Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study
Autor: | Pier Luigi Calvo, Silvia Riva, Giuseppe Maggiore, Raffaele Iorio, Paola De Angelis, Maurizio Cheli, Lorenzo D'Antiga, Giuseppe Indolfi, Pietro Vajro, Emanuele Nicastro, Angelo Di Giorgio, Mara Cananzi |
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Přispěvatelé: | Di Giorgio, A., De Angelis, P., Cheli, M., Vajro, P., Iorio, R., Cananzi, M., Riva, S., Maggiore, G., Indolfi, G., Calvo, P. L., Nicastro, E., D'Antiga, L. |
Jazyk: | angličtina |
Rok vydání: | 2019 |
Předmět: |
Male
Diseases Infant Newborn Diseases 0302 clinical medicine Child Children Venous Thrombosis medicine.diagnostic_test Portal Vein Gastroenterology Portal vein thrombosis Venous thrombosis Italy 030220 oncology & carcinogenesis Child Preschool Hypertension Portal hypertension 030211 gastroenterology & hepatology Female Gastrointestinal Hemorrhage Infant Premature medicine.medical_specialty Gastrointestinal bleeding Adolescent Socio-culturale Esophageal and Gastric Varices Hypersplenism Catheterization 03 medical and health sciences Hypertension Portal medicine Humans Transjugular Intrahepatic Portasystemic Shunt Extra-hepatic portal vein obstruction Non-cirrhotic portal hypertension Infant Infant Newborn Portasystemic Shunt Transjugular Intrahepatic Splenomegaly Preschool Survival rate Premature Children Extra-hepatic portal vein obstruction Non-cirrhotic portal hypertension Portal vein thrombosis Hepatology business.industry medicine.disease Newborn Surgery Endoscopy Etiology Portal business Varices |
Popis: | Objectives Non-cirrhotic portal vein thrombosis (PVT) is a main cause of portal hypertension in children. We describe the characteristics at presentation and outcome of a cohort of patients with PVT to determine clinical features and predictors of outcome. Methods We recorded: (1) Associated factors: prematurity, congenital malformations, neonatal illnesses, umbilical vein catheterization (UVC), deep infections, surgery; (2) congenital and acquired prothrombotic disorders; (3) features at last follow up including survival rate and need for surgery. Results 187 patients, mean age at diagnosis 4 ± 3.7 years, had a history of prematurity (61%); UVC (65%); neonatal illnesses (79%). The diagnosis followed the detection of splenomegaly (40%), gastrointestinal bleeding (36%), hypersplenism (6%), or was incidental (18%). Of 71 patients who had endoscopy at presentation 62 (87%) had oesophageal varices. After 11.3 years’ follow up 63 (34%) required surgery or TIPS. Ten-year survival rate was 98%, with 90% shunt patency. Spleen size, variceal bleeding and hypersplenism at presentation were predictors of surgery or TIPS (p Conclusion PVT is associated with congenital and acquired co-morbidities. History of prematurity, neonatal illnesses and UVC should lead to rule out PVT. Large spleen, variceal bleeding and hypersplenism at presentation predict the need for eventual surgery in a third of cases. |
Databáze: | OpenAIRE |
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