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Extrahepatic portal vein thrombosis in children and adolescents:Influence of genetic thrombophilic disorders

World Journal of Gastroenterology 2010年第48期16卷 6123-6127页

作者： Andrea Pietrobattista Matteo Luciani Juan G Abraldes Manila Candusso Simona Pancotti massimo soldati Lidia Monti Giuliano Torre Valerio Nobili Liver Unit Research InstituteBambino Gesù Children's Hospital Department of Hematology Bambino Gesù Children's Hospital Hepatic Hemodynamic Laboratory Liver UnitHospital ClínicIDIBAPS and CIBERhedUniversity of Barcelona Department of Laboratory Pediatrics Bambino Gesù Children's Hospital Radiology Department Bambino Gesù Children's Hospital

AIM:To explore the prevalence of local and genetic thrombophilic disorders as risk factors for portal vein thrombosis (PVT) in our series,the largest ever published in pediatric literature. METHODS:We conducted a case-control study enrolling 31 children with PVT and 26 age-matched controls. All were screened for thrombophilia,including genetic disorders,protein C,protein S and homocysteine deficiencies. All coagulation parameters were studied at least 3 mo after the diagnosis of portal vein ***:In our study we showed that most pediatric patients with PVT have local prothrombotic risk factors,which are probably the most important factors leading to PVT. However,there is a clear association between the presence of prothrombotic disorders and PVT,suggesting that these increase the risk of thrombosis in patients with local factors such as perinatal umbilical vein catheterization or sepsis. CONCLUSION:Patients with PVT should be screened for inherited prothrombotic disorders regardless of a history of an obvious local risk factor.

关键词： Portal vein thrombosis Children Thrombophilic disorders Protein C Protein S

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