{"id":"https://openalex.org/W2319609136","doi":"https://doi.org/10.1002/j.1536-4801.1997.tb00649.x","title":"Progressive Familial Intrahepatic Cholestasis Among the Arab Population in Israel","display_name":"Progressive Familial Intrahepatic Cholestasis Among the Arab Population in Israel","publication_year":1997,"publication_date":"1997-05-01","ids":{"openalex":"https://openalex.org/W2319609136","doi":"https://doi.org/10.1002/j.1536-4801.1997.tb00649.x","mag":"2319609136","pmid":"https://pubmed.ncbi.nlm.nih.gov/9161950"},"language":"en","primary_location":{"id":"doi:10.1002/j.1536-4801.1997.tb00649.x","doi":"https://doi.org/10.1002/j.1536-4801.1997.tb00649.x","is_oa":false,"landing_page_url":"https://doi.org/10.1002/j.1536-4801.1997.tb00649.x","pdf_url":null,"source":{"id":"https://openalex.org/S197964821","display_name":"Journal of Pediatric Gastroenterology and Nutrition","issn_l":"0277-2116","issn":["0277-2116","1536-4801"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-100","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":"Journal of Pediatric Gastroenterology and Nutrition","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","pubmed"],"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/A5025934483","display_name":"Yehezkel Naveh","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I174306211","display_name":"Technion \u2013 Israel Institute of Technology","ror":"https://ror.org/03qryx823","country_code":"IL","type":"education","lineage":["https://openalex.org/I174306211"]},{"id":"https://openalex.org/I2801301638","display_name":"Rambam Health Care Campus","ror":"https://ror.org/01fm87m50","country_code":"IL","type":"healthcare","lineage":["https://openalex.org/I2801301638"]}],"countries":["IL"],"is_corresponding":true,"raw_author_name":"Yehezkel Naveh","raw_affiliation_strings":["Department of Pediatrics Haifa Israel","The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel","Department of Pediatrics, Rambam Medical Center, Haifa, Israel"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics Haifa Israel","institution_ids":[]},{"raw_affiliation_string":"The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel","institution_ids":["https://openalex.org/I174306211"]},{"raw_affiliation_string":"Department of Pediatrics, Rambam Medical Center, Haifa, Israel","institution_ids":["https://openalex.org/I2801301638"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111642841","display_name":"Lucyna Bassan","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2801301638","display_name":"Rambam Health Care Campus","ror":"https://ror.org/01fm87m50","country_code":"IL","type":"healthcare","lineage":["https://openalex.org/I2801301638"]}],"countries":["IL"],"is_corresponding":false,"raw_author_name":"Lucyna Bassan","raw_affiliation_strings":["Department of Pathology Rambam Medical Center Haifa Israel","Pathology, Rambam Medical Center,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology Rambam Medical Center Haifa Israel","institution_ids":["https://openalex.org/I2801301638"]},{"raw_affiliation_string":"Pathology, Rambam Medical Center,","institution_ids":["https://openalex.org/I2801301638"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5110182673","display_name":"Eldad Rosenthal","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Eldad Rosenthal","raw_affiliation_strings":["Department of Pediatrics Haifa Israel","\"Pediatrics and \u2020Pathology"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics Haifa Israel","institution_ids":[]},{"raw_affiliation_string":"\"Pediatrics and \u2020Pathology","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5034328678","display_name":"Drora Berkowitz","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I174306211","display_name":"Technion \u2013 Israel Institute of Technology","ror":"https://ror.org/03qryx823","country_code":"IL","type":"education","lineage":["https://openalex.org/I174306211"]}],"countries":["IL"],"is_corresponding":false,"raw_author_name":"Drora Berkowitz","raw_affiliation_strings":["Department of Pediatrics Haifa Israel","The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics Haifa Israel","institution_ids":[]},{"raw_affiliation_string":"The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel","institution_ids":["https://openalex.org/I174306211"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5079937075","display_name":"Michael Jaffe","orcid":"https://orcid.org/0000-0002-5089-5039","observed_orcids":["https://orcid.org/0000-0002-5089-5039"]},"institutions":[{"id":"https://openalex.org/I174306211","display_name":"Technion \u2013 Israel Institute of