{"id":"https://openalex.org/W4387733041","doi":"https://doi.org/10.14309/01.ajg.0000964364.18595.ae","title":"S3681 Congenital Hepatic Fibrosis in Adulthood","display_name":"S3681 Congenital Hepatic Fibrosis in Adulthood","publication_year":2023,"publication_date":"2023-10-01","ids":{"openalex":"https://openalex.org/W4387733041","doi":"https://doi.org/10.14309/01.ajg.0000964364.18595.ae"},"language":"en","primary_location":{"id":"doi:10.14309/01.ajg.0000964364.18595.ae","doi":"https://doi.org/10.14309/01.ajg.0000964364.18595.ae","is_oa":false,"landing_page_url":"https://doi.org/10.14309/01.ajg.0000964364.18595.ae","pdf_url":null,"source":{"id":"https://openalex.org/S66441642","display_name":"The American Journal of Gastroenterology","issn_l":"0002-9270","issn":["0002-9270","1572-0241"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American Journal of Gastroenterology","raw_type":"journal-article"},"volume":null,"type":"conference-abstract","indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5070973168","display_name":"Muaaz Masood","orcid":"https://orcid.org/0000-0001-5219-6277","observed_orcids":["https://orcid.org/0000-0001-5219-6277"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Muaaz Masood","raw_affiliation_strings":["Presenter"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Presenter","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5063647285","display_name":"Richard A. Kozarek","orcid":"https://orcid.org/0000-0002-9745-7538","observed_orcids":["https://orcid.org/0000-0002-9745-7538"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Richard Kozarek","raw_affiliation_strings":["Virginia Mason Franciscan Health, Seattle, WA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Virginia Mason Franciscan Health, Seattle, WA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5011828646","display_name":"Blaire E. Burman","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Blaire Burman","raw_affiliation_strings":["Virginia Mason Franciscan Health, Seattle, WA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Virginia Mason Franciscan Health, Seattle, WA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5052084689","display_name":"Rajesh Krishnamoorthi","orcid":"https://orcid.org/0000-0002-1889-2129","observed_orcids":["https://orcid.org/0000-0002-1889-2129"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Rajesh Krishnamoorthi","raw_affiliation_strings":["Virginia Mason Franciscan Health, Seattle, WA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Virginia Mason Franciscan Health, Seattle, WA","institution_ids":[]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5052488593","display_name":"Asma Siddique","orcid":"https://orcid.org/0000-0001-8035-7912","observed_orcids":["https://orcid.org/0000-0001-8035-7912"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Asma Siddique","raw_affiliation_strings":["Virginia Mason Franciscan Health, Seattle, WA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Virginia Mason Franciscan Health, Seattle, WA","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":["https://openalex.org/A5070973168"],"corresponding_institution_ids":[],"apc_list":{"value":4390,"currency":"USD","value_usd":4390},"apc_paid":null,"fwci":null,"has_fulltext":false,"cited_by_count":1,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":"118","issue":"10S","first_page":"S2385","last_page":"S2385"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":0.7515000104904175,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":0.7515000104904175,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11412","display_name":"Genetic and Kidney Cyst Diseases","score":0.21230000257492065,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11834","display_name":"Liver Diseases and Immunity","score":0.015699999406933784,"subfield":{"id":"https://openalex.org/subfields/2721","display_name":"Hepatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/congenital-hepatic-fibrosis","display_name":"congenital hepatic