| Texto completo | |
| Autor(es): |
R.P.S. Soares
[1]
;
S.P. Bydlowski
[2]
;
N.M. Nascimento
[2]
;
A.M. Thomaz
[3]
;
E.N.M. Bastos
[3]
;
A.A. Lopes
[3]
Número total de Autores: 6
|
| Afiliação do(s) autor(es): | [1] Fundacao Prosangue Hemoctr Sao Paulo, Sao Paulo - Brazil
[2] Univ Sao Paulo, Fac Med, Lab Invest Med 31, Sao Paulo - Brazil
[3] Univ Sao Paulo, Fac Med, Inst Coracao, Sao Paulo - Brazil
Número total de Afiliações: 3
|
| Tipo de documento: | Artigo Científico |
| Fonte: | Brazilian Journal of Medical and Biological Research; v. 46, n. 4, p. 375-381, 2013-04-05. |
| Resumo | |
Changes in plasma von Willebrand factor concentration (VWF:Ag) 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, VWF:Ag 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 VWF:Ag (respectively, 2.23 and 2.83 times baseline, P < 0.0001), resulting in an increased VWF:Ag/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. (AU) | |
| Processo FAPESP: | 05/59890-9 - Comportamento da adamts-13 em relacao ao fator de von willebrand na condicao de hipoxemia. estudo em portadores de cardiopatias congenitas cianogenicas. |
| Beneficiário: | Rosangela de Paula Silva Soares |
| Modalidade de apoio: | Auxílio à Pesquisa - Regular |