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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0105" class="elsevierStylePara elsevierViewall"><elsevierMultimedia ident="tb0005"></elsevierMultimedia></p><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Background</span><p id="par0020" class="elsevierStylePara elsevierViewall">Cystic fibrosis &#40;CF&#41; is the most common multisystem hereditary autosomal recessive disease in white people&#46; CF can affect the digestive system&#44; sweat glands and reproductive system&#44; among other organs and systems&#46; Its greatest morbidity and mortality is the result of the progressive deterioration of the respiratory system&#46; Since the first published report by Andersen in 1938 on patients with CF&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> at time when less than 50&#37; of patients lived longer than 1 year&#44; survival after diagnosis has clearly been steadily improving&#46; Toward the 1960s&#44; the median survival was 4 years&#46; By 1990&#44; the rate had increased to 28 years and currently exceeds 40 years&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The spectacular increase in life expectancy of these patients is the result of multiple factors that are essentially related to the implementation of specialized units and the use of new therapeutic modalities&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;5</span></a> CF has thereby become a disease that is not exclusively pediatric but rather one that also affects adults&#44; who present differential clinical manifestations&#44; such as osteoporosis and diabetes mellitus&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#8211;11</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Progress in diagnostic techniques&#44; with the inclusion of genetic tests&#44; has led us to other atypical clinical forms of CF&#44; which are frequently monosymptomatic and are present mainly in patients diagnosed in adulthood who frequently have negative or questionable results on sweat tests&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">10&#44;12</span></a> However&#44; the sweat test can also give negative or uncertain results for adults with typical clinical forms of CF&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9&#44;13</span></a> The main clinical forms of CF presentation are summarized in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#46; Understanding the clinical characteristics of these new forms of presentation is important for establishing a diagnosis of suspected CF in adulthood&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;10&#44;14</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">The main objective of this study was to determine the prevalence of patients with CF diagnosed after their 18th birthday&#46; For the secondary objectives&#44; we analyzed the reasons that motivated the referral to specialized CF units&#44; the most common clinical characteristics presented by the patients at that time and the genetic mutations detected&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Patients and methods</span><p id="par0035" class="elsevierStylePara elsevierViewall">Observational&#44; cross-sectional descriptive study of all patients &#40;18 years of age or older&#41; diagnosed with CF&#44; in 5 Spanish specialized units belonging to the hospitals La Paz &#40;Madrid&#41;&#44; Ramon y Cajal &#40;Madrid&#41;&#44; 12 de Octubre &#40;Madrid&#41;&#44; La Princesa &#40;Madrid&#41; and La Fe &#40;Valencia&#41; between March 1 and May 31&#44; 2012&#46; We analyzed all demographic characteristics&#44; clinical manifestations associated with CF&#44; genetic abnormalities&#44; respiratory function and sputum microbiology of every individual at the time of the study&#46; The CF diagnosis was established based on the presence of at least 1 clinical criterion or a family history of CF and the demonstration of abnormal functioning of the cystic fibrosis transmembrane conductance regulator &#40;CFTR&#41; by means of the sweat test results &#40;chloride ion concentration in sweat&#41; or the presence of 2 disease-causing mutations&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;7</span></a> Data were extracted from each patient&#39;s medical records&#46; The study followed the ethical research standards of the institutions participating in the study&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">All patients underwent a genetic study that included the search for 47 mutations using the Immunogenetics test<span class="elsevierStyleSup">&#174;</span>&#44; <span class="elsevierStyleItalic">Innolipa</span> CF-17&#44; CF-19 and CFTR Deletions&#43;6&#46; In specific cases&#44; when diagnostic questions remained after the initial genetic study and especially in cases in which no mutation was identified&#44; complete gene sequencing was requested&#46; The mutations have been expressed using the old nomenclature&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Based on the 2002 Spanish Society of Pneumology and Thoracic Surgery &#40;SEPAR&#41; guidelines&#44; the respiratory function parameters studied were the forced vital capacity &#40;FVC&#41; in milliliters and percentage of the predicted value&#44; the forced expiratory volume in one second &#40;FEV1&#41; in milliliters and the percentage of the predicted value and the FEV1&#47;FVC ratio analyzed at the time of the study cutoff &#40;or the closest to that&#41;&#46; We recorded the sputum microbiology data &#40;bacteria&#44; mycobacteria and fungi&#41; at the time of the data collection or as soon thereafter as possible&#46; We analyzed whether the bronchial infection was chronic&#44; intermittent or a primary infection&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The statistical analysis was conducted using the SPSS<span class="elsevierStyleSup">&#174;</span> statistical package&#44; version 22&#46;0&#46; Quantitative variables are shown as mean<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>standard deviation&#46; Qualitative variables