{"id":"https://openalex.org/W2806792719","doi":"https://doi.org/10.1080/17476348.2018.1483723","title":"Unmet needs in cystic fibrosis: the next steps in improving outcomes","display_name":"Unmet needs in cystic fibrosis: the next steps in improving outcomes","publication_year":2018,"publication_date":"2018-06-01","ids":{"openalex":"https://openalex.org/W2806792719","doi":"https://doi.org/10.1080/17476348.2018.1483723","mag":"2806792719","pmid":"https://pubmed.ncbi.nlm.nih.gov/29855230","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/6300137"},"language":"en","primary_location":{"id":"doi:10.1080/17476348.2018.1483723","doi":"https://doi.org/10.1080/17476348.2018.1483723","is_oa":false,"landing_page_url":"https://doi.org/10.1080/17476348.2018.1483723","pdf_url":null,"source":{"id":"https://openalex.org/S121778365","display_name":"Expert Review of Respiratory Medicine","issn_l":"1747-6348","issn":["1747-6348","1747-6356"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320547","host_organization_name":"Taylor & Francis","host_organization_lineage":["https://openalex.org/P4310320547","https://openalex.org/P4310320449"],"host_organization_lineage_names":["Taylor & Francis","Informa"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Expert Review of Respiratory Medicine","raw_type":"journal-article"},"volume":null,"type":"review","indexed_in":["crossref","pubmed"],"open_access":{"is_oa":true,"oa_status":"green","oa_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/6300137","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5004213410","display_name":"Natalie E. West","orcid":"https://orcid.org/0000-0002-0025-2422","observed_orcids":["https://orcid.org/0000-0002-0025-2422"]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Natalie E. West","raw_affiliation_strings":["Department of Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, USA","institution_ids":["https://openalex.org/I145311948"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5005915390","display_name":"Patrick A. Flume","orcid":"https://orcid.org/0000-0002-1560-6879","observed_orcids":["https://orcid.org/0000-0002-1560-6879"]},"institutions":[{"id":"https://openalex.org/I153297377","display_name":"Medical University of South Carolina","ror":"https://ror.org/012jban78","country_code":"US","type":"education","lineage":["https://openalex.org/I153297377"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Patrick A. Flume","raw_affiliation_strings":["Departments of Medicine and Pediatrics, Medical University of South Carolina, Charleston, SC, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Departments of Medicine and Pediatrics, Medical University of South Carolina, Charleston, SC, USA","institution_ids":["https://openalex.org/I153297377"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5005915390"],"corresponding_institution_ids":["https://openalex.org/I153297377"],"apc_list":null,"apc_paid":null,"fwci":0.6657,"has_fulltext":false,"cited_by_count":19,"citation_normalized_percentile":{"value":0.56695345,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":98},"biblio":{"volume":"12","issue":"7","first_page":"585","last_page":"593"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10665","display_name":"Cystic Fibrosis Research Advances","score":1.0,"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"}},"topics":[{"id":"https://openalex.org/T10665","display_name":"Cystic Fibrosis Research Advances","score":1.0,"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/T10143","display_name":"Chronic Obstructive Pulmonary Disease (COPD) Research","score":0.0,"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/T11651","display_name":"Inhalation and Respiratory Drug Delivery","score":0.0,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/cystic-fibrosis","display_name":"cystic fibrosis","score":1.0},{"id":"https://openalex.org/keywords/unmet-medical-needs","display_name":"unmet medical needs","score":0.9440000057220459},{"id":"https://openalex.org/keywords/cftr-modulators","display_name":"CFTR modulators","score":0.9919999837875366},{"id":"https://openalex.org/keywords/end-stage-lung-disease","display_name":"end-stage lung disease","score":0.847000002861023},{"id":"https://openalex.org/keywords/pulmonary-exacerbations","display_name":"pulmonary exacerbations","score":0.9639999866485596},{"id":"https://openalex.org/keywords/anti-inflammatory-therapy","display_name":"anti-inflammatory therapy","score":0.7350000143051147},{"id":"https://openalex.org/keywords/anti-infective-therapy","display_name":"anti-infective therapy","score":0.9139999747276306},{"id":"https://openalex.org/keywords/lung-transplantation","display_name":"lung transplantation","score":0.9110000133514404},{"id":"https://openalex.org/keywords/extrapulmonary-complications","display_name":"extrapulmonary