Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile
Background & aims : Progressive familial intrahepatic cholestasis (PFIC) is characterized by pruritus, intrahepatic cholestasis, low serum γ-glutamyltransferase levels, and characteristic “Byler bile” on electron microscopy. Many patients require liver transplantation, but partial external bilia...
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Veröffentlicht in: | Gastroenterology (New York, N.Y. 1943) N.Y. 1943), 2003-10, Vol.125 (4), p.1227-1234 |
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creator | Kurbegov, Amethyst C Setchell, Kenneth D.R Haas, Joel E Mierau, Gary W Narkewicz, Michael Bancroft, John D Karrer, Frederick Sokol, Ronald J |
description | Background & aims
:
Progressive familial intrahepatic cholestasis (PFIC) is characterized by pruritus, intrahepatic cholestasis, low serum γ-glutamyltransferase levels, and characteristic “Byler bile” on electron microscopy. Many patients require liver transplantation, but partial external biliary diversion (PEBD) has shown therapeutic promise. However, the effect of PEBD on liver morphology and bile composition has not been evaluated.
Methods
:
We reviewed liver biopsy specimens from 3 children with low γ-glutamyltransferase PFIC before and after PEBD. Follow-up liver biopsies were performed 9–60 months after PEBD. Light and electron microscopic features were scored blindly. Biliary bile acid composition was analyzed by gas chromatography—mass spectrometry before and after PEBD in 1 patient and after PEBD in 2 patients.
Results
:
Following PEBD, all patients improved clinically. Preoperative biopsy specimens showed characteristic features of PFIC, including portal fibrosis, chronic inflammation, cholestasis, giant cell transformation, and central venous mural sclerosis. Ultrastructural findings included coarse, granular canalicular Byler bile, effaced canalicular microvilli, and proliferative pericanalicular microfilaments. Following diversion, histology showed almost complete resolution of cholestasis, portal fibrosis, and inflammation with resolution of ultrastructural abnormalities. Biliary bile acids before PEBD consisted predominantly of cholic acid. After PEBD, the proportion of chenodeoxycholic acid increased significantly in 1 patient and was above the PFIC range in a second patient.
Conclusions
:
The resolution of hepatic morphologic abnormalities following PEBD supports PEBD as an effective therapy for PFIC. The improved biliary bile acid composition suggests enhanced bile acid secretion after PEBD, perhaps by induction of alternative canalicular transport proteins. |
doi_str_mv | 10.1016/S0016-5085(03)01199-5 |
format | Article |
fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_75719517</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S0016508503011995</els_id><sourcerecordid>75719517</sourcerecordid><originalsourceid>FETCH-LOGICAL-c391t-d85192f4ab29dbfd49bb5ef335594928984997a99fe69ae6baacb66bdab0271d3</originalsourceid><addsrcrecordid>eNqFkMFO3DAQhi1UBFvKI1D5UtQe0tpJnMRcUItaQELqoe3ZGttjMHLixc6uxNvXYVfl2Iste775Z_QRcsbZZ8549-UXK2cl2CA-suYT41zKShyQFRf1UJVa_Yas_iHH5G3Oj4wx2Qz8iBzzVvB-YO2KzN988JCeqfVbTNnHibqY6DrF-4Q5l0_qYFyYQP00J3jANczeUPMQA-YZss8X1I-lYYuWhiWFjjGtSzneP1OYLNU-IAXj7RLryuMdOXQQMp7u7xPy58f331c31d3P69urr3eVaSSfKzsILmvXgq6l1c62UmuBrmmEkK2sBzm0UvYgpcNOAnYawOiu0xY0q3tumxNyvsstc582ZVs1-mwwBJgwbrLqRc9lMVFAsQNNijkndGqd_Fi0KM7Uolu96FaLS8Ua9aJbidL3fj9go0e0r117vwX4sAcgGwguwWR8fuUE7zrZ1oW73HFYdGw9JpWNx8mg9QnNrGz0_1nlL9VXn1Y</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>75719517</pqid></control><display><type>article</type><title>Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile</title><source>MEDLINE</source><source>Access via ScienceDirect (Elsevier)</source><source>Alma/SFX Local Collection</source><creator>Kurbegov, Amethyst C ; Setchell, Kenneth D.R ; Haas, Joel E ; Mierau, Gary W ; Narkewicz, Michael ; Bancroft, John D ; Karrer, Frederick ; Sokol, Ronald J</creator><creatorcontrib>Kurbegov, Amethyst C ; Setchell, Kenneth D.R ; Haas, Joel E ; Mierau, Gary W ; Narkewicz, Michael ; Bancroft, John D ; Karrer, Frederick ; Sokol, Ronald J</creatorcontrib><description>Background & aims
:
Progressive familial intrahepatic cholestasis (PFIC) is characterized by pruritus, intrahepatic cholestasis, low serum γ-glutamyltransferase levels, and characteristic “Byler bile” on electron microscopy. Many patients require liver transplantation, but partial external biliary diversion (PEBD) has shown therapeutic promise. However, the effect of PEBD on liver morphology and bile composition has not been evaluated.