are expressed as the number of patients and their percentage&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Results</span><p id="par0055" class="elsevierStylePara elsevierViewall">Eighty-nine patients were diagnosed with CF at the age of 18 or older at one of the 5 units participating in the study&#46; The total number of patients treated in the previously mentioned units was 600&#59; the percentage of patients who met the inclusion criteria was therefore 14&#46;8&#37;&#46; Of these&#44; 45 patients were women &#40;50&#46;6&#37;&#41; and 44 were men &#40;49&#46;4&#37;&#41;&#44; with a mean age at diagnosis of 36&#46;4 years &#40;median age&#44; 33 years&#59; range&#44; 18&#8211;78 years&#41;&#46; Eight of the patients &#40;8&#46;9&#37;&#41; were older than 60 at the time of the diagnosis&#46; Eighty-one patients &#40;91&#37;&#41; were of Spanish nationality &#40;<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>&#41;&#46; The sweat test was diagnostic in 77 patients &#40;86&#46;5&#37;&#41;&#46; The most frequently detected mutations were the heterozygous F508<span class="elsevierStyleItalic">del</span> and G542X&#44; which occurred in 40&#46;44&#37; &#40;36&#41; and 7&#46;86&#37; &#40;7&#41; of the patients&#44; respectively&#46; Mutations with frequencies below 1&#46;5&#37; were found in 15 patients &#40;16&#46;85&#37;&#41; &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>&#41;&#46; In all cases&#44; a diagnosis of CF was established if a mutation in the genetic study was detected&#44; regardless of the sweat test results&#46; Genetic sequencing was requested for the patients diagnosed with CF in whom no mutation was identified&#46; The definitive results for several of these patients were not available at the time this manuscript was written&#46;</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0060" class="elsevierStylePara elsevierViewall">The clinical and microbiological characteristics present at the time of diagnosis are listed in <a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>&#46; The most common clinical findings were the presence of bronchiectasis in 33 patients &#40;37&#46;1&#37;&#41;&#44; followed by sterility in 12 &#40;13&#46;5&#37;&#41;&#46; The most common colonizing microorganisms were methicillin-sensitive <span class="elsevierStyleItalic">Staphylococcus aureus</span> &#40;MSSA&#41; &#40;23&#46;6&#37;&#41; and <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> &#40;<span class="elsevierStyleItalic">P&#46; aeruginosa</span>&#41; &#40;13&#46;5&#37;&#41;&#46; The results for the respiratory function parameters indicated that the majority of patients showed a mild obstructive ventilatory impairment &#40;<a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a>&#41;&#46; We found data indicative of pancreatic insufficiency in 18 patients &#40;20&#46;2&#37;&#41; &#40;intake of pancreatic enzymes and biochemical data from the malabsorption study&#41; in 18 patients &#40;20&#46;2&#37;&#41;&#46;</p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia><elsevierMultimedia ident="tbl0025"></elsevierMultimedia></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Discussion</span><p id="par0065" class="elsevierStylePara elsevierViewall">In our study&#44; the prevalence of patients diagnosed with CF in adulthood was 14&#46;8&#37;&#46; If we compare this percentage with the data in the literature&#44; we find that recent studies have indicated a prevalence of CF diagnosis in adults approaching 10&#37; or even lower&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> In contrast with the data obtained by de Gracia et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> in Catalonia showing a prevalence of 20&#46;5&#37;&#44; our group had a somewhat lower prevalence&#46; The significant presence in our series of patients diagnosed after their 60th birthday should be noted &#40;8&#46;9&#37;&#41;&#46; The international literature indicates that the percentage of patients older than 40 years among adults with CF diagnosed in adulthood&#44; independently of the age at diagnosis&#44; is 7&#46;6&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Our study describes the demographic&#44; clinical and genetic characteristics and sputum microbiology of patients diagnosed with CF in adulthood&#46; At the time of diagnosis&#44; respiratory involvement and sterility were the most common clinical manifestations&#46; Of note is the presence of bronchiectasis overinfected by <span class="elsevierStyleItalic">P&#46; aeruginosa</span> or by MSSA&#46; We should also note the presence of recurrent respiratory infections and allergic bronchopulmonary aspergillosis &#40;ABPA&#41;&#44; which led to the diagnosis of CF in 7 and 5 cases&#44; respectively&#46; However&#44; the majority of our patients had good pancreatic function&#44; and none of our patients showed recurrent pancreatitis&#44; despite being a frequent symptom at diagnosis in other series&#44; both in children and adults with CF&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">14&#44;16&#44;17</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Our data contrast with those reported in a number of previous publications in which the patients diagnosed later in life had more advanced stages of the disease&#44; with increased repercussions on both pancreatic and respiratory functions&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a> Our patients had mild functional impairment&#46; This &#8220;contradiction&#8221; could be due to the fact that&#44; up until the discovery of the CF gene and even at present&#44; most adults diagnosed with CF had already shown obvious clinical manifestations since the first months of life&#44; despite being diagnosed much later in life in evolved stages of the disease&#46; These patients were frequently diagnosed with asthma or COPD&#44; based on clinical pictures of chronic productive cough and on spirometric patterns of obstructive pulmonary disorder&#46; This contradiction could also be explained by the genetic mutations detected in our