complications","score":0.9409999847412109},{"id":"https://openalex.org/keywords/pulmonary-therapy","display_name":"pulmonary therapy","score":0.30300000309944153}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9247885942459106},{"id":"https://openalex.org/C2776938444","wikidata":"https://www.wikidata.org/wiki/Q178194","display_name":"Cystic fibrosis","level":2,"score":0.7751812934875488},{"id":"https://openalex.org/C177713679","wikidata":"https://www.wikidata.org/wiki/Q679690","display_name":"Intensive care medicine","level":1,"score":0.5615550875663757},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.2897791862487793}],"mesh":[{"descriptor_ui":"D001987","descriptor_name":"Bronchiectasis","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":false},{"descriptor_ui":"D003550","descriptor_name":"Cystic Fibrosis","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007249","descriptor_name":"Inflammation","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":false},{"descriptor_ui":"D012141","descriptor_name":"Respiratory Tract Infections","qualifier_ui":"Q000517","qualifier_name":"prevention & control","is_major_topic":false},{"descriptor_ui":"D016040","descriptor_name":"Lung Transplantation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D020380","descriptor_name":"Needs Assessment","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D063868","descriptor_name":"Patient Outcome Assessment","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":4,"locations":[{"id":"doi:10.1080/17476348.2018.1483723","doi":"https://doi.org/10.1080/17476348.2018.1483723","is_oa":false,"landing_page_url":"https://doi.org/10.1080/17476348.2018.1483723","pdf_url":null,"source":{"id":"https://openalex.org/S121778365","display_name":"Expert Review of Respiratory Medicine","issn_l":"1747-6348","issn":["1747-6348","1747-6356"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-70","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310320547","host_organization_name":"Taylor & Francis","host_organization_lineage":["https://openalex.org/P4310320547","https://openalex.org/P4310320449"],"host_organization_lineage_names":["Taylor & Francis","Informa"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Expert Review of Respiratory Medicine","raw_type":"journal-article"},{"id":"pmid:29855230","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/29855230","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":"Expert review of respiratory medicine","raw_type":"Journal Article"},{"id":"pmh:oai:europepmc.org:5569605","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/6300137","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":null,"raw_type":"Text"},{"id":"pmh:oai:pubmedcentral.nih.gov:6300137","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/6300137","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"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":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":"Expert Rev Respir Med","raw_type":"Text"}],"best_oa_location":{"id":"pmh:oai:europepmc.org:5569605","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/6300137","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":null,"raw_type":"Text"},"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":1.0}],"study_designs":[],"awards":[{"id":"https://openalex.org/G8277385850","display_name":"South Carolina Clinical & Translational Research Institute (SCTR)","funder_award_id":"UL1TR001450","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"}],"funders":[{"id":"https://openalex.org/F4320332161","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88"},{"id":"https://openalex.org/F4320337472","display_name":"National Center for Advancing Translational Sciences","ror":"https://ror.org/04pw6fb54"}],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":87,"referenced_works":["https://openalex.org/W166245712","https://openalex.org/W1589654967","https://openalex.org/W1715680901","https://openalex.org/W1920268374","https://openalex.org/W1971512067","https://openalex.org/W1971558827","https://openalex.org/W1986316308","https://openalex.org/W1994703990","https://openalex.org/W1994733853","https://openalex.org/W1995117300","https://openalex.org/W2003493994","https://openalex.org/W2009467539","https://openalex.org/W2010756316","https://openalex.org/W2011293971","https://openalex.org/W2048091641","https://openalex.org/W2053850651","https://openalex.org/W2060894544","https://openalex.org/W2063756601","https://openalex.org/W2066510676","https://openalex.org/W2070450626","https://openalex.org/W2071034664","https://openalex.org/W2074235114","https://openalex.org/W2075069205","https://openalex.org/W2076762373","https://openalex.org/W2078078890","https://openalex.org/W2087127819","https://openalex.org/W2092027840","https://openalex.org/W2098507554","https://openalex.org/W2102261662","https://openalex.org/W2102496493