Expert management of congenital portosystemic shunts and their complications

Valérie Anne McLin; Stéphanie Franchi-Abella; Timothée Brütsch; Atessa Bahadori; Valeria Casotti; Jean de Ville de Goyet; Grégoire Dumery; Emmanuel Gonzales; Florent Guérin; Sebastien Hascoet; Nigel Heaton; B. atrice Kuhlmann; Frédéric Lador; Virginie Lambert; Paolo Marra; Aurélie Plessier; Alberto Quaglia; Anne-Laure Rougemont; Laurent Savale; Moinak Sen Sarma; Olivier Sitbon; Riccardo Antonio Superina; Hajime Uchida; Mirjam van Albada; Hubert Petrus Johannes van der Doef; Valérie Vilgrain; Julie Wacker; Nitash Zwaveling; Dominique Debray; Barbara Elisabeth Wildhaber

doi:10.1016/j.jhepr.2023.100933

Expert management of congenital portosystemic shunts and their complications

Valérie Anne McLin^*
, Stéphanie Franchi-Abella
, Timothée Brütsch
, Atessa Bahadori
, Valeria Casotti
, Jean de Ville de Goyet
, Grégoire Dumery
, Emmanuel Gonzales
, Florent Guérin
, Sebastien Hascoet
, Nigel Heaton
, B. atrice Kuhlmann
, Frédéric Lador
, Virginie Lambert
, Paolo Marra
, Aurélie Plessier
, Alberto Quaglia
, Anne-Laure Rougemont
, Laurent Savale
, Moinak Sen Sarma

Olivier Sitbon, Riccardo Antonio Superina, Hajime Uchida, Mirjam van Albada, Hubert Petrus Johannes van der Doef, Valérie Vilgrain, Julie Wacker, Nitash Zwaveling, Dominique Debray, Barbara Elisabeth Wildhaber

^*Corresponding author for this work

University of Geneva
ERN RARE LIVER
Université Paris-Saclay
Assistance publique – Hôpitaux de Paris
Laboratoire d’Imagerie Biomédicale Multimodale Paris Saclay
ERN Transplant Child
Communication in Science
USC Ematologia, ASST PAPA GIOVANNI XXIII, Bergamo, Italy
IRCCS Istituto Mediterraneo per i Trapianti e Terapie ad Alta Specializzazione (ISMETT) - Palermo
Institut national de la santé et de la recherche médicale
INSERM U999, Hospital Marie Lannelongue, Le Plessis Robinson, France
Université Paris Cité
King's College Hospital, London
Cantonal Hospital Aarau
Institut mutualiste Montsouris
University of Milan - Bicocca
Hôpital Beaujon
VALDIG
Department of Immunology, Royal Free London NHS Foundation Trust, London, UK
ERN Lung
Sanjay Gandhi Postgraduate Institute of Medical Sciences
Northwestern University
National Center for Child Health and Development
University of Groningen, University Medical Center Groningen
Universitaires de Médecine Légale
Amsterdam UMC - University of Amsterdam
Centre de Référence des maladies rares du foie de l'enfant

Research output: Contribution to journal › Review article › Academic › peer-review

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Abstract

Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

Original language	English
Article number	100933
Journal	JHEP Reports
Volume	6
Issue number	1
DOIs	https://doi.org/10.1016/j.jhepr.2023.100933
Publication status	Published - 1 Jan 2024
Externally published	Yes

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

Keywords

adenoma
congenital portosystemic shunt
focal nodular hyperplasia
hepatocellular carcinoma
hyperandrogenism
hyperinsulinism
hypoglycemia
occlusion test
portal pressure
puberty
pulmonary hypertension
β-catenin

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10.1016/j.jhepr.2023.100933

Expert-management-of-congenital-portosystemic-shunts-and-their-complications.pdfFinal published version, 2.4 MBLicence: CC BY

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McLin, V. A., Franchi-Abella, S., Brütsch, T., Bahadori, A., Casotti, V., de Ville de Goyet, J., Dumery, G., Gonzales, E., Guérin, F., Hascoet, S., Heaton, N., Kuhlmann, B. A., Lador, F., Lambert, V., Marra, P., Plessier, A., Quaglia, A., Rougemont, A.-L., Savale, L., ... Wildhaber, B. E. (2024). Expert management of congenital portosystemic shunts and their complications. JHEP Reports, 6(1), Article 100933. https://doi.org/10.1016/j.jhepr.2023.100933

