Expert management of congenital portosystemic shunts and their complications

被引：8

作者：

Mclin, Valerie Anne ^{[1
]}

Franchi-Abella, Stephanie ^{[2
,3
,4
,5
,6
]}

Bruetsch, Timothee ^{[7
]}

Bahadori, Atessa ^{[8
]}

Casotti, Valeria ^{[6
,9
]}

de Goyet, Jean de Ville ^{[10
]}

Dumery, Gregoire ^{[2
,11
]}

Gonzales, Emmanuel ^{[2
,5
,6
,12
,13
]}

Guerin, Florent ^{[2
,5
,6
,14
]}

Hascoet, Sebastien ^{[15
,16
]}

Heaton, Nigel ^{[17
]}

Kuhlmann, Beatrice ^{[18
]}

Lador, Frederic ^{[19
]}

Lambert, Virginie ^{[20
]}

Marra, Paolo ^{[21
]}

Plessier, Aurelie ^{[22
,23
]}

Quaglia, Alberto ^{[24
]}

Rougemont, Anne-Laure ^{[25
]}

Savale, Laurent ^{[26
,27
,28
]}

Sen Sarma, Moinak ^{[29
]}

Sitbon, Olivier ^{[26
,27
,28
]}

Superina, Riccardo Antonio ^{[30
]}

Uchida, Hajime ^{[31
]}

van Albada, Mirjam ^{[32
]}

van der Doef, Hubert Petrus Johannes ^{[33
]}

Vilgrain, Valerie ^{[34
,35
]}

Wacker, Julie ^{[36
,37
]}

Zwaveling, Nitash ^{[38
]}

Debray, Dominique ^{[5
,6
,39
,40
]}

Wildhaber, Barbara Elisabeth ^{[5
,41
]}

机构：

[1] Univ Geneva, Swiss Pediat Liver Ctr, Dept Pediat Gynecol & Obstet, Gastroenterol Hepatol & Pediat Nutr Unit, Geneva, Switzerland

[2] Univ Paris Saclay, Fac Med, Le Kremlin Bicetre, France

[3] Hop Bicetre, Ctr Reference Malad Rares Foie Enfant, Serv Radiol Pediat Diagnost & Intervent, APHP, Le Kremlin Bicetre, France

[4] BIOMAPS, INSERM, CEA, CNRS,UMR 9011, Orsay, France

[5] ERN Rare Liver, Hamburg, Germany

[6] ERN Transplant Child, Madrid, Spain

[7] Commun Sci, Rue Tunnel 7, Carouge, Switzerland

[8] Univ Geneva, Dept Pediat Gynecol & Obstet, Geneva, Switzerland

[9] ASST Papa Giovanni XXIII Hosp, Pediat Hepatol Gastroenterol & Transplant Ctr, Bergamo, Italy

[10] UPMC, ISMETT, Palermo, Italy

[11] Hop Bicetre, Serv Gynecol Obstet, AP HP, Le Kremlin Bicetre, France

[12] FHU Hepatinov, Hop Bicetre, Ctr Reference Malad Rares Foie Enfant, AP HP,Serv Dhepatol & Transplantat Hepat Pediat, Le Kremlin Bicetre, France

[13] INSERM, UMRS 1193, Orsay, France

[14] Hop Bicetre, AP HP, Serv Chirurg Pediat, Le Kremlin Bicetre, France

[15] Hop Marie Lannelongue, Dept Congenital Heart Dis, Le Plessis Robinson, France

[16] Univ Paris, UMR S 999, INSERM, Paris, France

[17] Kings Coll Hosp London, Inst Liver Studies, London, England

[18] Cantonal Hosp Aarau KSA, Pediat Endocrinol, Aarau, Switzerland

[19] Univ Geneva, Serv Pneumol, Geneva, Switzerland

[20] Inst Mutualiste Montsouris, Cardiol Congenitale, Paris, France

[21] Univ Milano Bicocca, Papa Giovanni Hosp 23, Sch Med & Surg, Dept Radiol, Bergamo, Italy

[22] Hop Beaujon, Ctr Reference Malad Vasc Foie, Serv Hepatol, Clichy, France

[23] VALDIG, Barcelona, Spain

[24] Royal Free London NHS Fdn Trust, Dept Cellular Pathol, Canc Inst, UCL, London, England

[25] Univ Geneva, Div Clin Pathol, Diagnost Dept, Swiss Pediat Liver Ctr, Geneva, Switzerland

[26] Hop Bicetre, AP HP, Ctr Reference Hypertens Plum, Serv Pneumol & Soins Intens Respiratoires, Le Kremlin Bicetre, France

[27] Hop Marie Lannelongue, INSERM, UMR S 999, Le Plessis Robinson, France

[28] ERN Lung, Frankfurt, Germany

[29] Sanjay Gandhi Postgrad Inst Med Sci, Dept Pediat Gastroenterol, Lucknow, India

[30] Northwestern Univ, Feinberg Sch Med, Ann & Robert H Lurie Childrens Hosp Chicago, Chicago, IL USA

[31] Natl Ctr Child Hlth & Dev, Organ Transplantat Ctr, Tokyo, Japan

[32] Univ Groningen, Univ Med Ctr Groningen, Dept Paediat & Congenital Cardiol, Groningen, Netherlands

[33] Univ Med Ctr Groningen, Dept Paediat, Div Paediat Gastroenterol & hHepatol, Groningen, Netherlands

[34] Univ Paris Cite, INSERM, CRI, Paris, France

[35] Hop Beaujon Nord, APHP, Dept Radiol, Clichy, France

[36] Univ Geneva, Dept Pediat Gynecol & Obstet, Pediat Cardiol Unit, Geneva, Switzerland

[37] Univ Geneva & Lausanne, Ctr Univ Romand Cardiol & Chirurg Cardiaque Pediat, Lausanne, Switzerland

[38] Univ Amsterdam, Med Ctr, Dept Pediat Endocrinol, Amsterdam, Netherlands

[39] Hop Necker Enfants Malad, Unite Hepatol Pediat & Transplantat Hepat, AP HP, Paris, France

[40] Ctr Reference Malad Rares Foie Enfant, FILFOIE, Paris, France

[41] Univ Geneva, Swiss Pediat Liver Ctr, Dept Pediat Surg, Dept Pediat Gynecol & Obstet, Geneva, Switzerland

来源：

JHEP REPORTS | 2024年 / 6卷 / 01期

关键词：

congenital portosystemic shunt; pulmonary hypertension; hepatocellular carcinoma; adenoma; focal nodular hyperplasia; b-catenin; hyperandrogenism; puberty; hypoglycemia; hyperinsulinism; occlusion test; portal pressure; PORTOPULMONARY HYPERTENSION; ABERNETHY MALFORMATION; PULMONARY-HYPERTENSION; LIVER-TRANSPLANTATION; VENOUS SHUNT; CHILDREN; CLASSIFICATION; DIAGNOSIS; MANIFESTATIONS; OUTCOMES;

D O I：

10.1016/j.jhepr.2023.100933

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

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. (c) 2023 The Author(s). Published by Elsevier B.V. on behalf of European Association for the Study of the Liver (EASL). This is an open access article under the CC BY license (http://creativecommons.org/licenses/ by/4.0/).

引用

页数：15

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