{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T12:52:15Z","timestamp":1789131135593,"version":"build-2803163510"},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T00:00:00Z","timestamp":1764633600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T00:00:00Z","timestamp":1764633600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Polycystic liver disease (\n                    <jats:styled-content style=\"fixed-case\">PLD<\/jats:styled-content>\n                    ) is a rare genetic disorder characterised by progressive liver enlargement due to multiple cysts. The main symptoms are liver volume\u2010related. Although randomised controlled trials have shown that somatostatin analogues (\n                    <jats:styled-content style=\"fixed-case\">SSAs<\/jats:styled-content>\n                    ) reduce liver volume as well as symptoms, specific guidance on when and how to use\n                    <jats:styled-content style=\"fixed-case\">SSAs<\/jats:styled-content>\n                    in clinical practice is still lacking. A panel of 15 hepatologists and nephrologists developed practical guidance on\n                    <jats:styled-content style=\"fixed-case\">SSA<\/jats:styled-content>\n                    use, based on a systematic literature search, expert surveys, and clinical experience. This consensus was reached during a two\u2010day workshop by the European Reference Network on Hepatological Diseases, using 11 predefined key questions and an iterative Delphi process.\n                    <jats:styled-content style=\"fixed-case\">PLD<\/jats:styled-content>\n                    patients with liver volume\u2010related symptoms, diffuse disease, and significant hepatomegaly are eligible for\n                    <jats:styled-content style=\"fixed-case\">SSA<\/jats:styled-content>\n                    therapy, regardless of kidney function. Disease burden should be assessed with validated\n                    <jats:styled-content style=\"fixed-case\">PROMs<\/jats:styled-content>\n                    as well as liver volumetry before and during treatment. Symptom relief without liver growth is a valid treatment response. Therapy may continue until the natural course of disease slows down (e.g., with the onset of menopause). Trials report benefits of\n                    <jats:styled-content style=\"fixed-case\">SSA<\/jats:styled-content>\n                    therapy for up to 3\u20134\u2009years. Early discontinuation may cause a rebound of liver volume. As\n                    <jats:styled-content style=\"fixed-case\">SSA<\/jats:styled-content>\n                    therapy remains off\u2010label, limited access and reimbursement hamper widespread use in Europe. We present state\u2010of\u2010the\u2010art guidance on the practical use of the only available medical therapy for severe\n                    <jats:styled-content style=\"fixed-case\">PLD<\/jats:styled-content>\n                    including eligibility, start and stop criteria and identifying research gaps. This consensus\u2010based guidance provides much\u2010needed practical recommendations for the use of somatostatin analogues in managing severe polycystic liver disease. By defining eligibility, treatment goals, and monitoring strategies, it supports more standardised, patient\u2010centered care across Europe.\n                  <\/jats:p>","DOI":"10.1111\/liv.70451","type":"journal-article","created":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T10:56:56Z","timestamp":1764673016000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Medical Management of Polycystic Liver Disease: A Position Statement From the European Reference Network on Hepatological Diseases"],"prefix":"10.1111","volume":"46","author":[{"given":"Sophia","family":"Heinrich","sequence":"first","affiliation":[{"name":"Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology Hannover Medical School  Hannover Germany"},{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreia Margarida Carvalho","family":"de Matos","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Unidade Local de Sa\u00fade de Coimbra, EPE, Unidade de Transplantacao Hep\u00e1tica de Adultos, Servico de Medicina Iinterna \u2013 Unidade Funcional de Doenca Hep\u00e1tica, Centro Hospitalar e Universit\u00e1rio de Coimbra  Coimbra Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Colmenero","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Liver Transplantation, Liver Unit, Hospital Cl\u00ednic, Biomedical Research Institute August Pi i Sunyer (IDIBAPS), Biomedical Research Networking Center (CIBER) University of Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5369-3734","authenticated-orcid":false,"given":"Ahmed M.","family":"Elsharkawy","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"HR Birmingham Biomedical Research Centre University Hospitals Birmingham NHS Foundation Trust, University of Birmingham  Birmingham UK"},{"name":"Liver Unit Queen Elizabeth Hospital Birmingham  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"So\u0148a","family":"Fra\u0148kov\u00e1","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Institut klinick\u00e9 a experiment\u00e1ln\u00ed medic\u00edny, IK+EM  Praha Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Halbritter","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Medical Intensive Care Charit\u00e9 Universit\u00e4tsmedizin Berlin  Berlin Germany"},{"name":"European Reference Network for Rare Kidney Diseases (ERKNet)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Mrzljak","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Liver