{"id":"https://openalex.org/W76649006","doi":"https://doi.org/10.1093/joneph/19.4.529","title":"Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis","display_name":"Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis","publication_year":2006,"publication_date":"2006-07-01","ids":{"openalex":"https://openalex.org/W76649006","doi":"https://doi.org/10.1093/joneph/19.4.529","mag":"76649006","pmid":"https://pubmed.ncbi.nlm.nih.gov/17048214"},"language":"en","primary_location":{"id":"doi:10.1093/joneph/19.4.529","doi":"https://doi.org/10.1093/joneph/19.4.529","is_oa":false,"landing_page_url":"https://doi.org/10.1093/joneph/19.4.529","pdf_url":null,"source":{"id":"https://openalex.org/S201014839","display_name":"Journal of Nephrology","issn_l":"1121-8428","issn":["1121-8428","1724-6059"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-c","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310319900","host_organization_name":"Springer Science+Business Media","host_organization_lineage":["https://openalex.org/P4310319900","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Springer Science+Business Media","Springer Nature"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Nephrology","raw_type":"journal-article"},"volume":null,"type":"article","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/A5057284288","display_name":"Nancy N Xu","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Nancy Xu","raw_affiliation_strings":["Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","institution_ids":["https://openalex.org/I1330342723"]},{"raw_affiliation_string":"Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111411359","display_name":"James F. Glockner","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"James F. Glockner","raw_affiliation_strings":["Department of Diagnostic Radiology, Mayo Clinic College of Medicine, Rochester, MN - USA 2"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Diagnostic Radiology, Mayo Clinic College of Medicine, Rochester, MN - USA 2","institution_ids":["https://openalex.org/I1330342723"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5000198802","display_name":"Sandro Rossetti","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sandro Rossetti","raw_affiliation_strings":["Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","Mayo College of Medicine"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","institution_ids":["https://openalex.org/I1330342723"]},{"raw_affiliation_string":"Mayo College of Medicine","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5069559198","display_name":"Dusica Babovic\u2010Vuksanovic","orcid":"https://orcid.org/0000-0001-6981-1352","observed_orcids":["https://orcid.org/0000-0001-6981-1352"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Dusica Babovic-Vuksanovic","raw_affiliation_strings":["Department of Medical Genetics, Mayo Clinic College of Medicine, Rochester, MN - USA 3","Mayo College of Medicine"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medical Genetics, Mayo Clinic College of Medicine, Rochester, MN - USA 3","institution_ids":["https://openalex.org/I1330342723"]},{"raw_affiliation_string":"Mayo College of Medicine","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5002238355","display_name":"Peter C. Harris","orcid":"https://orcid.org/0000-0002-5304-6593","observed_orcids":["https://orcid.org/0000-0002-5304-6593"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Peter C. Harris","raw_affiliation_strings":["Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","Nephrology and Hypertension"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","institution_ids":["https://openalex.org/I1330342723"]},{"raw_affiliation_string":"Nephrology and Hypertension","institution_ids":[]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5070149489","display_name":"Vicente E. Torres","orcid":"https://orcid.org/0000-0003-2008-1576","observed_orcids":["https://orcid.org/0000-0003-2008-1576"]},"institutions":[{"id":"https://openalex.org/I1330342723","display_name":"Mayo Clinic","ror":"https://ror.org/02qp3tb03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1330342723"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Vicente E. Torres","raw_affiliation_strings":["Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","Nephrology and Hypertension"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Nephrology, Mayo Clinic College of Medicine, Rochester, MN - USA 