Methods
:
We reviewed liver biopsy specimens from 3 children with low γ-glutamyltransferase PFIC before and after PEBD. Follow-up liver biopsies were performed 9–60 months after PEBD. Light and electron microscopic features were scored blindly. Biliary bile acid composition was analyzed by gas chromatography—mass spectrometry before and after PEBD in 1 patient and after PEBD in 2 patients.
Results
:
Following PEBD, all patients improved clinically. Preoperative biopsy specimens showed characteristic features of PFIC, including portal fibrosis, chronic inflammation, cholestasis, giant cell transformation, and central venous mural sclerosis. Ultrastructural findings included coarse, granular canalicular Byler bile, effaced canalicular microvilli, and proliferative pericanalicular microfilaments. Following diversion, histology showed almost complete resolution of cholestasis, portal fibrosis, and inflammation with resolution of ultrastructural abnormalities. Biliary bile acids before PEBD consisted predominantly of cholic acid. After PEBD, the proportion of chenodeoxycholic acid increased significantly in 1 patient and was above the PFIC range in a second patient.
Conclusions
:
The resolution of hepatic morphologic abnormalities following PEBD supports PEBD as an effective therapy for PFIC. The improved biliary bile acid composition suggests enhanced bile acid secretion after PEBD, perhaps by induction of alternative canalicular transport proteins.</description><identifier>ISSN: 0016-5085</identifier><identifier>EISSN: 1528-0012</identifier><identifier>DOI: 10.1016/S0016-5085(03)01199-5</identifier><identifier>PMID: 14517804</identifier><identifier>CODEN: GASTAB</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Bile Acids and Salts - analysis ; Bile Acids and Salts - biosynthesis ; Biliary Tract Surgical Procedures ; Biological and medical sciences ; Biopsy ; Child ; Child, Preschool ; Cholestasis, Intrahepatic - metabolism ; Cholestasis, Intrahepatic - pathology ; Cholestasis, Intrahepatic - surgery ; Female ; gamma-Glutamyltransferase - blood ; Gas Chromatography-Mass Spectrometry ; Humans ; Liver - metabolism ; Liver - pathology ; Liver - ultrastructure ; Liver, biliary tract, pancreas, portal circulation, spleen ; Male ; Medical sciences ; Microscopy, Electron, Scanning ; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases ; Surgery of the digestive system</subject><ispartof>Gastroenterology (New York, N.Y. 1943), 2003-10, Vol.125 (4), p.1227-1234</ispartof><rights>2003 American Gastroenterological Association</rights><rights>2004 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c391t-d85192f4ab29dbfd49bb5ef335594928984997a99fe69ae6baacb66bdab0271d3</citedby><cites>FETCH-LOGICAL-c391t-d85192f4ab29dbfd49bb5ef335594928984997a99fe69ae6baacb66bdab0271d3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0016-5085(03)01199-5$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=15166942$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/14517804$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kurbegov, Amethyst C</creatorcontrib><creatorcontrib>Setchell, Kenneth D.R</creatorcontrib><creatorcontrib>Haas, Joel E</creatorcontrib><creatorcontrib>Mierau, Gary W</creatorcontrib><creatorcontrib>Narkewicz, Michael</creatorcontrib><creatorcontrib>Bancroft, John D</creatorcontrib><creatorcontrib>Karrer, Frederick</creatorcontrib><creatorcontrib>Sokol, Ronald J</creatorcontrib><title>Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile</title><title>Gastroenterology (New York, N.Y. 1943)</title><addtitle>Gastroenterology</addtitle><description>Background & aims
:
Progressive familial intrahepatic cholestasis (PFIC) is characterized by pruritus, intrahepatic cholestasis, low serum γ-glutamyltransferase levels, and characteristic “Byler bile” on electron microscopy. Many patients require liver transplantation, but partial external biliary diversion (PEBD) has shown therapeutic promise. However, the effect of PEBD on liver morphology and bile composition has not been evaluated.