patients&#44; given that&#44; for many of them&#44; only minority mutations or those considered mild &#40;&#62;16&#37;&#41; were detected&#44; mutations that are usually associated with little clinical repercussion&#46;<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">19&#8211;23</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">The development of specialized CF units&#44; with their multidisciplinary protocols&#44; has been shown to be one of the determinants in the current improved survival and quality of life of patients with CF&#46;<a class="elsevierStyleCrossRefs" href="#bib0120"><span class="elsevierStyleSup">24&#44;25</span></a> Educational activities have enabled not only pulmonologists but also physicians from other specialties and especially family doctors to consider CF in patients of any age with atypical phenotypes&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">The discovery in 1989 of the gene responsible for CF has enabled the expansion of our understanding of the pathogenesis and pathophysiology of this disease&#46; To date&#44; more than 900 mutations and more than 100 polymorphisms and variants have been identified&#46; Up to 6 distinct molecular mechanisms are known to be involved in the abnormal production of CFTR&#46; These mechanisms are responsible for the considerable variability in the pathophysiological abnormalities of the affected organs and as a result&#44; for their degree of involvement or integrity&#44; resulting in various phenotypic expressions of the disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;24&#8211;27</span></a> The first mutation described&#44; and the most common worldwide&#44; is F508<span class="elsevierStyleItalic">del</span>&#44; with characteristic mutations specific to various ethnic groups&#46; Spain is one of the countries with the greatest heterogeneity in this respect&#44; with clear differences between the south&#44; north and Mediterranean regions&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">28</span></a> In Spain&#44; 75 mutations represent 90&#46;2&#37; of all those encountered&#44; and the 2 most common are F508<span class="elsevierStyleItalic">del</span> &#40;53&#46;2&#37;&#41; and G542X &#40;8&#46;43&#37;&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">27&#44;29&#44;30</span></a> In our series&#44; the most common mutation was F508<span class="elsevierStyleItalic">del</span>&#44; coinciding with data recorded in other publications&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;9&#44;27</span></a> The presence of significant phenotypic differences between our patients &#40;consisting exclusively of adults&#41; should be noted&#44; as well as the characteristics of the classical disease&#44; which preferentially affect children &#40;especially at the pancreatic level&#41;&#46; These differences are likely due to the considerable quantity of polymorphisms and genetic variants&#44; which are being discovered thanks to technological developments in the field of molecular genetics&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Our study has a number of limitations&#44; given that some of the patients were waiting for the definitive results of the massive gene sequencing&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">CF should also be considered a disease that can be diagnosed in adulthood&#46; The sweat test should be incorporated within the standard differential diagnosis techniques for adult patients when faced with clinical conditions such as bronchiectasis &#40;with or without bronchial colonization by <span class="elsevierStyleItalic">P&#46; aeruginosa</span> or <span class="elsevierStyleItalic">S&#46; aureus</span>&#41;&#44; recurrent respiratory infections&#44; ABPA&#44; sterility or acute recurrent pancreatitis with no well-defined cause&#46; However&#44; as occurred with 12 of our 89 patients&#44; the sweat test can provide indeterminate or negative results&#46; Other diagnostic techniques should therefore be considered&#44; such as the nasal potential difference test&#44; especially in cases with indeterminate chloride ion concentration values &#40;40&#8211;60<span class="elsevierStyleHsp" style=""></span>mEquiv&#46;&#47;L&#41; or questionable genetic tests&#46;<a class="elsevierStyleCrossRefs" href="#bib0155"><span class="elsevierStyleSup">31&#8211;33</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">The diagnosis of CF should be considered in the differential diagnosis of bronchiectasis in patients of any age&#44; especially because the diagnosis of this disease entails essential genetic counseling for the patient&#39;s relatives&#46; It would also be of interest to compare this group of patients diagnosed in adulthood with those diagnosed before their 18th birthday&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Conflicts of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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            4 => "Conclusiones"
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          "titulo" => "Palabras clave"
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          "titulo" => "Background"
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          "identificador" => "sec0020"
          "titulo" => "Patients and methods"
        ]
        6 => array:2 [
          "identificador" => "sec0025"
          "titulo" => "Results"
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          "titulo" => "Discussion"
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          "titulo" => "Conflicts of interest"
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    "pdfFichero" => "main.pdf"
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    "fechaRecibido" => "2014-02-03"
    "fechaAceptado" => "2014-05-03"
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        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
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          "palabras" => array:4 [
            0 => "Cystic fibrosis"
            1 => "Adult"
            2 => "Epidemiology"