1","institution_ids":["https://openalex.org/I1330342723"]},{"raw_affiliation_string":"Nephrology and Hypertension","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5070149489"],"corresponding_institution_ids":["https://openalex.org/I1330342723"],"apc_list":{"value":4337,"currency":"USD","value_usd":4337},"apc_paid":null,"fwci":2.6517,"has_fulltext":false,"cited_by_count":59,"citation_normalized_percentile":{"value":0.89190745,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":98},"biblio":{"volume":"19","issue":"4","first_page":"529","last_page":"534"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11412","display_name":"Genetic and Kidney Cyst Diseases","score":0.9965999722480774,"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"}},"topics":[{"id":"https://openalex.org/T11412","display_name":"Genetic and Kidney Cyst Diseases","score":0.9965999722480774,"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/T10665","display_name":"Cystic Fibrosis Research Advances","score":0.0032999999821186066,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"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/T12049","display_name":"Tuberous Sclerosis Complex Research","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology"},"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/autosomal-dominant-polycystic-kidney-disease","display_name":"autosomal dominant polycystic kidney disease","score":1.0},{"id":"https://openalex.org/keywords/cystic-fibrosis","display_name":"cystic fibrosis","score":0.9990000128746033},{"id":"https://openalex.org/keywords/cftr","display_name":"CFTR","score":0.8230000138282776},{"id":"https://openalex.org/keywords/cftr-mutations","display_name":"CFTR mutations","score":0.5479999780654907},{"id":"https://openalex.org/keywords/f508del-mutation","display_name":"F508del mutation","score":0.7919999957084656},{"id":"https://openalex.org/keywords/renal-cysts","display_name":"renal cysts","score":0.9129999876022339},{"id":"https://openalex.org/keywords/liver-cysts","display_name":"liver cysts","score":0.9860000014305115},{"id":"https://openalex.org/keywords/kidney-volume","display_name":"kidney volume","score":0.7279999852180481}],"concepts":[{"id":"https://openalex.org/C2776266639","wikidata":"https://www.wikidata.org/wiki/Q2732398","display_name":"Autosomal dominant polycystic kidney disease","level":3,"score":0.9231572151184082},{"id":"https://openalex.org/C100773827","wikidata":"https://www.wikidata.org/wiki/Q82302467","display_name":"PKD1","level":4,"score":0.8490724563598633},{"id":"https://openalex.org/C2776938444","wikidata":"https://www.wikidata.org/wiki/Q178194","display_name":"Cystic fibrosis","level":2,"score":0.8214601874351501},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8053343892097473},{"id":"https://openalex.org/C2778428886","wikidata":"https://www.wikidata.org/wiki/Q420470","display_name":"Cystic fibrosis transmembrane conductance regulator","level":3,"score":0.6214237809181213},{"id":"https://openalex.org/C2780091579","wikidata":"https://www.wikidata.org/wiki/Q9377","display_name":"Kidney","level":2,"score":0.5172434449195862},{"id":"https://openalex.org/C127716648","wikidata":"https://www.wikidata.org/wiki/Q104053","display_name":"Phenotype","level":3,"score":0.49308082461357117},{"id":"https://openalex.org/C2780145431","wikidata":"https://www.wikidata.org/wiki/Q15443105","display_name":"Polycystic kidney disease","level":3,"score":0.48467329144477844},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.48424121737480164},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.4769526720046997},{"id":"https://openalex.org/C2780597408","wikidata":"https://www.wikidata.org/wiki/Q193211","display_name":"Cyst","level":2,"score":0.4650019407272339},{"id":"https://openalex.org/C501734568","wikidata":"https://www.wikidata.org/wiki/Q42918","display_name":"Mutation","level":3,"score":0.4443763792514801},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.429006963968277},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.14375361800193787},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.11213237047195435},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.1044570803642273}],"mesh":[{"descriptor_ui":"D003550","descriptor_name":"Cystic Fibrosis","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":true},{"descriptor_ui":"D003550","descriptor_name":"Cystic Fibrosis","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","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":"D008279","descriptor_name":"Magnetic