Abstract
Resistance to activated protein C is a recently discovered genetic defect with a high prevalence in adult patients with thromboembolic disease. It is an autosomal dominant disorder and is ten times more common in these patients than antithrombin III-, protein C- and protein S deficiency together. In spite of this high prevalence among adults with thromboembolic disease no clinical manifestation in infancy so far has been reported. We describe a 4-year-old boy with a complex cardiac malformation and inherited resistance to activated protein C, who developed multiple thromboses after cardiac catheterization in early infancy.
Conclusion
Resistance to activated protein C can cause thrombosis in infants and children if additional risk factors for the development of thrombosis are present.
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Abbreviations
- APTT :
-
activated partial thromboplastin time
- APC :
-
activated protein C
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Zenz, W., Muntean, W., Gallistl, S. et al. Inherited resistance to activated protein C in a boy with multiple thromboses in early infancy. Eur J Pediatr 154, 285–288 (1995). https://doi.org/10.1007/BF01957363
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DOI: https://doi.org/10.1007/BF01957363