Brief Article
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World J Gastroenterol. Dec 28, 2010; 16(48): 6123-6127
Published online Dec 28, 2010. doi: 10.3748/wjg.v16.i48.6123
Extrahepatic portal vein thrombosis in children and adolescents: Influence of genetic thrombophilic disorders
Andrea Pietrobattista, Matteo Luciani, Juan G Abraldes, Manila Candusso, Simona Pancotti, Massimo Soldati, Lidia Monti, Giuliano Torre, Valerio Nobili
Andrea Pietrobattista, Manila Candusso, Giuliano Torre, Valerio Nobili, Liver Unit, Research Institute, Bambino Gesù Children’s Hospital, 00165 Rome, Italy
Matteo Luciani, Department of Hematology, Bambino Gesù Children’s Hospital, 00165 Rome, Italy
Juan G Abraldes, Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, IDIBAPS and CIBERhed, University of Barcelona, 080036 Barcelona, Spain
Simona Pancotti, Massimo Soldati, Department of Laboratory Pediatrics, Bambino Gesù Children’s Hospital, 00165 Rome, Italy
Lidia Monti, Radiology Department, Bambino Gesù Children’s Hospital, 00165 Rome, Italy
Author contributions: Pietrobattista A and Luciani M designed the study and contributed equally to this work; Abraldes JG, Candusso M, Pancotti S, Soldati M and Monti L contributed to the collection and analysis of data; Torre G and Nobili V contributed to the manuscript preparation and revisions.
Supported by Grant from Bambino Gesù Children’s Hospital
Correspondence to: Valerio Nobili, MD, Liver Unit, Research Institute, Bambino Gesù Children’s Hospital, Piazza S. Onofrio 4, 00165 Rome, Italy. nobili66@yahoo.it
Telephone: +39-6-68592243 Fax: +39-6-68592192
Received: January 26, 2010
Revised: March 10, 2010
Accepted: March 17, 2010
Published online: December 28, 2010
Abstract

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 obstruction.

RESULTS: 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.

Keywords: Portal vein thrombosis; Children; Thrombophilic disorders; Protein C; Protein S