{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T12:54:33Z","timestamp":1792068873924,"version":"4.4.0"},"reference-count":33,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2014,8,1]],"date-time":"2014-08-01T00:00:00Z","timestamp":1406851200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["elsevier.es","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Revista Cl\u00ednica Espa\u00f1ola"],"published-print":{"date-parts":[[2014,8]]},"DOI":"10.1016\/j.rce.2014.05.001","type":"journal-article","created":{"date-parts":[[2014,6,16]],"date-time":"2014-06-16T21:01:02Z","timestamp":1402952462000},"page":"289-295","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":7,"title":["Fibrosis qu\u00edstica diagnosticada en edad adulta"],"prefix":"10.1016","volume":"214","author":[{"given":"M.","family":"Ler\u00edn","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Prados","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.T.","family":"Mart\u00ednez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Ma\u00edz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Gir\u00f3n","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Sol\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.J.","family":"Cabanillas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"\u00c1lvarez-Sala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.rce.2014.05.001_bib0005","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1056\/NEJMra043184","article-title":"Cystic fibrosis","volume":"352","author":"Rowe","year":"2005","journal-title":"N Engl J Med"},{"key":"10.1016\/j.rce.2014.05.001_bib0010","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1164\/rccm.201207-1160OE","article-title":"Cystic fibrosis pulmonary guidelines chronic medications for maintenance of lung health","volume":"187","author":"Mogayzel","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.rce.2014.05.001_bib0015","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/S0300-2896(01)75101-0","article-title":"Normativa del diagn\u00f3stico y el tratamiento de la afecci\u00f3n respiratoria en la fibrosis qu\u00edstica","volume":"37","author":"Ma\u00edz","year":"2001","journal-title":"Arch Bronconeumol"},{"key":"10.1016\/j.rce.2014.05.001_bib0020","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1016\/j.jcf.2012.10.004","article-title":"Treatment of lung infection in patients with cystic fibrosis: Current and future strategies","volume":"11","author":"D\u00f6ring","year":"2012","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.rce.2014.05.001_bib0025","unstructured":"Clinical guidelines: care of children with cystic fibrosis. Royal Brompton Hospital. 2014, 6.th edition. Disponibles en: http:\/\/www.rbht.nhs.uk\/healthprofessionals\/clinical-departments\/paediatrics\/childrencf\/Consultado el 08.abril.2014."},{"key":"10.1016\/j.rce.2014.05.001_bib0030","series-title":"Cystic fibrosis in the adults.","first-page":"27","article-title":"Genetics, diagnosis and clinical phenotype","author":"Knowles","year":"1999"},{"key":"10.1016\/j.rce.2014.05.001_bib0035","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1016\/S0025-7753(02)73513-6","article-title":"Fibrosis qu\u00edstica del adulto: estudio de 111 pacientes","volume":"119","author":"De Gracia","year":"2002","journal-title":"Med Clin (Barc)"},{"key":"10.1016\/j.rce.2014.05.001_bib0040","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1157\/13046439","article-title":"Fibrosis qu\u00edstica en la edad adulta: nuevas formas cl\u00ednicas","volume":"120","author":"Cabrera","year":"2003","journal-title":"Med Clin (Barc)."