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Title: Congenital extrahepatic portosystemic shunts (Abernethy malformation): An international observational study
Author: Baiges Aznar, Anna
Turon, Fanny
Simón Talero, Macarena
Tasayco, Stephanie
Bueno, Javier
Zekrini, Kamal
Plessier, Aurélie
Franchi-Abella, Stéphanie
Guerin, Florent
Mukund, Amar
Eapen, C.E.
Goel, Ashish
Shyamkumar, Nidugala K.
Coenen, Sandra
Gottardi, Andrea de
Majumdar, Avik
Onali, Simona
Shukla, Akash
Carrilho, Flair José
Nacif, Lucas
Primignani, Massimo
Tosetti, Giulia
La Mura, Vincenzo
Nevens, Frederick
Witters, Peter
Tripathi, Dhiraj
Tellez, Luis
Martínez González, Javier
Álvarez-Navascués, Carmen
López Fraile López, Miguel
Procopet, Bogdan
Piscaglia, Fabio
Koning, Barbara de
Llop, Elba
Romero Cristobal, Mario
Tjwa, Eric
Monescillo-Francia, Alberto
Senzolo, Marco
Perez-LaFuente, Mercedes
Garcia Criado, María Ángeles
Segarra, Antonio
Kumar Sarin, Shiv
Hernández Gea, Virginia
Patch, David
Laleman, Wim
Hartog, Hermien
Valla, Dominique
Genescà, Joan
García Pagán, Juan Carlos
Keywords: Malalties del fetge
Malalties hereditàries
Liver diseases
Genetic diseases
Issue Date: 18-Jun-2019
Publisher: Wiley
Abstract: Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.
Note: Versió postprint del document publicat a:
It is part of: Hepatology, 2019, vol. 71, num. 2, p. 658-669
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ISSN: 0270-9139
Appears in Collections:Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)
Articles publicats en revistes (Fonaments Clínics)

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