            3 => "Genetics"
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      ]
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        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palabras clave"
          "identificador" => "xpalclavsec335451"
          "palabras" => array:4 [
            0 => "Fibrosis qu&#237;stica"
            1 => "Adulto"
            2 => "Epidemiolog&#237;a"
            3 => "Gen&#233;tica"
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    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<span class="elsevierStyleSectionTitle" id="sect0010">Aim</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">To know the prevalence of the patients diagnosed of cystic fibrosis &#40;CF&#41; older than 18 years old of five specific Spanish Units and to analyze their clinical&#44; genetic and microbiological characteristics&#46;</p> <span class="elsevierStyleSectionTitle" id="sect0015">Patients and methods</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Observational&#44; cross-sectional&#44; descriptive study of patients diagnosed with CF at age or older than 18 years&#46; The variables analyzed were&#58; current age&#44; age at diagnosis&#44; sex&#44; nationality&#44; lung function parameters&#44; pathologies presented at diagnosis&#44; microbiological features and genetic findings&#46;</p> <span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Eigthy nine patients &#40;14&#46;8&#37; of the total of 600 CF patients followed at the participating units&#41;&#44; of which 45 patients were female &#40;50&#46;6&#37;&#41; and 44 were males &#40;49&#46;4&#37;&#41;&#44; were included with a mean age at diagnosis of 36&#46;4 years&#46; Eigthy one patients &#40;91&#37;&#41; were Spaniards&#46; The sweat test was diagnostic in 77 &#40;86&#46;5&#37;&#41; of the patients studied&#46; The sweat test was diagnostic in 77 of the 89 patients studied &#40;86&#46;5&#37;&#41;&#46; The most frequently detected mutations were F508<span class="elsevierStyleItalic">del</span>&#47;other and G542X&#47;other&#44; and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients &#40;37&#46;1&#37;&#41; followed by sterility in 12 patients &#40;13&#46;5&#37;&#41;&#46; The most common colonizing organisms were meticillin-sensitive <span class="elsevierStyleItalic">Staphylococcus aureus</span> &#40;<span class="elsevierStyleItalic">S&#46; aureus</span>&#41; &#40;23&#46;6&#37;&#41; and <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> &#40;<span class="elsevierStyleItalic">P</span>&#46; <span class="elsevierStyleItalic">aeruginosa</span>&#41; &#40;13&#46;5&#37;&#41;&#46; Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement&#46; The sweat test used to be indeterminate&#46;</p> <span class="elsevierStyleSectionTitle" id="sect0025">Conclusions</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">CF is also a disease which diagnosis can be in adulthood&#46; CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement&#44; so their prognosis tends to be favorable&#46;</p>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span class="elsevierStyleSectionTitle" id="sect0035">Objetivo</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Precisar la prevalencia de enfermos con fibrosis qu&#237;stica &#40;FQ&#41; diagnosticados en edades superiores a los 18 a&#241;os y analizar sus caracter&#237;sticas cl&#237;nicas&#44; gen&#233;ticas y microbiol&#243;gicas&#46;</p> <span class="elsevierStyleSectionTitle" id="sect0040">Pacientes y m&#233;todos</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Estudio observacional&#44; transversal y descriptivo de todos los pacientes diagnosticados de FQ a edad igual o superior a los 18 a&#241;os&#46; Las variables analizadas fueron&#58; edad actual&#44; edad al diagn&#243;stico&#44; sexo&#44; nacionalidad&#44; par&#225;metros de funci&#243;n pulmonar&#44; patolog&#237;as presentes al momento del diagn&#243;stico&#44; caracter&#237;sticas microbiol&#243;gicas y hallazgos gen&#233;ticos&#46;</p> <span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Se incluyeron 89 pacientes &#40;14&#44;8&#37;&#44; del total de 600 pacientes en seguimiento en las unidades participantes&#41;&#44; 45 mujeres &#40;50&#44;6&#37;&#41; y 44 varones &#40;49&#44;4&#37;&#41;&#44; con una edad media al diagn&#243;stico de 36&#44;4 a&#241;os&#46; Ochenta y un pacientes &#40;91&#37;&#41; eran de nacionalidad espa&#241;ola&#46; La prueba del sudor fue diagn&#243;stica en 77 de los 89 estudiados &#40;86&#44;5&#37;&#41;&#46; Las mutaciones detectadas con mayor frecuencia fueron la F508del&#47;otra y la G542X&#47;otra y los hallazgos cl&#237;nicos m&#225;s frecuentes en el momento del diagn&#243;stico fueron las bronquiectasias en 33 pacientes &#40;37&#44;1&#37;&#41; y la esterilidad en 12 &#40;13&#44;5&#37;&#41;&#46; Los microorganismos colonizadores m&#225;s frecuentes fueron <span class="elsevierStyleItalic">Staphylococcus aureus</span> &#40;<span class="elsevierStyleItalic">S&#46; aureus</span>&#41; sensible a meticilina &#40;23&#44;6&#37;&#41; y <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> &#40;<span class="elsevierStyleItalic">P</span>&#46; <span class="elsevierStyleItalic">aeruginosa</span>&#41; &#40;13&#44;5&#37;&#41;&#46; La mayor&#237;a de los pacientes presentaban una alteraci&#243;n ventilatoria obstructiva leve y no ten&#237;a afectaci&#243;n pancre&#225;tica&#46; La prueba del sudor con frecuencia ofreci&#243; resultados no concluyentes&#46;</p> <span class="elsevierStyleSectionTitle" id="sect0050">Conclusiones</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">La FQ es tambi&#233;n una enfermedad de diagn&#243;stico en la edad adulta&#46; Los pacientes diagnosticados en edad adulta presentan una funci&#243;n pulmonar levemente alterada y una baja incidencia de afectaci&#243;n pancre&#225;tica&#44; por lo que su pron&#243;stico tiende a ser favorable&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0030">Please cite this article as&#58; Ler&#237;n M&#44; Prados C&#44; Mart&#237;nez MT&#44; Ma&#237;z L&#44; Gir&#243;n R&#44; Sol&#233; A&#44; et al&#46; Fibrosis qu&#237;stica diagnosticada en edad adulta&#46; Rev Clin Esp&#46; 2014&#59;214&#58;289&#8211;295&#46;</p>"