fibrosis","score":0.9990000128746033},{"id":"https://openalex.org/keywords/ductal-plate-malformation","display_name":"ductal plate malformation","score":0.8379999995231628},{"id":"https://openalex.org/keywords/portal-hypertension","display_name":"portal hypertension","score":0.7379999756813049},{"id":"https://openalex.org/keywords/liver-biopsy-pathology","display_name":"liver biopsy pathology","score":0.4970000088214874},{"id":"https://openalex.org/keywords/antimitochondrial-antibodies","display_name":"antimitochondrial antibodies","score":0.7639999985694885},{"id":"https://openalex.org/keywords/cholestasis","display_name":"cholestasis","score":0.5669999718666077},{"id":"https://openalex.org/keywords/cholangitis","display_name":"cholangitis","score":0.4399999976158142},{"id":"https://openalex.org/keywords/metabolic-syndrome","display_name":"metabolic syndrome","score":0.6510000228881836},{"id":"https://openalex.org/keywords/cholangiocarcinoma","display_name":"cholangiocarcinoma","score":0.5170000195503235}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9550143480300903},{"id":"https://openalex.org/C2776366509","wikidata":"https://www.wikidata.org/wiki/Q1395683","display_name":"Congenital hepatic fibrosis","level":4,"score":0.7505511045455933},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.6378370523452759},{"id":"https://openalex.org/C2777766500","wikidata":"https://www.wikidata.org/wiki/Q360841","display_name":"Liver biopsy","level":3,"score":0.6133249998092651},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.5977810025215149},{"id":"https://openalex.org/C2778808290","wikidata":"https://www.wikidata.org/wiki/Q124604","display_name":"Portal hypertension","level":3,"score":0.5608054399490356},{"id":"https://openalex.org/C2777214474","wikidata":"https://www.wikidata.org/wiki/Q147778","display_name":"Cirrhosis","level":2,"score":0.4917011857032776},{"id":"https://openalex.org/C41260117","wikidata":"https://www.wikidata.org/wiki/Q668064","display_name":"Hepatology","level":2,"score":0.4743027687072754},{"id":"https://openalex.org/C2780788601","wikidata":"https://www.wikidata.org/wiki/Q12233655","display_name":"Intrahepatic bile ducts","level":3,"score":0.4737337827682495},{"id":"https://openalex.org/C2780559512","wikidata":"https://www.wikidata.org/wiki/Q605709","display_name":"Fibrosis","level":2,"score":0.4361388683319092},{"id":"https://openalex.org/C2775934546","wikidata":"https://www.wikidata.org/wiki/Q179991","display_name":"Biopsy","level":2,"score":0.4357840418815613},{"id":"https://openalex.org/C2779777945","wikidata":"https://www.wikidata.org/wiki/Q851111","display_name":"Bile duct","level":2,"score":0.43332916498184204},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.42795437574386597}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.14309/01.ajg.0000964364.18595.ae","doi":"https://doi.org/10.14309/01.ajg.0000964364.18595.ae","is_oa":false,"landing_page_url":"https://doi.org/10.14309/01.ajg.0000964364.18595.ae","pdf_url":null,"source":{"id":"https://openalex.org/S66441642","display_name":"The American Journal of Gastroenterology","issn_l":"0002-9270","issn":["0002-9270","1572-0241"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American Journal of Gastroenterology","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"display_name":"Good health and well-being","score":1.0,"id":"https://metadata.un.org/sdg/3"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W4390406214","https://openalex.org/W2782913475","https://openalex.org/W2409780121","https://openalex.org/W3131872055","https://openalex.org/W2914342134","https://openalex.org/W3209956066","https://openalex.org/W4387327890","https://openalex.org/W2978103344","https://openalex.org/W2352077747","https://openalex.org/W2911562632"],"abstract_inverted_index":{"Introduction:":[0],"Congenital":[1],"hepatic":[2,272],"fibrosis":[3,203],"(CHF)":[4],"is":[5,19,34,51,288,307,318,330],"a":[6,57,77,351],"rare,":[7],"autosomal":[8],"recessive,":[9],"fibropolycystic":[10],"disease":[11,27,361],"which":[12,54],"occurs":[13],"due":[14