Related Experiment Video
Updated: Aug 17, 2026
Analysis of Group IV Viral SSHHPS Using In Vitro and In Silico Methods
Published on: December 21, 2019
VWF-cleaving protease (ADAMTS13) in premature infants
Lena Hellström-Westas1, David Ley, Ann-Cathrine Berg
1Department of Paediatrics, Lund University, Lund, Sweden. lena.westas@skane.se
Insights
Preterm infants may have lower levels of ADAMTS13, an enzyme deficient in thrombotic thrombocytopenic purpura. This deficiency might contribute to neurological complications like intraventricular hemorrhage and periventricular leucomalacia in premature infants.
Area of Science:
- Neonatal research
- Hematology
- Pediatric neurology
Background:
- Thrombotic thrombocytopenic purpura (TTP) is linked to deficiency in ADAMTS13, an enzyme that cleaves von Willebrand factor (VWF).
- This deficiency can cause platelet aggregation and ischemia.
- Low ADAMTS13 levels are hypothesized to contribute to intraventricular hemorrhage (IVH) and periventricular leucomalacia (PVL) in preterm infants.
Purpose of the Study:
- To investigate ADAMTS13 levels in preterm infants.
- To explore the potential association between ADAMTS13 levels and neurological complications in neonates.
Main Methods:
- Compared ADAMTS13 levels, VWF antigen, and VWF collagen binding activity in 9 preterm infants and 10 healthy term infants.
- Analyzed blood samples from umbilical cords at delivery.
Main Results:
- Preterm infants showed a trend towards lower ADAMTS13 levels compared to term infants.
- ADAMTS13 levels positively correlated with gestational age and birthweight in preterm infants.
- One preterm infant with the lowest ADAMTS13 level developed germinal matrix hemorrhage and PVL.
Conclusions:
- Preterm infants exhibit low ADAMTS13 levels, suggesting a potential role in neonatal complications.
- Enzyme substitution therapy is a potential future treatment for IVH and PVL if further studies confirm the association.
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.
More Related Videos
06:57Analysis of Transforming Growth Factor ß Family Cleavage Products Secreted Into the Blastocoele of Xenopus laevis Embryos
Published on: July 21, 2021
07:53A Fluorogenic Peptide Cleavage Assay to Screen for Proteolytic Activity: Applications for coronavirus spike protein activation
Published on: January 9, 2019