Cystic Fibrosis Related Liver Disease: Research Challenges and Future Perspectives
OBJECTIVES:Hepatobiliary complications are a leading cause of morbidity and mortality in cystic fibrosis (CF) patients. However, knowledge of the underlying pathological aspects and optimal clinical management is sorely lacking. METHODS:We provide a summary of the lectures given by international spe...
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| Veröffentlicht in: | Journal of pediatric gastroenterology and nutrition 2017-10, Vol.65 (4), p.443-448 |
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| creator | Debray, Dominique Narkewicz, Michael R Bodewes, Frank A.J.A Colombo, Carla Housset, Chantal de Jonge, Hugo R Jonker, Johan W Kelly, Deirdre A Ling, Simon C Poynard, Thierry Sogni, Philippe Trauner, Michael Witters, Peter Baumann, Ulrich Wilschanski, Michael Verkade, Henkjan J |
| description | OBJECTIVES:Hepatobiliary complications are a leading cause of morbidity and mortality in cystic fibrosis (CF) patients. However, knowledge of the underlying pathological aspects and optimal clinical management is sorely lacking.
METHODS:We provide a summary of the lectures given by international speakers at the ESPGHAN monothematic conference on CF-related liver disease (CFLD) held in Paris in January 2016, to discuss the status of our current knowledge of liver disease in CF patients, to define the critical areas that need to be addressed and to resolve actions to elucidate relevant mechanisms of disease to optimise future therapeutic options.
CONCLUSIONS:The need for a universal consensus on the definition of CFLD to clarify disease stage and to identify relevant biomarkers to assess disease severity was highlighted. A deeper understanding of the pathophysiology and prognostic factors for the long-term evolution of CFLD is fundamental to move forward, and has a strong bearing on identifying potential treatments. Novel experimental models and new treatment options under investigation are discussed and offer hope for the near future of CFLD. |
| doi_str_mv | 10.1097/MPG.0000000000001676 |
| format | Article |
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METHODS:We provide a summary of the lectures given by international speakers at the ESPGHAN monothematic conference on CF-related liver disease (CFLD) held in Paris in January 2016, to discuss the status of our current knowledge of liver disease in CF patients, to define the critical areas that need to be addressed and to resolve actions to elucidate relevant mechanisms of disease to optimise future therapeutic options.
CONCLUSIONS:The need for a universal consensus on the definition of CFLD to clarify disease stage and to identify relevant biomarkers to assess disease severity was highlighted. A deeper understanding of the pathophysiology and prognostic factors for the long-term evolution of CFLD is fundamental to move forward, and has a strong bearing on identifying potential treatments. Novel experimental models and new treatment options under investigation are discussed and offer hope for the near future of CFLD.</description><identifier>ISSN: 0277-2116</identifier><identifier>EISSN: 1536-4801</identifier><identifier>DOI: 10.1097/MPG.0000000000001676</identifier><identifier>PMID: 28753176</identifier><language>eng</language><publisher>United States: by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology</publisher><subject>Biomarkers - metabolism ; Cystic Fibrosis - complications ; Cystic Fibrosis - diagnosis ; Cystic Fibrosis - metabolism ; Cystic Fibrosis - therapy ; Gastrointestinal Agents - therapeutic use ; Humans ; Life Sciences ; Liver Diseases - diagnosis ; Liver Diseases - etiology ; Liver Diseases - metabolism ; Liver Diseases - therapy ; Liver Transplantation ; Pancreas Transplantation ; Prognosis</subject><ispartof>Journal of pediatric gastroenterology and nutrition, 2017-10, Vol.65 (4), p.443-448</ispartof><rights>2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3396-fd7aef72e313b1274137b289ae1242e896d3c8d19cfa949c00c7fc353ec475623</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,309,310,314,776,780,785,786,881,23909,23910,25118,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/28753176$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.sorbonne-universite.fr/hal-03975233$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Debray, Dominique</creatorcontrib><creatorcontrib>Narkewicz, Michael R</creatorcontrib><creatorcontrib>Bodewes, Frank A.J.A</creatorcontrib><creatorcontrib>Colombo, Carla</creatorcontrib><creatorcontrib>Housset, Chantal</creatorcontrib><creatorcontrib>de Jonge, Hugo R</creatorcontrib><creatorcontrib>Jonker, Johan W</creatorcontrib><creatorcontrib>Kelly, Deirdre A</creatorcontrib><creatorcontrib>Ling, Simon C</creatorcontrib><creatorcontrib>Poynard, Thierry</creatorcontrib><creatorcontrib>Sogni, Philippe</creatorcontrib><creatorcontrib>Trauner, Michael</creatorcontrib><creatorcontrib>Witters, Peter</creatorcontrib><creatorcontrib>Baumann, Ulrich</creatorcontrib><creatorcontrib>Wilschanski, Michael</creatorcontrib><creatorcontrib>Verkade, Henkjan J</creatorcontrib><title>Cystic Fibrosis Related Liver Disease: Research Challenges and Future Perspectives</title><title>Journal of pediatric gastroenterology and nutrition</title><addtitle>J Pediatr Gastroenterol Nutr</addtitle><description>OBJECTIVES:Hepatobiliary complications are a leading cause of morbidity and mortality in cystic fibrosis (CF) patients. However, knowledge of the underlying pathological aspects and optimal clinical management is sorely lacking.