},{"key":"10.1016\/j.rce.2014.05.001_bib0045","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1016\/S0022-3476(05)80242-2","article-title":"Pancreatitis in young children with cystic fibrosis","volume":"120","author":"Atlas","year":"1992","journal-title":"J Pediar"},{"key":"10.1016\/j.rce.2014.05.001_bib0050","doi-asserted-by":"crossref","first-page":"1S","DOI":"10.1378\/chest.125.1_suppl.1S","article-title":"Cystic fibrosis adult care: consensus conference report","volume":"125","author":"Yankaskas","year":"2004","journal-title":"Chest"},{"key":"10.1016\/j.rce.2014.05.001_bib0055","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1378\/chest.118.2.427","article-title":"Health status and sociodemographic characteristics of adults receiving a cystic fibrosis diagnosis after 18 years","volume":"118","author":"Widerman","year":"2000","journal-title":"Chest"},{"key":"10.1016\/j.rce.2014.05.001_bib0060","article-title":"Extensive sequence analysis of CFTR, SCNN1A, SCNN1B SCNN1G and SERPINA1 suggests an oligogenic basis for cystic fibrosis-like phenotypes","author":"Ramos","year":"2013","journal-title":"Clin Genet"},{"key":"10.1016\/j.rce.2014.05.001_bib0065","first-page":"121","article-title":"Cystic fibrosis in adults 75 cases and review of 232 cases in literature","volume":"66","author":"Di Sant\u2019Agnese","year":"1979","journal-title":"Am J Med"},{"key":"10.1016\/j.rce.2014.05.001_bib0070","first-page":"1311","article-title":"Evaluation and monitoring in cystic fibrosis centers","volume":"144","year":"1990","journal-title":"Am J Dis Child"},{"key":"10.1016\/j.rce.2014.05.001_bib0075","first-page":"200","article-title":"Ageing in cystic fibrosis and long-term survival","volume":"13","author":"Simmonds","year":"2012","journal-title":"Paediatr Respir Rev"},{"key":"10.1016\/j.rce.2014.05.001_bib0080","unstructured":"Cystic Fibrosis Mutation Database. The Cystic Fibrosis Genetic Analysis Consortium. [consultado 8 Ab 2014] Disponible en: www.genet.sickkids.on.ca\/cftr\/"},{"key":"10.1016\/j.rce.2014.05.001_bib0085","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1136\/thx.2004.031153","article-title":"Genotype-phenotype correlation for pulmonary function in cystic fibrosis","volume":"60","author":"De Gracia","year":"2005","journal-title":"Thorax"},{"key":"10.1016\/j.rce.2014.05.001_bib0090","series-title":"Cystic fibrosis in the adults.","first-page":"3","article-title":"Cystic fibrosis gene and functions of CFTR","author":"Stutts","year":"1999"},{"key":"10.1016\/j.rce.2014.05.001_bib0095","first-page":"1341","article-title":"Atypical cystic fibrosis Identification in the primary care setting","volume":"58","author":"Scharam","year":"2012","journal-title":"Can Fam Physician"},{"key":"10.1016\/j.rce.2014.05.001_bib0100","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1183\/09031936.00166412","article-title":"Factors associated with FEV1 decline in cystic fibrosis: analysis of the data of the ECFS patient registry","volume":"43","author":"Kerem","year":"2014","journal-title":"Eur Respir J"},{"key":"10.1016\/j.rce.2014.05.001_bib0105","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/j.jcf.2012.03.009","article-title":"Risk factors for rate of decline in FEV1 in adults with cystic fibrosis","volume":"11","author":"Konstan","year":"2012","journal-title":"J Cyst Fibros"},{"key":"10.1016\/j.rce.2014.05.001_bib0110","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1183\/09059180.00008112","article-title":"Personalised medicine for cystic fibrosis: treating the basic defect","volume":"22","author":"Elborn","year":"2013","journal-title":"Eur Respir Rev"},{"key":"10.1016\/j.rce.2014.05.001_bib0115","first-page":"166","article-title":"Non-pulmonary chronic diseases in adults with cystic fibrosis: analysis of data from the cystic fibrosis registry","volume":"106","author":"Somerville","year":"2013","journal-title":"Ir Med J"},{"key":"10.1016\/j.rce.2014.05.001_bib0120","doi-asserted-by":"crossref","first-page":"3729","DOI":"10.1074\/jbc.275.6.3729","article-title":"Cystic fibrosis transmembrane conductance regulator structure and function of an epithelial chloride channel","volume":"275","author":"Akabas","year":"2000","journal-title":"J Biol Chem"},{"key":"10.1016\/j.rce.2014.05.001_bib0125","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1007\/s004390050643","article-title":"High heterogeneity for cystic fibrosis in Spanish families: 75 mutations account for 90% of chromosomes","volume":"101","author":"Casals","year":"1997","journal-title":"Hum