Technology","ror":"https://ror.org/03qryx823","country_code":"IL","type":"education","lineage":["https://openalex.org/I174306211"]},{"id":"https://openalex.org/I4210113309","display_name":"Bnai Zion Medical Center","ror":"https://ror.org/01yvj7247","country_code":"IL","type":"healthcare","lineage":["https://openalex.org/I4210113309"]}],"countries":["IL"],"is_corresponding":false,"raw_author_name":"Michael Jaffe","raw_affiliation_strings":["Department of Pediatrics Bnai Zion Medical Center Haifa Israel","The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics Bnai Zion Medical Center Haifa Israel","institution_ids":["https://openalex.org/I4210113309"]},{"raw_affiliation_string":"The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel","institution_ids":["https://openalex.org/I174306211"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5102999526","display_name":"Hanna Mandel","orcid":"https://orcid.org/0000-0002-5230-4247","observed_orcids":["https://orcid.org/0000-0002-5230-4247"]},"institutions":[{"id":"https://openalex.org/I174306211","display_name":"Technion \u2013 Israel Institute of Technology","ror":"https://ror.org/03qryx823","country_code":"IL","type":"education","lineage":["https://openalex.org/I174306211"]}],"countries":["IL"],"is_corresponding":false,"raw_author_name":"Hanna Mandel","raw_affiliation_strings":["Department of Pediatrics Haifa Israel","The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics Haifa Israel","institution_ids":[]},{"raw_affiliation_string":"The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel","institution_ids":["https://openalex.org/I174306211"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5089288712","display_name":"Moshe Berant","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I174306211","display_name":"Technion \u2013 Israel Institute of Technology","ror":"https://ror.org/03qryx823","country_code":"IL","type":"education","lineage":["https://openalex.org/I174306211"]}],"countries":["IL"],"is_corresponding":false,"raw_author_name":"Moshe Berant","raw_affiliation_strings":["Department of Pediatrics Haifa Israel","The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics Haifa Israel","institution_ids":[]},{"raw_affiliation_string":"The Bruce Rappaport Faculty of Medicine Technion\u2010Israel Institute of Technology Haifa Israel","institution_ids":["https://openalex.org/I174306211"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":3,"corresponding_author_ids":["https://openalex.org/A5025934483"],"corresponding_institution_ids":["https://openalex.org/I174306211","https://openalex.org/I2801301638"],"apc_list":{"value":3780,"currency":"USD","value_usd":3780},"apc_paid":null,"fwci":3.5991,"has_fulltext":false,"cited_by_count":28,"citation_normalized_percentile":{"value":0.91776316,"is_in_top_1_percent":false,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":88,"max":96},"biblio":{"volume":"24","issue":"5","first_page":"548","last_page":"554"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":0.9937000274658203,"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":0.9937000274658203,"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/T11834","display_name":"Liver Diseases and Immunity","score":0.00139999995008111,"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"}},{"id":"https://openalex.org/T10570","display_name":"Drug Transport and Resistance Mechanisms","score":0.0012000000569969416,"subfield":{"id":"https://openalex.org/subfields/2730","display_name":"Oncology"},"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/progressive-familial-intrahepatic-cholestasis","display_name":"progressive familial intrahepatic cholestasis","score":1.0},{"id":"https://openalex.org/keywords/consanguinity","display_name":"consanguinity","score":0.8820000290870667},{"id":"https://openalex.org/keywords/autosomal-recessive-inheritance","display_name":"autosomal recessive inheritance","score":0.8989999890327454},{"id":"https://openalex.org/keywords/cholestatic-jaundice","display_name":"cholestatic jaundice","score":0.7580000162124634},{"id":"https://openalex.org/keywords/infantile-cholestasis","display_name":"infantile cholestasis","score":0.640999972820282},{"id":"https://openalex.org/keywords/liver-cirrhosis","display_name":"liver cirrhosis","score":0.9359999895095825},{"id":"https://openalex.org/keywords/liver-transplantation","display_name":"liver transplantation","score":0.8069999814033508},{"id":"https://openalex.org/keywords/phenobarbital","display_name":"phenobarbital","score":0.7110000252723694},{"id":"https://openalex.org/keywords/cholestyramine","display_name":"cholestyramine","score":0.9829999804496765},{"id":"https://openalex.org/keywords/ursodeoxycholic-acid","display_name":"ursodeoxycholic