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          "leyenda" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Adapted from L&#46; Ma&#237;z et al&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p>"
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                  \t\t\t\t">Classical characteristics &#40;predominantly in children&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Characteristics in adults&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">1&#46; Chronic sinopulmonary disease manifested by</span><span class="elsevierStyleHsp" style=""></span>Persistent bronchial colonization&#47;infection by typical microorganisms&#58; <span class="elsevierStyleItalic">Staphylococcus aureus&#44; Haemophilus influenzae&#44; Pseudomonas aeruginosa or Burkholderia cepacia</span><span class="elsevierStyleHsp" style=""></span>Chronic cough and expectoration<span class="elsevierStyleHsp" style=""></span>Persistent abnormalities in the chest radiography&#47;TCAR&#58; bronchiectasis&#44; infiltrates&#44; atelectasis&#44; hyperinflation<span class="elsevierStyleHsp" style=""></span>Airway obstruction with wheezing and air trapping<span class="elsevierStyleHsp" style=""></span>Nasal polyps&#59; radiological abnormalities in the paranasal sinuses<span class="elsevierStyleHsp" style=""></span>Acropachy<span class="elsevierStyleItalic">2&#46; Gastrointestinal and nutritional disorders&#58;</span><span class="elsevierStyleHsp" style=""></span>Intestinal&#58; meconium ileus&#44; rectal prolapse&#44; intestinal obstruction syndrome<span class="elsevierStyleHsp" style=""></span>Pancreatic&#58; pancreatic insufficiency&#44; recurrent pancreatitis<span class="elsevierStyleHsp" style=""></span>Hepatic&#58; focal biliary cirrhosis or multilobular cirrhosis<span class="elsevierStyleHsp" style=""></span>Nutritional&#58; malnutrition&#44; hypoproteinemia and edema&#44; complications secondary to vitamin deficiencies<span class="elsevierStyleItalic">3&#46; Salt-wasting syndromes&#58;</span><span class="elsevierStyleHsp" style=""></span>Chronic metabolic alkalosis&#44; acute salt wasting&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">&#8226; Bronchiectasis of unknown etiology&#8226; Chronic bronchial colonization&#47;infection by typical microorganisms&#58; <span class="elsevierStyleItalic">Staphylococcus aureus&#44; Haemophilus influenzae&#44; Pseudomonas aeruginosa or Burkholderia cepacia</span>&#8226; Recurrent pneumonia of unknown etiology&#8226; Allergic bronchopulmonary aspergillosis&#8226; Recurrent pancreatitis&#8226; Obstructive azoospermia due to congenital absence of vas deferens&nbsp;\t\t\t\t\t\t\n
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          "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Comparison of the clinical presentations suggestive of a diagnosis of cystic fibrosis diagnosed in adulthood&#46;</p>"
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                  \t\t\t\t" style="border-bottom: 2px solid black">Demographic characteristics&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" style="border-bottom: 2px solid black"><span class="elsevierStyleItalic">N</span><span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>89&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">&#37;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Median&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Range&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Sex &#40;male&#47;female&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">44&#47;45&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">49&#46;4&#47;50&#46;6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
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                  """
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                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;24&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">R334W&#47;other&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;24&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">L206W&#47;other&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;24&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Mutations with a frequency &#60;1&#46;5&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">16&#46;85&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Unknown&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">13&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">14&#46;60&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab529120.png"
              ]
            ]
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Mutations detected in the 89 patients diagnosed with cystic fibrosis in adulthood who were enrolled in the study &#40;178 chromosomes&#41;&#46;</p>"
        ]
      ]
      3 => array:7 [
        "identificador" => "tbl0020"
        "etiqueta" => "Table 4"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "tabla" => array:2 [
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Clinical characteristics&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">No&#46; cases &#40;<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>89&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Colonizing microorganism&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">No&#46; cases &#40;<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>89&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Bronchiectasis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">37&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No colonization&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">20&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">22&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">ENT involvement&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3&#46;4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Pseudomonas