METHODS:We provide a summary of the lectures given by international speakers at the ESPGHAN monothematic conference on CF-related liver disease (CFLD) held in Paris in January 2016, to discuss the status of our current knowledge of liver disease in CF patients, to define the critical areas that need to be addressed and to resolve actions to elucidate relevant mechanisms of disease to optimise future therapeutic options.
CONCLUSIONS:The need for a universal consensus on the definition of CFLD to clarify disease stage and to identify relevant biomarkers to assess disease severity was highlighted. A deeper understanding of the pathophysiology and prognostic factors for the long-term evolution of CFLD is fundamental to move forward, and has a strong bearing on identifying potential treatments. Novel experimental models and new treatment options under investigation are discussed and offer hope for the near future of CFLD.</description><subject>Biomarkers - metabolism</subject><subject>Cystic Fibrosis - complications</subject><subject>Cystic Fibrosis - diagnosis</subject><subject>Cystic Fibrosis - metabolism</subject><subject>Cystic Fibrosis - therapy</subject><subject>Gastrointestinal Agents - therapeutic use</subject><subject>Humans</subject><subject>Life Sciences</subject><subject>Liver Diseases - diagnosis</subject><subject>Liver Diseases - etiology</subject><subject>Liver Diseases - metabolism</subject><subject>Liver Diseases - therapy</subject><subject>Liver Transplantation</subject><subject>Pancreas Transplantation</subject><subject>Prognosis</subject><issn>0277-2116</issn><issn>1536-4801</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kF1PwjAUhhujEfz4B8bsUi-G_dq6ekdQwAQjIXrdlO5MpoVhu0H495aAxHhhb9qcPO97mgehK4I7BEtx9zwedPCvQ1KRHqE2SVga8wyTY9TGVIiYEpK20Jn3HwESPMGnqEUzkTAi0jaa9Da-Lk3UL6eu8qWPJmB1DXk0KlfgoofSg_ZwH8bh4cws6s20tbB4Bx_pRR71m7pxEI3B-SWYOoT8BToptPVwub_P0Vv_8bU3jEcvg6dedxQbxmQaF7nQUAgKjLApoYITJqY0kxoI5RQymebMZDmRptCSS4OxEYVhCQPDRZJSdo5ud73hR2rpyrl2G1XpUg27I7WdYSZFQhlbkcDe7Nilq74a8LWal96AtXoBVeMVkZQnknHJA8p3qAlCvIPi0E2w2ppXwbz6az7Ervcbmukc8kPoR3UAsh2wrmwddH3aZg1OzUDbevZ_9zcZjo3Y</recordid><startdate>20171001</startdate><enddate>20171001</enddate><creator>Debray, Dominique</creator><creator>Narkewicz, Michael R</creator><creator>Bodewes, Frank A.J.A</creator><creator>Colombo, Carla</creator><creator>Housset, Chantal</creator><creator>de Jonge, Hugo R</creator><creator>Jonker, Johan W</creator><creator>Kelly, Deirdre A</creator><creator>Ling, Simon C</creator><creator>Poynard, Thierry</creator><creator>Sogni, Philippe</creator><creator>Trauner, Michael</creator><creator>Witters, Peter</creator><creator>Baumann, Ulrich</creator><creator>Wilschanski, Michael</creator><creator>Verkade, Henkjan J</creator><general>by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology</general><general>Lippincott, Williams & Wilkins</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>1XC</scope></search><sort><creationdate>20171001</creationdate><title>Cystic Fibrosis Related Liver Disease: Research Challenges and Future Perspectives</title><author>Debray, Dominique ; Narkewicz, Michael R ; Bodewes, Frank A.J.A ; Colombo, Carla ; Housset, Chantal ; de Jonge, Hugo R ; Jonker, Johan W ; Kelly, Deirdre A ; Ling, Simon C ; Poynard, Thierry ; Sogni, Philippe ; Trauner, Michael ; Witters, Peter ; Baumann, Ulrich ; Wilschanski, Michael ; Verkade, Henkjan J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3396-fd7aef72e313b1274137b289ae1242e896d3c8d19cfa949c00c7fc353ec475623</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Biomarkers - metabolism</topic><topic>Cystic Fibrosis - complications</topic><topic>Cystic Fibrosis - diagnosis</topic><topic>Cystic Fibrosis - metabolism</topic><topic>Cystic Fibrosis - therapy</topic><topic>Gastrointestinal Agents - therapeutic use</topic><topic>Humans</topic><topic>Life Sciences</topic><topic>Liver Diseases - diagnosis</topic><topic>Liver Diseases - etiology</topic><topic>Liver Diseases - metabolism</topic><topic>Liver Diseases - therapy</topic><topic>Liver