],"ductal":[15],"plate":[16],"malformation":[17],"and":[18,28,89,97,127,139,164,174,196,204,218,227,235,244,264,278,300,358],"associated":[20,298],"with":[21,66,93,142,220,238,355],"several":[22],"inherited":[23],"syndromes":[24,299],"i.e.":[25],"Caroli":[26],"polycystic":[29],"kidney":[30],"disease.":[31,281],"While":[32],"CHF":[33,256,349],"commonly":[35],"diagnosed":[36],"in":[37,42,285,295,353],"childhood,":[38],"it":[39],"may":[40,267,292,322],"present":[41],"adults":[43,354],"as":[44,46,284,320,350],"late":[45],"age":[47],"40-50.":[48],"Clinical":[49],"presentation":[50],"often":[52],"variable":[53],"can":[55],"pose":[56],"challenge":[58],"for":[59,76,87,135,194,241,246,297,311],"diagnosis.":[60,312],"Case":[61],"Description/Methods:":[62],"A":[63,212],"45-year-old":[64],"man":[65],"metabolic":[67],"syndrome":[68],"who":[69],"was":[70,104,146,150,159,192,225,229],"referred":[71],"to":[72,132,336],"the":[73,133,216,221,308,337],"Hepatology":[74],"clinic":[75],"second":[78,213],"opinion.":[79],"He":[80,158],"underwent":[81],"evaluation":[82,296],"at":[83,106,332],"an":[84,152,315],"outside":[85,189],"hospital":[86],"pruritus":[88,236],"elevated":[90],"liver":[91,190,232,327,360],"enzymes":[92,233],"ALT":[94],"56":[95],"U/L":[96],"AP":[98],"186":[99],"U/L.":[100],"Antimitochondrial":[101],"antibody":[102],"(AMA)":[103],"positive":[105],"35.6":[107],"U.":[108],"Liver":[109,305],"biopsy":[110,191,306],"noted":[111],"proliferation":[112],"of":[113,188,199,210,255,275,302,339,362],"abnormal":[114],"bile":[115,118,200],"ducts,":[116,201],"focal":[117],"duct":[119],"inflammation,":[120],"single":[121],"non-necrotizing":[122],"portal":[123,172,260,276,356],"granuloma,":[124],"mild":[125],"steatosis":[126],"focally":[128],"bridging":[129],"fibrosis.":[130],"Due":[131,335],"concern":[134],"primary":[136],"biliary":[137,182],"cirrhosis":[138,324],"possible":[140],"overlap":[141],"autoimmune":[143],"hepatitis,":[144],"ursodiol":[145,228],"initiated,":[147],"but":[148],"there":[149],"not":[151,180],"adequate":[153],"response":[154],"despite":[155],"dose":[156],"escalation.":[157],"subsequently":[160],"trialed":[161],"on":[162],"budesonide":[163],"obeticholic":[165],"acid":[166],"without":[167],"clinical":[168],"improvement.":[169],"Imaging":[170,291],"revealed":[171],"hypertension":[173,277,357],"esophageal":[175],"varices.":[176],"The":[177],"patient":[178,217],"did":[179],"have":[181,257,271],"or":[183,270,325],"renal":[184,279],"abnormalities.":[185],"Pathology":[186],"review":[187],"significant":[193],"dilation":[195],"peripheral":[197],"location":[198],"extensive":[202],"no":[205],"inflammation":[206],"\u2013":[207],"findings":[208,321],"suggestive":[209],"CHF.":[211],"hepatologist":[214],"evaluated":[215],"concurred":[219],"findings.":[222],"Repeat":[223],"AMA":[224],"negative":[226],"discontinued.":[230],"His":[231],"normalized":[234],"improved":[237],"cholestyramine.":[239],"EGD":[240],"variceal":[242],"screening":[243],"MRCP":[245],"cholangiocarcinoma":[247],"(CCA)":[248],"surveillance":[249,342],"were":[250],"scheduled.":[251],"Discussion:":[252],"Four":[253],"forms":[254],"been":[258],"described:":[259],"hypertension,":[261],"cholangitic,":[262],"mixed":[263],"latent.":[265],"Patients":[266],"be":[268,293,344],"asymptomatic":[269],"fibrosis,":[273],"sequelae":[274],"cystic":[280],"Isolated":[282],"CHF,":[283],"our":[286],"case,":[287],"extremely":[289],"uncommon.":[290],"helpful":[294],"exclusion":[301],"other":[303,326],"etiologies.":[304],"gold":[309],"standard":[310],"Review":[313],"by":[314],"experienced":[316],"pathologist":[317],"important":[319],"mimic":[323],"conditions.":[328],"Treatment":[329],"directed":[331],"managing":[333],"complications.":[334],"risk":[338],"developing":[340],"CCA,":[341],"should":[343,347],"performed.":[345],"Clinicians":[346],"consider":[348],"differential":[352],"chronic":[359],"unclear":[363],"etiology.":[364]},"counts_by_year":[{"year":2025,"cited_by_count":1}],"updated_date":"2026-08-21T09:56:20.448147","created_date":"2023-10-19T00:00:00"}