aeruginosa</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">13&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Pancreatitis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">MRSA<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Sterility&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">13&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">MSSA<a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">21&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">23&#46;6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">ABPA<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">5&#46;6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleItalic">Haemophilus influenzae</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Collagenopathies&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Two or more colonizers&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">28&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">31&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Difficult-to-control asthma&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">0&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Chronic cough&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Recurrent respiratory infections&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">7&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Hemoptysis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Family history of CF&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">9&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">10&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Two or more diseases at diagnosis&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">16&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">18&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
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                0 => "xTab529119.png"
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            1 => array:3 [
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            ]
          ]
        ]
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          "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Clinical and microbiological characteristics of the 89 patients diagnosed with cystic fibrosis in adulthood who were enrolled in the study&#46;</p>"
        ]
      ]
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                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Mean &#40;<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>89&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Median&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Standard deviation&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">FEV1 &#40;mL&#41;<a class="elsevierStyleCrossRef" href="#tblfn0020"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">2640&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">2&#46;460&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">1&#46;12&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">FEV1 &#40;&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">77&#46;2&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">76&#37;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">25&#46;20&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">FVC &#40;mL&#41;<a class="elsevierStyleCrossRef" href="#tblfn0025"><span class="elsevierStyleSup">b</span></a>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">3240&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">3090&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">1&#46;14&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">FVC &#40;&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">83&#46;6&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">85&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">25&#46;19&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t">FEV1&#47;FVC&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">71&#46;05&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">74&#37;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">14&#46;83&nbsp;\t\t\t\t\t\t\n
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              "identificador" => "tblfn0025"
              "etiqueta" => "b"
              "nota" => "<p class="elsevierStyleNotepara" id="npar0025">Forced vital capacity&#46;</p>"
            ]
          ]
        ]
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          "en" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">Main respiratory function parameters of the 89 patients diagnosed with cystic fibrosis in adulthood who were enrolled in the study&#46;</p>"
        ]
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        "tipo" => "MULTIMEDIATEXTO"
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          "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">What we know&#63;</span><p id="par0005" class="elsevierStylePara elsevierViewall">Cystic fibrosis &#40;CF&#41; is the most common hereditary autosomal recessive disease in whites&#46; The considerable increase in survival and the advances in genetic diagnosis have changed this disease from an exclusively pediatric illness&#46; This study seeks to determine the prevalence of patients with cystic fibrosis diagnosed in adulthood&#44; their clinical characteristics and detected genetic mutations&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">What this article provides&#63;</span><p id="par0010" class="elsevierStylePara elsevierViewall">A total of 14&#46;8&#37; of the patients were diagnosed in adulthood&#46; Respiratory involvement and sterility were the most common clinical manifestations&#46; The patients&#8217; lung function was not significantly affected&#44; and there was little pancreatic involvement&#59; their prognosis is therefore favorable&#46; The most common mutation was F508<span class="elsevierStyleItalic">del</span>&#44; followed by G542X&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">The Editors</p></span></span>"
        ]
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