Methods
:
We reviewed liver biopsy specimens from 3 children with low γ-glutamyltransferase PFIC before and after PEBD. Follow-up liver biopsies were performed 9–60 months after PEBD. Light and electron microscopic features were scored blindly. Biliary bile acid composition was analyzed by gas chromatography—mass spectrometry before and after PEBD in 1 patient and after PEBD in 2 patients.
Results
:
Following PEBD, all patients improved clinically. Preoperative biopsy specimens showed characteristic features of PFIC, including portal fibrosis, chronic inflammation, cholestasis, giant cell transformation, and central venous mural sclerosis. Ultrastructural findings included coarse, granular canalicular Byler bile, effaced canalicular microvilli, and proliferative pericanalicular microfilaments. Following diversion, histology showed almost complete resolution of cholestasis, portal fibrosis, and inflammation with resolution of ultrastructural abnormalities. Biliary bile acids before PEBD consisted predominantly of cholic acid. After PEBD, the proportion of chenodeoxycholic acid increased significantly in 1 patient and was above the PFIC range in a second patient.
Conclusions
:
The resolution of hepatic morphologic abnormalities following PEBD supports PEBD as an effective therapy for PFIC. The improved biliary bile acid composition suggests enhanced bile acid secretion after PEBD, perhaps by induction of alternative canalicular transport proteins.</description><subject>Bile Acids and Salts - analysis</subject><subject>Bile Acids and Salts - biosynthesis</subject><subject>Biliary Tract Surgical Procedures</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Cholestasis, Intrahepatic - metabolism</subject><subject>Cholestasis, Intrahepatic - pathology</subject><subject>Cholestasis, Intrahepatic - surgery</subject><subject>Female</subject><subject>gamma-Glutamyltransferase - blood</subject><subject>Gas Chromatography-Mass Spectrometry</subject><subject>Humans</subject><subject>Liver - metabolism</subject><subject>Liver - pathology</subject><subject>Liver - ultrastructure</subject><subject>Liver, biliary tract, pancreas, portal circulation, spleen</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Microscopy, Electron, Scanning</subject><subject>Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases</subject><subject>Surgery of the digestive system</subject><issn>0016-5085</issn><issn>1528-0012</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkMFO3DAQhi1UBFvKI1D5UtQe0tpJnMRcUItaQELqoe3ZGttjMHLixc6uxNvXYVfl2Iste775Z_QRcsbZZ8549-UXK2cl2CA-suYT41zKShyQFRf1UJVa_Yas_iHH5G3Oj4wx2Qz8iBzzVvB-YO2KzN988JCeqfVbTNnHibqY6DrF-4Q5l0_qYFyYQP00J3jANczeUPMQA-YZss8X1I-lYYuWhiWFjjGtSzneP1OYLNU-IAXj7RLryuMdOXQQMp7u7xPy58f331c31d3P69urr3eVaSSfKzsILmvXgq6l1c62UmuBrmmEkK2sBzm0UvYgpcNOAnYawOiu0xY0q3tumxNyvsstc582ZVs1-mwwBJgwbrLqRc9lMVFAsQNNijkndGqd_Fi0KM7Uolu96FaLS8Ua9aJbidL3fj9go0e0r117vwX4sAcgGwguwWR8fuUE7zrZ1oW73HFYdGw9JpWNx8mg9QnNrGz0_1nlL9VXn1Y</recordid><startdate>20031001</startdate><enddate>20031001</enddate><creator>Kurbegov, Amethyst C</creator><creator>Setchell, Kenneth D.R</creator><creator>Haas, Joel E</creator><creator>Mierau, Gary W</creator><creator>Narkewicz, Michael</creator><creator>Bancroft, John D</creator><creator>Karrer, Frederick</creator><creator>Sokol, Ronald J</creator><general>Elsevier Inc</general><general>Elsevier</general><scope>IQODW</scope><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></search><sort><creationdate>20031001</creationdate><title>Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile</title><author>Kurbegov, Amethyst C ; Setchell, Kenneth D.R ; Haas, Joel E ; Mierau, Gary W ; Narkewicz, Michael ; Bancroft, John D ; Karrer, Frederick ; Sokol, Ronald J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c391t-d85192f4ab29dbfd49bb5ef335594928984997a99fe69ae6baacb66bdab0271d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Bile Acids and Salts - analysis</topic><topic>Bile Acids and Salts - biosynthesis</topic><topic>Biliary Tract Surgical Procedures</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Cholestasis, Intrahepatic - metabolism</topic><topic>Cholestasis, Intrahepatic - pathology</topic><topic>Cholestasis, Intrahepatic - surgery</topic><topic>Female</topic><topic>gamma-Glutamyltransferase - blood</topic><topic>Gas Chromatography-Mass Spectrometry</topic><topic>Humans</topic><topic>Liver - metabolism</topic><topic>Liver - pathology</topic><topic>Liver - ultrastructure</topic><topic>Liver, biliary tract, pancreas, portal circulation, spleen</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Microscopy, Electron, Scanning</topic><topic>Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases</topic><topic>Surgery of the digestive system</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kurbegov, Amethyst C</creatorcontrib><creatorcontrib>Setchell, Kenneth D.R</creatorcontrib><creatorcontrib>Haas, Joel E</creatorcontrib><creatorcontrib>Mierau, Gary W</creatorcontrib><creatorcontrib>Narkewicz, Michael</creatorcontrib><creatorcontrib>Bancroft, John D</creatorcontrib><creatorcontrib>Karrer, Frederick</creatorcontrib><creatorcontrib>Sokol, Ronald J</creatorcontrib><collection>Pascal-Francis</collection><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><jtitle>Gastroenterology (New York, N.Y. 1943)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kurbegov, Amethyst C</au><au>Setchell, Kenneth D.R</au><au>Haas, Joel E</au><au>Mierau, Gary W</au><au>Narkewicz, Michael</au><au>Bancroft, John D</au><au>Karrer, Frederick</au><au>Sokol, Ronald J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile</atitle><jtitle>Gastroenterology (New York, N.Y. 1943)</jtitle><addtitle>Gastroenterology</addtitle><date>2003-10-01</date><risdate>2003</risdate><volume>125</volume><issue>4</issue><spage>1227</spage><epage>1234</epage><pages>1227-1234</pages><issn>0016-5085</issn><eissn>1528-0012</eissn><coden>GASTAB</coden><abstract>Background & aims
:
Progressive familial intrahepatic cholestasis (PFIC) is characterized by pruritus, intrahepatic cholestasis, low serum γ-glutamyltransferase levels, and characteristic “Byler bile” on electron microscopy. Many patients require liver transplantation, but partial external biliary diversion (PEBD) has shown therapeutic promise. However, the effect of PEBD on liver morphology and bile composition has not been evaluated.
Methods
:
We reviewed liver biopsy specimens from 3 children with low γ-glutamyltransferase PFIC before and after PEBD. Follow-up liver biopsies were performed 9–60 months after PEBD. Light and electron microscopic features were scored blindly. Biliary bile acid composition was analyzed by gas chromatography—mass spectrometry before and after PEBD in 1 patient and after PEBD in 2 patients.
Results
:
Following PEBD, all patients improved clinically. Preoperative biopsy specimens showed characteristic features of PFIC, including portal fibrosis, chronic inflammation, cholestasis, giant cell transformation, and central venous mural sclerosis. Ultrastructural findings included coarse, granular canalicular Byler bile, effaced canalicular microvilli, and proliferative pericanalicular microfilaments. Following diversion, histology showed almost complete resolution of cholestasis, portal fibrosis, and inflammation with resolution of ultrastructural abnormalities. Biliary bile acids before PEBD consisted predominantly of cholic acid. After PEBD, the proportion of chenodeoxycholic acid increased significantly in 1 patient and was above the PFIC range in a second patient.
Conclusions
:
The resolution of hepatic morphologic abnormalities following PEBD supports PEBD as an effective therapy for PFIC. The improved biliary bile acid composition suggests enhanced bile acid secretion after PEBD, perhaps by induction of alternative canalicular transport proteins.</abstract><cop>New York, NY</cop><pub>Elsevier Inc</pub><pmid>14517804</pmid><doi>10.1016/S0016-5085(03)01199-5</doi><tpages>8</tpages></addata></record> |
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subjects | Bile Acids and Salts - analysis Bile Acids and Salts - biosynthesis Biliary Tract Surgical Procedures Biological and medical sciences Biopsy Child Child, Preschool Cholestasis, Intrahepatic - metabolism Cholestasis, Intrahepatic - pathology Cholestasis, Intrahepatic - surgery Female gamma-Glutamyltransferase - blood Gas Chromatography-Mass Spectrometry Humans Liver - metabolism Liver - pathology Liver - ultrastructure Liver, biliary tract, pancreas, portal circulation, spleen Male Medical sciences Microscopy, Electron, Scanning Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Surgery of the digestive system |
title | Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile |
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