Document Detail


VWF-cleaving protease (ADAMTS13) in premature infants.
MedLine Citation:
PMID:  15981755     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Patients with thrombotic thrombocytopenic purpura (TTP) are deficient in von Willebrand factor (VWF)-cleaving protease, called ADAMTS13, and are prone to develop abnormal intravascular platelet aggregation leading to focal cerebral ischaemia. We speculated that low levels of ADAMTS13 are present in premature infants. This might result in platelet aggregation with subsequent ischaemia, vessel rupture and haemorrhage, and thus contribute to intraventricular haemorrhage and periventricular leucomalacia (IVH and PVL). PATIENTS AND METHODS: Nine preterm infants with gestational ages 23.7 to 30.9 (median 25.7) wk, and 10 healthy term control infants with gestational ages 36.9 to 39 (median 37.9) wk were included. Blood was sampled from the umbilical cord at delivery, and levels of ADAMTS13, VWF antigen and VWF collagen binding activity were analysed. RESULTS: The mean ADAMTS13 level in preterm infants was lower than in the term infants, but the difference between the groups was not statistically significant. However, in the preterm group there was a positive correlation between ADAMTS13 and both gestational age (r = 0.70, p = 0.035) and birthweight (r = 0.83, p = 0.005). Three preterm infants had ADAMTS13 of 18-20%. One of these developed a germinal matrix haemorrhage and PVL, and this infant had the lowest measured ADAMTS13 of all. The levels of VWF antigenand VWF collagen bindingactivity were higher in the preterm infants. CONCLUSION: This pilot study showed that preterm infants have low levels of ADAMTS13. Enzyme substitution may be a therapeutic option if an association with IVH or PVL can be confirmed in larger patient groups.
Authors:
Lena Hellström-Westas; David Ley; Ann-Cathrine Berg; Ann-Charlotte Kristoffersson; Lars Holmberg
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta paediatrica (Oslo, Norway : 1992)     Volume:  94     ISSN:  0803-5253     ISO Abbreviation:  Acta Paediatr.     Publication Date:  2005 Feb 
Date Detail:
Created Date:  2005-06-28     Completed Date:  2005-07-15     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9205968     Medline TA:  Acta Paediatr     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  205-10     Citation Subset:  IM    
Affiliation:
Department of Paediatrics, Lund University, Lund, Sweden. lena.westas@skane.se
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MeSH Terms
Descriptor/Qualifier:
ADAM Proteins
Birth Weight
Case-Control Studies
Gestational Age
Humans
Infant, Newborn
Infant, Premature / physiology*
Linear Models
Metalloendopeptidases / blood*
Pilot Projects
von Willebrand Factor / metabolism*
Chemical
Reg. No./Substance:
0/von Willebrand Factor; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human; EC 3.4.24.-/Metalloendopeptidases

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