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        0 => array:2 [
          "identificador" => "bibs0005"
          "bibliografiaReferencia" => array:33 [
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              "etiqueta" => "1"
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                    0 => array:2 [
                      "titulo" => "Cystic fibrosis"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "S&#46;M&#46; Rowe"
                            1 => "S&#46; Miller"
                            2 => "E&#46;J&#46; Sorscher"
                          ]
                        ]
                      ]
                    ]
                  ]
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                    0 => array:2 [
                      "doi" => "10.1056/NEJMra043184"
                      "Revista" => array:6 [
                        "tituloSerie" => "N Engl J Med"
                        "fecha" => "2005"
                        "volumen" => "352"
                        "paginaInicial" => "1992"
                        "paginaFinal" => "2001"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15888700"
                            "web" => "Medline"
                          ]
                        ]
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              "identificador" => "bib0010"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Cystic fibrosis pulmonary guidelines chronic medications for maintenance of lung health"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "P&#46;J&#46; Mogayzel"
                            1 => "E&#46;T&#46; Naureckas"
                            2 => "K&#46;A&#46; Robinson"
                            3 => "G&#46; Mueller"
                            4 => "D&#46; Hadjiliadis"
                            5 => "J&#46;B&#46; Hoag"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Am J Respir Crit Care Med"
                        "fecha" => "2013"
                        "volumen" => "187"
                        "paginaInicial" => "680"
                        "paginaFinal" => "689"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23540878"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0015"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Normativa del diagn&#243;stico y el tratamiento de la afecci&#243;n respiratoria en la fibrosis qu&#237;stica"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "L&#46; Ma&#237;z"
                            1 => "F&#46; Baranda"
                            2 => "R&#46; Coll"
                            3 => "C&#46; Prados"
                            4 => "M&#46; Vendrell"
                            5 => "A&#46; Escribano"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:7 [
                        "tituloSerie" => "Arch Bronconeumol"
                        "fecha" => "2001"
                        "volumen" => "37"
                        "paginaInicial" => "316"
                        "paginaFinal" => "324"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11562317"
                            "web" => "Medline"
                          ]
                        ]
                        "itemHostRev" => array:3 [
                          "pii" => "S1521694209001570"
                          "estado" => "S300"
                          "issn" => "15216942"
                        ]
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                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0020"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Treatment of lung infection in patients with cystic fibrosis&#58; current and future strategies"
                      "autores" => array:1 [
                        0 => array:3 [
                          "colaboracion" => "for the Consensus Study Group"
                          "etal" => false
                          "autores" => array:4 [
                            0 => "G&#46; D&#246;ring"
                            1 => "P&#46; Flume"
                            2 => "H&#46; Harry Heijerman"
                            3 => "J&#46;S&#46; Elborn"
                          ]
                        ]
                      ]
                    ]
                  ]
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Vol. 214. Issue 6.
Pages 289-295 (August - September 2014)
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Vol. 214. Issue 6.
Pages 289-295 (August - September 2014)
Original article
Cystic fibrosis in adult age
Fibrosis quística diagnosticada en edad adulta
Visits
227
M. Lerína, C. Pradosa,
Corresponding author
, M.T. Martínezb, L. Maízc, R. Girónd, A. Solée, J.J. Cabanillasa, R. Álvarez-Salaa
a Unidad de Fibrosis Quística, Servicio de Neumología, Hospital Universitario La Paz, Madrid, Spain
b Unidad de Fibrosis Quística, Servicio de Neumología, Hospital Universitario 12 de Octubre, Madrid, Spain
c Unidad de Fibrosis Quística, Servicio de Neumología, Hospital Universitario Ramón y Cajal, Madrid, Spain
d Unidad de Fibrosis Quística, Servicio de Neumología, Hospital Universitario La Princesa, Madrid, Spain
e Unidad de Fibrosis Quística, Servicio de Neumología, Hospital Universitario La Fe, Valencia, Spain
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Tables (5)
Table 1. Comparison of the clinical presentations suggestive of a diagnosis of cystic fibrosis diagnosed in adulthood.
Table 2. Demographic characteristics of the 89 patients diagnosed with cystic fibrosis in adulthood who were enrolled in the study.
Table 3. Mutations detected in the 89 patients diagnosed with cystic fibrosis in adulthood who were enrolled in the study (178 chromosomes).
Table 4. Clinical and microbiological characteristics of the 89 patients diagnosed with cystic fibrosis in adulthood who were enrolled in the study.
Table 5. Main respiratory function parameters of the 89 patients diagnosed with cystic fibrosis in adulthood who were enrolled in the study.
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Abstract
Aim

