Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease
Braz. j. med. biol. res
;
46(4): 375-381, 05/abr. 2013. tab, graf
Article
in English
| LILACS
| ID: lil-671392
ABSTRACT
Changes in plasma von Willebrand factor concentration (VWFAg) and ADAMTS-13 activity (the metalloprotease that cleaves VWF physiologically) have been reported in several cardiovascular disorders with prognostic implications. We therefore determined the level of these proteins in the plasma of children with cyanotic congenital heart disease (CCHD) undergoing surgical treatment. Forty-eight children were enrolled (age 0.83 to 7.58 years). Measurements were performed at baseline and 48 h after surgery. ELISA, collagen-binding assays and Western blotting were used to estimate antigenic and biological activities, and proteolysis of VWF multimers. Preoperatively, VWFAg and ADAMTS-13 activity were decreased (65 and 71% of normal levels considered as 113 (105-129) U/dL and 91 ± 24% respectively, P < 0.003) and correlated (r = 0.39, P = 0.0064). High molecular weight VWF multimers were not related, suggesting an interaction of VWF with cell membranes, followed by proteolytic cleavage. A low preoperative ADAMTS-13 activity, a longer activated partial thromboplastin time and the need for cardiopulmonary bypass correlated with postoperative bleeding (P < 0.05). Postoperatively, ADAMTS-13 activity increased but less extensively than VWFAg (respectively, 2.23 and 2.83 times baseline, P < 0.0001), resulting in an increased VWFAg/ADAMTS-13 activity ratio (1.20 to 1.54, respectively, pre- and postoperative median values, P = 0.0029). ADAMTS-13 consumption was further confirmed by decreased ADAMTS-13 antigenic concentration (0.91 ± 0.30 to 0.70 ± 0.25 µg/mL, P < 0.0001) and persistent proteolysis of VWF multimers. We conclude that, in pediatric CCHD, changes in circulating ADAMTS-13 suggest enzyme consumption, associated with abnormal structure and function of VWF.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Von Willebrand Factor
/
ADAM Proteins
/
Heart Defects, Congenital
Type of study:
Prognostic study
Limits:
Child
/
Child, preschool
/
Humans
/
Infant
Language:
English
Journal:
Braz. j. med. biol. res
Journal subject:
Biology
/
Medicine
Year:
2013
Type:
Article
/
Project document
Affiliation country:
Brazil
Institution/Affiliation country:
Fundação Pró-Sangue Hemocentro de São Paulo/BR
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