Transplantation</topic><topic>Pancreas Transplantation</topic><topic>Prognosis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Debray, Dominique</creatorcontrib><creatorcontrib>Narkewicz, Michael R</creatorcontrib><creatorcontrib>Bodewes, Frank A.J.A</creatorcontrib><creatorcontrib>Colombo, Carla</creatorcontrib><creatorcontrib>Housset, Chantal</creatorcontrib><creatorcontrib>de Jonge, Hugo R</creatorcontrib><creatorcontrib>Jonker, Johan W</creatorcontrib><creatorcontrib>Kelly, Deirdre A</creatorcontrib><creatorcontrib>Ling, Simon C</creatorcontrib><creatorcontrib>Poynard, Thierry</creatorcontrib><creatorcontrib>Sogni, Philippe</creatorcontrib><creatorcontrib>Trauner, Michael</creatorcontrib><creatorcontrib>Witters, Peter</creatorcontrib><creatorcontrib>Baumann, Ulrich</creatorcontrib><creatorcontrib>Wilschanski, Michael</creatorcontrib><creatorcontrib>Verkade, Henkjan J</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><jtitle>Journal of pediatric gastroenterology and nutrition</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Debray, Dominique</au><au>Narkewicz, Michael R</au><au>Bodewes, Frank A.J.A</au><au>Colombo, Carla</au><au>Housset, Chantal</au><au>de Jonge, Hugo R</au><au>Jonker, Johan W</au><au>Kelly, Deirdre A</au><au>Ling, Simon C</au><au>Poynard, Thierry</au><au>Sogni, Philippe</au><au>Trauner, Michael</au><au>Witters, Peter</au><au>Baumann, Ulrich</au><au>Wilschanski, Michael</au><au>Verkade, Henkjan J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cystic Fibrosis Related Liver Disease: Research Challenges and Future Perspectives</atitle><jtitle>Journal of pediatric gastroenterology and nutrition</jtitle><addtitle>J Pediatr Gastroenterol Nutr</addtitle><date>2017-10-01</date><risdate>2017</risdate><volume>65</volume><issue>4</issue><spage>443</spage><epage>448</epage><pages>443-448</pages><issn>0277-2116</issn><eissn>1536-4801</eissn><abstract>OBJECTIVES:Hepatobiliary complications are a leading cause of morbidity and mortality in cystic fibrosis (CF) patients. However, knowledge of the underlying pathological aspects and optimal clinical management is sorely lacking.
METHODS:We provide a summary of the lectures given by international speakers at the ESPGHAN monothematic conference on CF-related liver disease (CFLD) held in Paris in January 2016, to discuss the status of our current knowledge of liver disease in CF patients, to define the critical areas that need to be addressed and to resolve actions to elucidate relevant mechanisms of disease to optimise future therapeutic options.
CONCLUSIONS:The need for a universal consensus on the definition of CFLD to clarify disease stage and to identify relevant biomarkers to assess disease severity was highlighted. A deeper understanding of the pathophysiology and prognostic factors for the long-term evolution of CFLD is fundamental to move forward, and has a strong bearing on identifying potential treatments. Novel experimental models and new treatment options under investigation are discussed and offer hope for the near future of CFLD.</abstract><cop>United States</cop><pub>by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology</pub><pmid>28753176</pmid><doi>10.1097/MPG.0000000000001676</doi><tpages>6</tpages></addata></record> |
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| ispartof | Journal of pediatric gastroenterology and nutrition, 2017-10, Vol.65 (4), p.443-448 |
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| source | Journals@Ovid Ovid Autoload; MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Biomarkers - metabolism Cystic Fibrosis - complications Cystic Fibrosis - diagnosis Cystic Fibrosis - metabolism Cystic Fibrosis - therapy Gastrointestinal Agents - therapeutic use Humans Life Sciences Liver Diseases - diagnosis Liver Diseases - etiology Liver Diseases - metabolism Liver Diseases - therapy Liver Transplantation Pancreas Transplantation Prognosis |
| title | Cystic Fibrosis Related Liver Disease: Research Challenges and Future Perspectives |
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