Resonance Imaging","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008875","descriptor_name":"Middle Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009154","descriptor_name":"Mutation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D016891","descriptor_name":"Polycystic Kidney, Autosomal Dominant","qualifier_ui":"Q000150","qualifier_name":"complications","is_major_topic":true},{"descriptor_ui":"D016891","descriptor_name":"Polycystic Kidney, Autosomal Dominant","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D019005","descriptor_name":"Cystic Fibrosis Transmembrane Conductance Regulator","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false}],"locations_count":3,"locations":[{"id":"doi:10.1093/joneph/19.4.529","doi":"https://doi.org/10.1093/joneph/19.4.529","is_oa":false,"landing_page_url":"https://doi.org/10.1093/joneph/19.4.529","pdf_url":null,"source":{"id":"https://openalex.org/S201014839","display_name":"Journal of Nephrology","issn_l":"1121-8428","issn":["1121-8428","1724-6059"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-c","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310319900","host_organization_name":"Springer Science+Business Media","host_organization_lineage":["https://openalex.org/P4310319900","https://openalex.org/P4310319965"],"host_organization_lineage_names":["Springer Science+Business Media","Springer Nature"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Nephrology","raw_type":"journal-article"},{"id":"pmid:17048214","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/17048214","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 nephrology","raw_type":"Case Reports"},{"id":"mag:76649006","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/17048214","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":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.98580002784729,"display_name":"Good health and well-being"}],"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/W2087639140","https://openalex.org/W2132434664","https://openalex.org/W2003666013","https://openalex.org/W1711736066","https://openalex.org/W2466551845","https://openalex.org/W3011885759","https://openalex.org/W1964743454","https://openalex.org/W1977202492","https://openalex.org/W2026415637","https://openalex.org/W7128297643"],"abstract_inverted_index":{"Autosomal":[0],"dominant":[1],"polycystic":[2],"kidney":[3,107],"disease":[4],"(ADPKD)":[5],"is":[6,44],"a":[7,73,123],"common,":[8],"inherited":[9],"disorder":[10],"characterized":[11],"by":[12],"the":[13,20,25,35,41,110],"progressive":[14],"enlargement":[15],"of":[16,37,79,114,125],"fluid-filled":[17],"cysts":[18,118],"in":[19,51,122,139],"kidneys":[21],"and":[22,104,109,112,116,133],"liver.":[23],"Since":[24],"cystic":[26,48,69,75],"fibrosis":[27,49,70],"transmembrane":[28],"conductance":[29],"regulator":[30],"(CFTR)":[31],"Cl--channel":[32],"may":[33],"mediate":[34],"secretion":[36],"Cl--and":[38],"fluid":[39],"into":[40],"cysts,":[42],"it":[43],"conceivable":[45],"that":[46,62,78],"coexisting":[47,67,100],"(CF)":[50,71],"patients":[52,64],"with":[53,65,68,99,128,142],"ADPKD":[54,66,103,129,143],"could":[55],"attenuate":[56],"their":[57],"development.":[58],"We":[59,93],"previously":[60],"reported":[61],"two":[63],"had":[72],"milder":[74],"phenotype":[76],"than":[77,138],"kindred":[80],"without":[81],"CFTR":[82],"mutations.":[83],"A":[84],"subsequent":[85],"report":[86],"failed":[87],"to":[88],"confirm":[89],"this":[90,126],"protective":[91],"effect.":[92],"now":[94],"have":[95],"identified":[96],"another":[97],"family":[98,127],"type":[101],"1":[102],"CF.":[105],"The":[106],"volumes":[108],"number":[111],"size":[113],"renal":[115],"hepatic":[117],"were":[119],"markedly":[120],"less":[121],"member":[124],"(PKD1":[130],"mutation":[131],"C508R)":[132],"CF":[134],"(homozygous":[135],"DeltaF508":[136],"mutation)":[137],"her":[140],"sister":[141],"alone":[144],"at":[145],"comparable":[146],"ages.":[147]},"counts_by_year":[{"year":2026,"cited_by_count":2},{"year":2025,"cited_by_count":1},{"year":2023,"cited_by_count":5},{"year":2022,"cited_by_count":2},{"year":2021,"cited_by_count":3},{"year":2020,"cited_by_count":3},{"year":2018,"cited_by_count":2},{"year":2017,"cited_by_count":2},{"year":2016,"cited_by_count":1},{"year":2015,"cited_by_count":1},{"year":2014,"cited_by_count":4},{"year":2013,"cited_by_count":4},{"year":2012,"cited_by_count":5}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2016-06-24T00:00:00"}