To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics.

Patients and methods

Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings.

Results

Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508del/other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus (S. aureus) (23.6%) and Pseudomonas aeruginosa (P. aeruginosa) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate.

Conclusions

CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable.

Keywords:
Cystic fibrosis
Adult
Epidemiology
Genetics
Resumen
Objetivo

Precisar la prevalencia de enfermos con fibrosis quística (FQ) diagnosticados en edades superiores a los 18 años y analizar sus características clínicas, genéticas y microbiológicas.

Pacientes y métodos

Estudio observacional, transversal y descriptivo de todos los pacientes diagnosticados de FQ a edad igual o superior a los 18 años. Las variables analizadas fueron: edad actual, edad al diagnóstico, sexo, nacionalidad, parámetros de función pulmonar, patologías presentes al momento del diagnóstico, características microbiológicas y hallazgos genéticos.

Resultados

Se incluyeron 89 pacientes (14,8%, del total de 600 pacientes en seguimiento en las unidades participantes), 45 mujeres (50,6%) y 44 varones (49,4%), con una edad media al diagnóstico de 36,4 años. Ochenta y un pacientes (91%) eran de nacionalidad española. La prueba del sudor fue diagnóstica en 77 de los 89 estudiados (86,5%). Las mutaciones detectadas con mayor frecuencia fueron la F508del/otra y la G542X/otra y los hallazgos clínicos más frecuentes en el momento del diagnóstico fueron las bronquiectasias en 33 pacientes (37,1%) y la esterilidad en 12 (13,5%). Los microorganismos colonizadores más frecuentes fueron Staphylococcus aureus (S. aureus) sensible a meticilina (23,6%) y Pseudomonas aeruginosa (P. aeruginosa) (13,5%). La mayoría de los pacientes presentaban una alteración ventilatoria obstructiva leve y no tenía afectación pancreática. La prueba del sudor con frecuencia ofreció resultados no concluyentes.

Conclusiones

La FQ es también una enfermedad de diagnóstico en la edad adulta. Los pacientes diagnosticados en edad adulta presentan una función pulmonar levemente alterada y una baja incidencia de afectación pancreática, por lo que su pronóstico tiende a ser favorable.

Palabras clave:
Fibrosis quística
Adulto
Epidemiología
Genética

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