title: Extrahepatic portal vein thrombosis in children and adolescents: Influence of genetic thrombophilic disorders.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  patients with PVT should be screened for inherited prothrombotic disorders regardless of a history of an obvious local risk factor.
authors.affiliation: Research Institute, Bambino Gesù Children's Hospital, Piazza S. Onofrio 4, 00165 Rome, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Pietrobattista
authors.forename: Matteo
authors.initials: M
authors.lastname: Luciani
authors.forename: Juan G
authors.initials: JG
authors.lastname: Abraldes
authors.forename: Manila
authors.initials: M
authors.lastname: Candusso
authors.forename: Simona
authors.initials: S
authors.lastname: Pancotti
authors.forename: Massimo
authors.initials: M
authors.lastname: Soldati
authors.forename: Lidia
authors.initials: L
authors.lastname: Monti
authors.forename: Giuliano
authors.initials: G
authors.lastname: Torre
authors.forename: Valerio
authors.initials: V
authors.lastname: Nobili
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D006710:Homocysteine / Q000172:deficiency; D006801:Humans; D008297:Male; D011169:Portal Vein / Q000473:pathology*; D011446:Prospective Studies; D011486:Protein C / Q000378:metabolism; D017293:Protein S / Q000378:metabolism; D012307:Risk Factors; D019851:Thrombophilia / Q000150:complications* / Q000453:epidemiology / Q000235:genetics*; D020246:Venous Thrombosis / Q000453:epidemiology / Q000209:etiology*
chemical_list: D011486:Protein C; D017293:Protein S; D006710:Homocysteine