acid","score":0.9950000047683716},{"id":"https://openalex.org/keywords/liver-histopathology","display_name":"liver histopathology","score":0.6330000162124634},{"id":"https://openalex.org/keywords/arabs","display_name":"Arabs","score":0.4090000092983246},{"id":"https://openalex.org/keywords/differential-diagnosis","display_name":"differential diagnosis","score":0.7699999809265137}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9523625373840332},{"id":"https://openalex.org/C2778132995","wikidata":"https://www.wikidata.org/wiki/Q1018534","display_name":"Progressive familial intrahepatic cholestasis","level":4,"score":0.844446063041687},{"id":"https://openalex.org/C2778593092","wikidata":"https://www.wikidata.org/wiki/Q1075923","display_name":"Cholestasis","level":2,"score":0.783403754234314},{"id":"https://openalex.org/C2908647359","wikidata":"https://www.wikidata.org/wiki/Q2625603","display_name":"Population","level":2,"score":0.5026123523712158},{"id":"https://openalex.org/C187212893","wikidata":"https://www.wikidata.org/wiki/Q123028","display_name":"Pediatrics","level":1,"score":0.3722743093967438},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.34113609790802},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3358054757118225},{"id":"https://openalex.org/C99454951","wikidata":"https://www.wikidata.org/wiki/Q932068","display_name":"Environmental health","level":1,"score":0.1835150420665741},{"id":"https://openalex.org/C2779609443","wikidata":"https://www.wikidata.org/wiki/Q1368191","display_name":"Liver transplantation","level":3,"score":0.054452598094940186},{"id":"https://openalex.org/C2911091166","wikidata":"https://www.wikidata.org/wiki/Q106419912","display_name":"Transplantation","level":2,"score":0.0}],"mesh":[{"descriptor_ui":"D001707","descriptor_name":"Biopsy, Needle","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002779","descriptor_name":"Cholestasis","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":false},{"descriptor_ui":"D002780","descriptor_name":"Cholestasis, Intrahepatic","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D002780","descriptor_name":"Cholestasis, Intrahepatic","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D002780","descriptor_name":"Cholestasis, Intrahepatic","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":false},{"descriptor_ui":"D003241","descriptor_name":"Consanguinity","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D003937","descriptor_name":"Diagnosis, Differential","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005190","descriptor_name":"Family","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005500","descriptor_name":"Follow-Up Studies","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007223","descriptor_name":"Infant","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007231","descriptor_name":"Infant, Newborn","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007557","descriptor_name":"Israel","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008099","descriptor_name":"Liver","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D008099","descriptor_name":"Liver","qualifier_ui":"Q000648","qualifier_name":"ultrastructure","is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D010375","descriptor_name":"Pedigree","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D018912","descriptor_name":"Arabs","qualifier_ui":"Q000706","qualifier_name":"statistics & numerical data","is_major_topic":true}],"locations_count":3,"locations":[{"id":"doi:10.1002/j.1536-4801.1997.tb00649.x","doi":"https://doi.org/10.1002/j.1536-4801.1997.tb00649.x","is_oa":false,"landing_page_url":"https://doi.org/10.1002/j.1536-4801.1997.tb00649.x","pdf_url":null,"source":{"id":"https://openalex.org/S197964821","display_name":"Journal of Pediatric Gastroenterology and Nutrition","issn_l":"0277-2116","issn":["0277-2116","1536-4801"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-100","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":"Journal of Pediatric Gastroenterology and Nutrition","raw_type":"journal-article"},{"id":"doi:10.1097/00005176-199705000-00011","doi":"https://doi.org/10.1097/00005176-199705000-00011","is_oa":false,"landing_page_url":"https://doi.org/10.1097/00005176-199705000-00011","pdf_url":null,"source":{"id":"https://openalex.org/S197964821","display_name":"Journal of Pediatric Gastroenterology and Nutrition","issn_l":"0277-2116","issn":["0277-2116","1536-4801"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-100","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":"Journal of Pediatric Gastroenterology & Nutrition","raw_type":"journal-article"},{"id":"pmid:9161950","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/9161950","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of