","https://openalex.org/W2103043501","https://openalex.org/W2105534700","https://openalex.org/W2110828250","https://openalex.org/W2115113540","https://openalex.org/W2116707205","https://openalex.org/W2117107959","https://openalex.org/W2124135806","https://openalex.org/W2131902476","https://openalex.org/W2132707658","https://openalex.org/W2132740946","https://openalex.org/W2140868856","https://openalex.org/W2141642277","https://openalex.org/W2142447693","https://openalex.org/W2144947606","https://openalex.org/W2146292644","https://openalex.org/W2146312260","https://openalex.org/W2146484505","https://openalex.org/W2149520619","https://openalex.org/W2161450374","https://openalex.org/W2168816784","https://openalex.org/W2171464982","https://openalex.org/W2197922133","https://openalex.org/W2206257597","https://openalex.org/W2213284782","https://openalex.org/W2232650769","https://openalex.org/W2253886635","https://openalex.org/W2280524460","https://openalex.org/W2317332612","https://openalex.org/W2333398204","https://openalex.org/W2336210752","https://openalex.org/W2489287057","https://openalex.org/W2493038938","https://openalex.org/W2542408568","https://openalex.org/W2556885577","https://openalex.org/W2573740717","https://openalex.org/W2586527223","https://openalex.org/W2593978390","https://openalex.org/W2595084745","https://openalex.org/W2606768929","https://openalex.org/W2608524918","https://openalex.org/W2608768503","https://openalex.org/W2712005870","https://openalex.org/W2735583838","https://openalex.org/W2753466150","https://openalex.org/W2765096732","https://openalex.org/W2765825922","https://openalex.org/W2766472479","https://openalex.org/W2766800448","https://openalex.org/W2783230672","https://openalex.org/W2787382087","https://openalex.org/W2887115994","https://openalex.org/W3106798016","https://openalex.org/W4206217612","https://openalex.org/W4211056322","https://openalex.org/W4232928776","https://openalex.org/W4232966707","https://openalex.org/W4242633182"],"related_works":["https://openalex.org/W2619601827","https://openalex.org/W2009243638","https://openalex.org/W3028500919","https://openalex.org/W2896054772","https://openalex.org/W4394734760","https://openalex.org/W4386565887","https://openalex.org/W4224022791","https://openalex.org/W3214535171","https://openalex.org/W2018761346","https://openalex.org/W4239771142"],"abstract_inverted_index":{"INTRODUCTION:":[0],"Cystic":[1],"fibrosis":[2],"(CF)":[3],"outcomes":[4],"and":[5,20,127,130,146,186],"survival":[6],"have":[7],"improved":[8],"over":[9],"the":[10,26,39,46,50,79,94,118,167],"last":[11],"century":[12],"primarily":[13],"due":[14],"to":[15,70,84,109,179],"advancements":[16,105],"in":[17,49,62,99,106],"antibiotics,":[18],"nutritional,":[19],"pulmonary":[21,107,121],"therapies.":[22],"Reviewed":[23],"here":[24],"are":[25,143,164],"significant":[27],"unmet":[28],"needs":[29],"that":[30,44,56],"exist":[31],"for":[32,104,117,134,166,176],"individuals":[33],"with":[34],"CF.":[35,190],"Areas":[36],"covered:":[37],"With":[38],"recent":[40],"development":[41,124],"of":[42,75,97,114,120,125,149,169,189],"medications":[43],"address":[45,180],"underlying":[47],"defect":[48],"CF":[51,63,80,153,170],"protein,":[52],"there":[53,57,66,172],"is":[54,82,93,157],"hope":[55],"will":[58],"be":[59],"continued":[60],"improvement":[61],"outcomes.":[64],"However,":[65],"remains":[67,173],"a":[68,101,174],"need":[69,102,175],"prevent":[71],"or":[72],"stop":[73],"progression":[74],"CF-related":[76],"complications,":[77],"as":[78,152],"protein":[81],"important":[83],"several":[85],"body":[86],"systems.":[87],"As":[88],"end":[89],"stage":[90],"lung":[91,135,139,184],"disease":[92,156],"primary":[95],"cause":[96],"mortality":[98],"CF,":[100],"exists":[103],"therapies":[108,178],"reduce":[110],"time":[111],"burden,":[112],"identification":[113],"best":[115],"practices":[116],"treatment":[119],"exacerbations,":[122],"further":[123],"anti-infective":[126],"anti-inflammatory":[128],"therapies,":[129],"appropriately":[131],"timed":[132],"referral":[133],"transplantation":[136],"at":[137],"end-stage":[138],"disease.":[140],"Extra-pulmonary":[141],"complications":[142,188],"increasingly":[144],"recognized":[145],"better":[147],"understanding":[148],"such":[150],"problems":[151],"related":[154],"liver":[155],"needed.":[158],"Expert":[159],"commentary:":[160],"While":[161],"CFTR":[162],"modulators":[163],"available":[165],"majority":[168],"patients,":[171],"effective":[177],"infection,":[181],"inflammation,":[182],"irreversible":[183],"disease,":[185],"extrapulmonary":[187]},"counts_by_year":[{"year":2025,"cited_by_count":1},{"year":2024,"cited_by_count":2},{"year":2023,"cited_by_count":1},{"year":2021,"cited_by_count":5},{"year":2020,"cited_by_count":5},{"year":2019,"cited_by_count":5}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2018-06-13T00:00:00"}