Transplant Center, University Hospital Center Zagreb School of Medicine, University of Zagreb  Zagreb Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roman\u2010Ulrich","family":"M\u00fcller","sequence":"additional","affiliation":[{"name":"Cluster of Excellence on Cellular Stress Responses in Aging\u2010Associated Diseases (CECAD) University of Cologne  Cologne Germany"},{"name":"Department II of Internal Medicine, University of Cologne Faculty of Medicine and University Hospital Cologne  Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafaela","family":"Pereira","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Hospital Curry Cabral  Lisboa Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pavel","family":"Strnad","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Medicine III University Hospital RWTH Aachen  Aachen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen A. J.","family":"Teemer","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jef","family":"Verbeek","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"University Hospitals KU Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karolina M.","family":"Wronka","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Hepatology, Transplantology and Internal Medicine Medical University of Warsaw  Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederik","family":"Nevens","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"University Hospitals KU Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Taubert","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology Hannover Medical School  Hannover Germany"},{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joost P. H.","family":"Drenth","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology Amsterdam University Medical Center  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the European Reference Network on Hepatological Liver Diseases (ERN RARE\u2010LIVER)","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,12,2]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1478\u20103231.2010.02247.x"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2009.07.052"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.2215\/cjn.05380709"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1681\/asn.2009121291"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2015.12.049"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2019.04.018"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.2215\/cjn.13661119"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2022.06.002"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2024.07.009"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0009\u20109236(99)70011\u20101"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2018.15870"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pmed.1002777"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40264\u2010016\u20100486\u2010x"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/cad.0000000000000967"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.28545"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2014.06.024"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ueg2.12387"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2023.12.007"},{"key":"e_1_2_13_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhepr.2022.100579"},{"key":"e_1_2_13_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2015.05.011"},{"key":"e_1_2_13_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/apt.12384"},{"key":"e_1_2_13_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0140\u20106736(13)61407\u20105"},{"key":"e_1_2_13_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598\u2010021\u201002812\u2010z"},{"key":"e_1_2_13_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/apt.13301"},{"key":"e_1_2_13_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/mcg.0000000000001749"},{"key":"e_1_2_13_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2017.11.024"},{"key":"e_1_2_13_28_1","doi-asserted-by":"publisher","DOI":"10.1177\/1756284818804784"},{"key":"e_1_2_13_29_1","doi-asserted-by":"publisher","DOI":"10.46619\/Cmj.2020.3-1023"},{"key":"e_1_2_13_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40268\u2010021\u201000342\u20107"},{"key":"e_1_2_13_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102265.2006.02639.x"},{"key":"e_1_2_13_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/risa.12947"},{"key":"e_1_2_13_33_1","doi-asserted-by":"publisher","DOI":"10.4065\/mcp.2010.0260"}],"container-title":["Liver International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.70451","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/liv.70451","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.70451","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T12:27:28Z","timestamp":1771331248000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/liv.70451"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,2]]},"references-count":32,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1111\/liv.70451"],"URL":"https:\/\/doi.org\/10.1111\/liv.70451","archive":["Portico"],"relation":{},"ISSN":["1478-3223","1478-3231"],"issn-type":[{"value":"1478-3223","type":"print"},{"value":"1478-3231","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,2]]},"assertion":[{"value":"2025-08-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-10","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-02","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70451"}}