Genet"},{"key":"10.1016\/j.rce.2014.05.001_bib0130","doi-asserted-by":"crossref","unstructured":"Teller\u00eda JJ, Alonso MJ, Calvo C, Alonso M, Blanco A. Spectrum of CFTR mutations in the Middle North of Spain and identification of a novel mutation (1341G-->A). Mutation in brief no. 252. Online. Hum Mutat 1999; 14: 89.","DOI":"10.1002\/(SICI)1098-1004(1999)14:1<89::AID-HUMU17>3.0.CO;2-8"},{"key":"10.1016\/j.rce.2014.05.001_bib0135","first-page":"681","article-title":"Fibrosis qu\u00edstica en Asturias: una frecuencia elevada de la mutaci\u00f3n delta F508","volume":"103","author":"Coto","year":"1994","journal-title":"Med Clin (Barc)"},{"key":"10.1016\/j.rce.2014.05.001_bib0140","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1016\/j.mehy.2009.12.018","article-title":"An evolutionary approach to the high frequency of the Delta F508 CFTR mutation in European populations","volume":"74","author":"Alfonso-S\u00e1nchez","year":"2010","journal-title":"Med Hypotheses"},{"key":"10.1016\/j.rce.2014.05.001_bib0145","unstructured":"Paz-Vali\u00f1as L., Garc\u00eda-Vega FJ. Cribado neonatal de la fibrosis qu\u00edstica. Santiago de Compostela: Servicio Galego de Sa\u00fade, Axencia de Avaliaci\u00f3n de Tecnolox\u00edas Sanitarias de Galicia, avalia-t; 2004. Serie Avaliaci\u00f3n de tecnolox\u00edas. Informes de avaliaci\u00f3n: INF2004\/02. [consultado 8 ab 2014] Disponible en: http:\/\/www.sergas.es\/cas\/Servicios\/docs\/AvaliacionTecnoloxias\/Fibr%20quistica%20%20INF2004%2002.pdf Consultado el 08.abril.2014."},{"key":"10.1016\/j.rce.2014.05.001_bib0150","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1159\/000029538","article-title":"Clinical features associated with a delayed diagnosis of cystic fibrosis","volume":"67","author":"Mc Closkey","year":"2000","journal-title":"Respiration"},{"key":"10.1016\/j.rce.2014.05.001_bib0155","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/S0300-2896(15)31897-4","article-title":"Positivizacion del test del sudor tras el inicio de afectaci\u00f3n pancre\u00e1tica en paciente adulto con fibrosis qu\u00edstica","volume":"24","author":"Rom\u00e1n","year":"1988","journal-title":"Arch Bronconeumol"},{"key":"10.1016\/j.rce.2014.05.001_bib0160","doi-asserted-by":"crossref","first-page":"1908","DOI":"10.1164\/ajrccm.155.6.9196094","article-title":"Conventional and modified nasal potencial-difference measurement in cystic fibrosis","volume":"155","author":"Hofmann","year":"1997","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.rce.2014.05.001_bib0165","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1157\/13083278","article-title":"Prueba de la diferencia de potencial nasal para el diagn\u00f3stico de la fibrosis qu\u00edstica","volume":"42","author":"Domingo-Ribas","year":"2006","journal-title":"Arch Bronconeumol"}],"container-title":["Revista Cl\u00ednica Espa\u00f1ola"],"original-title":[],"language":"es","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014256514002094?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014256514002094?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,10,3]],"date-time":"2018-10-03T10:22:38Z","timestamp":1538562158000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0014256514002094"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,8]]},"references-count":33,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2014,8]]}},"alternative-id":["S0014256514002094"],"URL":"https:\/\/doi.org\/10.1016\/j.rce.2014.05.001","relation":{},"ISSN":["0014-2565"],"issn-type":[{"value":"0014-2565","type":"print"}],"subject":[],"published":{"date-parts":[[2014,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Fibrosis qu\u00edstica diagnosticada en edad adulta","name":"articletitle","label":"Article Title"},{"value":"Revista Cl\u00ednica Espa\u00f1ola","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.rce.2014.05.001","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2014 Elsevier Espa\u00f1a, S.L. All rights reserved.","name":"copyright","label":"Copyright"}]}}