Maria Magnusson1, Vera Ignjatovic2, Winita Hardikar3, Paul Monagle4. 1. CLINTEC, Division of Pediatrics, Karolinska Institutet, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden MMK, Clinical Chemistry and Blood Coagulation Research, Karolinska Institutet, Stockholm, Sweden Department of Paediatrics, University of Melbourne, Melbourne, Australia Haematology Research, Murdoch Childrens Research Institute, Melbourne, Australia. 2. Department of Paediatrics, University of Melbourne, Melbourne, Australia Haematology Research, Murdoch Childrens Research Institute, Melbourne, Australia. 3. Department of Paediatrics, University of Melbourne, Melbourne, Australia Department of Gastroenterology, Royal Children's Hospital, Melbourne, Australia. 4. Department of Paediatrics, University of Melbourne, Melbourne, Australia Haematology Research, Murdoch Childrens Research Institute, Melbourne, Australia Department of Clinical Haematology, Royal Children's Hospital, Melbourne, Australia.
Abstract
UNLABELLED: Children with liver disease can develop severe bleeding episodes and thrombosis. Liver failure usually results in decreased levels of procoagulant and anticoagulant factors. Additional risk factors, including changes in vascular flow and endothelial function, are of importance for the development of bleeding or thrombosis in individual vascular beds. Detailed studies of haemostatic disturbances in the setting of paediatric liver disease are sparse and extrapolation from adult studies is common. The spectrum of liver diseases and the haemostatic system differs between children and adults. Specific paediatric liver diseases are reported to have more distinctive effects on haemostasis and the risk of bleeding and/or thrombosis. CONCLUSION: we propose a model regarding haemostasis in paediatric liver disease, taking into account a number of specific variables and mechanisms, as well as the type of liver disease, which will provide a framework for clinical decision-making in these complex patients. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
UNLABELLED: Children with liver disease can develop severe bleeding episodes and thrombosis. Liver failure usually results in decreased levels of procoagulant and anticoagulant factors. Additional risk factors, including changes in vascular flow and endothelial function, are of importance for the development of bleeding or thrombosis in individual vascular beds. Detailed studies of haemostatic disturbances in the setting of paediatric liver disease are sparse and extrapolation from adult studies is common. The spectrum of liver diseases and the haemostatic system differs between children and adults. Specific paediatric liver diseases are reported to have more distinctive effects on haemostasis and the risk of bleeding and/or thrombosis. CONCLUSION: we propose a model regarding haemostasis in paediatric liver disease, taking into account a number of specific variables and mechanisms, as well as the type of liver disease, which will provide a framework for clinical decision-making in these complex patients. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
Authors: Sara Zeynelabidin; Femke C C Klouwer; Joost C M Meijers; Monique H Suijker; Marc Engelen; Bwee Tien Poll-The; C Heleen van Ommen Journal: J Inherit Metab Dis Date: 2017-11-14 Impact factor: 4.982