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title = "Expert management of congenital portosystemic shunts and their complications",

abstract = "Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.",

keywords = "adenoma, congenital portosystemic shunt, focal nodular hyperplasia, hepatocellular carcinoma, hyperandrogenism, hyperinsulinism, hypoglycemia, occlusion test, portal pressure, puberty, pulmonary hypertension, β-catenin",

author = "McLin, \{Val{\'e}rie Anne\} and St{\'e}phanie Franchi-Abella and Timoth{\'e}e Br{\"u}tsch and Atessa Bahadori and Valeria Casotti and \{de Ville de Goyet\}, Jean and Gr{\'e}goire Dumery and Emmanuel Gonzales and Florent Gu{\'e}rin and Sebastien Hascoet and Nigel Heaton and Kuhlmann, \{B. atrice\} and Fr{\'e}d{\'e}ric Lador and Virginie Lambert and Paolo Marra and Aur{\'e}lie Plessier and Alberto Quaglia and Anne-Laure Rougemont and Laurent Savale and Sarma, \{Moinak Sen\} and Olivier Sitbon and Superina, \{Riccardo Antonio\} and Hajime Uchida and \{van Albada\}, Mirjam and \{Johannes van der Doef\}, \{Hubert Petrus\} and Val{\'e}rie Vilgrain and Julie Wacker and Nitash Zwaveling and Dominique Debray and Wildhaber, \{Barbara Elisabeth\}",

note = "Publisher Copyright: {\textcopyright} 2023 The Author(s)",

year = "2024",

month = jan,

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doi = "10.1016/j.jhepr.2023.100933",

language = "English",

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journal = "JHEP Reports",

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McLin, VA, Franchi-Abella, S, Brütsch, T, Bahadori, A, Casotti, V, de Ville de Goyet, J, Dumery, G, Gonzales, E, Guérin, F, Hascoet, S, Heaton, N, Kuhlmann, BA, Lador, F, Lambert, V, Marra, P, Plessier, A, Quaglia, A, Rougemont, A-L, Savale, L, Sarma, MS, Sitbon, O, Superina, RA, Uchida, H, van Albada, M, Johannes van der Doef, HP, Vilgrain, V, Wacker, J, Zwaveling, N, Debray, D & Wildhaber, BE 2024, 'Expert management of congenital portosystemic shunts and their complications', JHEP Reports, vol. 6, no. 1, 100933. https://doi.org/10.1016/j.jhepr.2023.100933

TY - JOUR

T1 - Expert management of congenital portosystemic shunts and their complications

AU - McLin, Valérie Anne

AU - Franchi-Abella, Stéphanie

AU - Brütsch, Timothée

AU - Bahadori, Atessa

AU - Casotti, Valeria

AU - de Ville de Goyet, Jean

AU - Dumery, Grégoire

AU - Gonzales, Emmanuel

AU - Guérin, Florent

AU - Hascoet, Sebastien

AU - Heaton, Nigel

AU - Kuhlmann, B. atrice

AU - Lador, Frédéric

AU - Lambert, Virginie

AU - Marra, Paolo

AU - Plessier, Aurélie

AU - Quaglia, Alberto

AU - Rougemont, Anne-Laure

AU - Savale, Laurent

AU - Sarma, Moinak Sen

AU - Sitbon, Olivier

AU - Superina, Riccardo Antonio

AU - Uchida, Hajime

AU - van Albada, Mirjam

AU - Johannes van der Doef, Hubert Petrus

AU - Vilgrain, Valérie

AU - Wacker, Julie

AU - Zwaveling, Nitash

AU - Debray, Dominique

AU - Wildhaber, Barbara Elisabeth

PY - 2024/1/1

Y1 - 2024/1/1

N2 - Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

AB - Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

KW - adenoma

KW - congenital portosystemic shunt

KW - focal nodular hyperplasia

KW - hepatocellular carcinoma

KW - hyperandrogenism

KW - hyperinsulinism

KW - hypoglycemia

KW - occlusion test

KW - portal pressure

KW - puberty

KW - pulmonary hypertension

KW - β-catenin

UR - https://www.scopus.com/pages/publications/85182174366

U2 - 10.1016/j.jhepr.2023.100933

DO - 10.1016/j.jhepr.2023.100933

M3 - Review article

C2 - 38234409

SN - 2589-5559

VL - 6

JO - JHEP Reports

JF - JHEP Reports

IS - 1

M1 - 100933

ER -

Expert management of congenital portosystemic shunts and their complications

Abstract

UN SDGs

Keywords

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