pediatric gastroenterology and nutrition","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":30,"referenced_works":["https://openalex.org/W1540621575","https://openalex.org/W1600735463","https://openalex.org/W1612407641","https://openalex.org/W1658261516","https://openalex.org/W1827984248","https://openalex.org/W1910305776","https://openalex.org/W1974585233","https://openalex.org/W1976450060","https://openalex.org/W1983329155","https://openalex.org/W1985460941","https://openalex.org/W1989815293","https://openalex.org/W2006137579","https://openalex.org/W2017126803","https://openalex.org/W2029709648","https://openalex.org/W2038307560","https://openalex.org/W2055630306","https://openalex.org/W2064368190","https://openalex.org/W2166050745","https://openalex.org/W2313737116","https://openalex.org/W2322281393","https://openalex.org/W2324057214","https://openalex.org/W2328636427","https://openalex.org/W2328987984","https://openalex.org/W2400060321","https://openalex.org/W2400440165","https://openalex.org/W2412177685","https://openalex.org/W2414705276","https://openalex.org/W2527767871","https://openalex.org/W4206145348","https://openalex.org/W4294443831"],"related_works":["https://openalex.org/W162113137","https://openalex.org/W4401545343","https://openalex.org/W1540621575","https://openalex.org/W1971925193","https://openalex.org/W3215206501","https://openalex.org/W2025504756","https://openalex.org/W2020815206","https://openalex.org/W2201910200","https://openalex.org/W4403652029","https://openalex.org/W6959987180"],"abstract_inverted_index":{"BACKGROUND:":[0],"Progressive":[1,220],"familial":[2,221],"intrahepatic":[3,158,222],"cholestasis,":[4,161],"which":[5],"constitutes":[6],"a":[7],"heterogeneous":[8],"group":[9],"of":[10,35,39,73,97,126,146,150,157,167,191,231,236],"imperfectly":[11],"delineated":[12],"syndromes":[13],"and":[14,84,102,132,144,213],"appears":[15],"to":[16,62,197,242],"be":[17,225],"inherited":[18],"as":[19],"an":[20],"autosomal":[21],"recessive":[22],"condition,":[23],"has":[24],"not":[25],"been":[26],"hitherto":[27],"reported":[28],"from":[29,49,195],"the":[30,36,69,93,142,151,177,192,204,228],"Middle":[31],"East,":[32],"in":[33,42,75,112,116,123,141,200,203,227],"spite":[34],"high":[37],"rate":[38],"consanguineous":[40,63,243],"marriage":[41],"this":[43],"region.":[44],"METHODS:":[45],"Sixteen":[46],"affected":[47,174],"children":[48,209],"six":[50],"Israeli":[51],"Arab":[52,244],"families":[53],"were":[54,60,105,110,139,188],"evaluated":[55],"over":[56],"30":[57],"years.":[58,134],"All":[59],"born":[61,241],"parents.":[64,245],"RESULTS:":[65],"Jaundice":[66],"appeared":[67],"during":[68],"first":[70,79],"3":[71,133],"weeks":[72],"life":[74],"15":[76],"babies.":[77],"When":[78],"referred,":[80],"10":[81],"had":[82,86],"hepatomegaly":[83],"nine":[85],"splenomegaly.":[87],"A":[88],"progression":[89],"toward":[90],"cirrhosis":[91],"was":[92,119,194],"rule.":[94],"Serum":[95],"levels":[96,109],"conjugated":[98],"bilirubin,":[99],"liver":[100,152,168,211,217],"enzymes,":[101],"alkaline":[103],"phosphatase":[104],"raised;":[106],"gamma-glutamyl":[107],"transpeptidase":[108],"normal":[111],"all":[113],"three":[114,208],"infants":[115,232],"whom":[117],"it":[118],"examined,":[120],"but":[121],"elevated":[122],"two":[124,206],"siblings":[125],"another":[127],"family":[128],"at":[129,171],"ages":[130],"2":[131],"No":[135],"abnormal":[136],"bile":[137,159],"acids":[138],"detected":[140],"serum":[143],"urine":[145],"patients.":[147],"Histologic":[148],"examination":[149],"showed":[153],"giant-cell":[154],"transformation,":[155],"paucity":[156],"ducts,":[160],"fibrosis,":[162],"or":[163,185],"cirrhosis.":[164],"The":[165],"pattern":[166],"pathology":[169],"differed":[170],"times":[172],"among":[173],"members":[175],"within":[176],"same":[178],"family.":[179],"Therapeutic":[180],"trials":[181],"with":[182,233],"phenobarbital,":[183],"cholestyramine,":[184],"ursodeoxycholic":[186],"acid":[187],"ineffective.":[189],"Survival":[190],"patients":[193],"5":[196],"18":[198],"months":[199],"four":[201],"families;":[202],"other":[205],"families,":[207],"received":[210],"transplants,":[212],"one":[214],"is":[215],"awaiting":[216],"transplantation.":[218],"CONCLUSIONS:":[219],"cholestasis":[223],"should":[224],"included":[226],"differential":[229],"diagnosis":[230],"cholestatic":[234],"jaundice":[235],"unknown":[237],"etiology,":[238],"especially":[239],"those":[240]},"counts_by_year":[{"year":2023,"cited_by_count":1},{"year":2018,"cited_by_count":2},{"year":2017,"cited_by_count":2},{"year":2013,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}