naseem
authors.initials: SN
authors.lastname: Adil
authors.forename: Bushra
authors.initials: B
authors.lastname: Afaq
authors.forename: Anwar
authors.initials: A
authors.lastname: Ul Haq
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000293:Adolescent; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010154:Pakistan / Q000453:epidemiology; D012189:Retrospective Studies; D018805:Sepsis / Q000097:blood* / Q000401:mortality; D015996:Survival Rate / Q000639:trends; D014841:von Willebrand Factor
chemical_list: D015415:Biomarkers; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
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