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hipotiroidismo congénito diagnosticados por cribado neonatal" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1210 "Ancho" => 2167 "Tamanyo" => 177761 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Algorithm for re-evaluation and definitive diagnosis of congenital hypothyroidism.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Amparo Rodríguez Sánchez, María J. Chueca Guindulain, María Alija Merillas, Susana Ares Segura, José Carlos Moreno Navarro, María Dolores Rodríguez Arnao" "autores" => array:7 [ 0 => array:2 [ "nombre" => "Amparo" "apellidos" => "Rodríguez Sánchez" ] 1 => array:2 [ "nombre" => "María J." 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List of authors of the Working Group on Cystic Fibrosis of the Sociedad de Neumología Pediátrica (Spanish Sociology of Paediatric Pulmonology)" ] ] ] 2 => array:3 [ "autoresLista" => "María José Alonso, Marina Álvarez, Anselmo Andrés Martín, María Isabel Barrio Gómez de Agüero, María Jesús Cabero Pérez, Pilar Caro Aguilera, María Cols Roig, Isidoro Cortell Aznar-Pérez, Jordi Costa Colomer, Isabel Delgado Pecellín, Amparo Escribano Montaner, Joan Figuerola Mulet, Gloria García Hernández, Pilar Guayarte, David Gil Ortega, David Gómez Pastrana, Adelaida Lamas Ferreiro, José Luis Marín Soria, Carlos Martín de Vicente, Martín Navarro Merino, Concepción Oliva Hernández, Javier Pérez Frías, Estela Pérez Ruiz, Sandra Rovira Amigo, Antonio Salcedo Posadas, Manuel Sánchez-Solís, Josep Sirvent Gómez, Carlos Vázquez Cordero, José Ramón Villa Asensi" "autores" => array:29 [ 0 => array:3 [ "nombre" => "María José" "apellidos" => "Alonso" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">aa</span>" "identificador" => "aff0020" ] ] ] 1 => array:3 [ "nombre" => "Marina" "apellidos" => "Álvarez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ab</span>" "identificador" => "aff0025" ] ] ] 2 => array:3 [ "nombre" => "Anselmo Andrés" "apellidos" => "Martín" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ac</span>" "identificador" => "aff0030" ] ] ] 3 => array:3 [ "nombre" => "María Isabel Barrio" "apellidos" => "Gómez de Agüero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ad</span>" "identificador" => "aff0035" ] ] ] 4 => array:3 [ "nombre" => "María Jesús" "apellidos" => "Cabero Pérez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ae</span>" "identificador" => "aff0040" ] ] ] 5 => array:3 [ "nombre" => "Pilar" "apellidos" => "Caro Aguilera" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">af</span>" "identificador" => "aff0045" ] ] ] 6 => array:3 [ "nombre" => "María" "apellidos" => "Cols Roig" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ag</span>" "identificador" => "aff0050" ] ] ] 7 => array:3 [ "nombre" => "Isidoro Cortell" "apellidos" => "Aznar-Pérez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ah</span>" "identificador" => "aff0055" ] ] ] 8 => array:3 [ "nombre" => "Jordi Costa" "apellidos" => "Colomer" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ag</span>" "identificador" => "aff0050" ] ] ] 9 => array:3 [ "nombre" => "Isabel" "apellidos" => "Delgado Pecellín" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">ai</span>" "identificador" => "aff0060" ] ] ] 10 => array:3 [ "nombre" => "Amparo" "apellidos" => "Escribano Montaner" "referencia" => array:1 [ 0 => 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Gastroenterology, Hepatology and Nutritional Support Unit, Hospital Universitari Vall d’Hebron, Barcelona" "etiqueta" => "ab" "identificador" => "aff0025" ] 2 => array:3 [ "entidad" => "Department of Paediatric Pulmonology, Department and Clinical Management Unit of Paediatrics, Hospital Universitario Virgen Macarena, Seville" "etiqueta" => "ac" "identificador" => "aff0030" ] 3 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario La Paz, Madrid" "etiqueta" => "ad" "identificador" => "aff0035" ] 4 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Marqués de Valdecilla, Santander" "etiqueta" => "ae" "identificador" => "aff0040" ] 5 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Materno-Infantil Universitario de Málaga, Malaga" "etiqueta" => "af" "identificador" => "aff0045" ] 6 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Department of Paediatrics, Hospital Sant Joan de Déu, Barcelona" "etiqueta" => "ag" "identificador" => "aff0050" ] 7 => array:3 [ "entidad" => "Department of Paediatric Allergy and Pulmonology, Hospital Universitario La Fe, Valencia" "etiqueta" => "ah" "identificador" => "aff0055" ] 8 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Virgen del Rocío, Seville" "etiqueta" => "ai" "identificador" => "aff0060" ] 9 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Clínico Universitario de Valencia, Universitat de València, Valencia" "etiqueta" => "aj" "identificador" => "aff0065" ] 10 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Son Espases, Palma de Mallorca" "etiqueta" => "ak" "identificador" => "aff0070" ] 11 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario 12 de Octubre, Madrid" "etiqueta" => "al" "identificador" => "aff0075" ] 12 => array:3 [ "entidad" => "Paediatric Gastroenterology Unit, Hospital Universitario Parc Taulí, Sabadell" "etiqueta" => "am" "identificador" => "aff0080" ] 13 => array:3 [ "entidad" => "Paediatric Gastroenterology, Hepatology and Nutrition Unit, Hospital Universitario Virgen de la Arrixaca, Murcia" "etiqueta" => "an" "identificador" => "aff0085" ] 14 => array:3 [ "entidad" => "Paediatric Pulmonology Unit, Hospital de Jerez, Jerez de la Frontera" "etiqueta" => "ao" "identificador" => "aff0090" ] 15 => array:3 [ "entidad" => "Cystic Fibrosis Unit, Hospital Universitario Ramón y Cajal, Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Madrid" "etiqueta" => "ap" "identificador" => "aff0095" ] 16 => array:3 [ "entidad" => "Neonatal Screening Laboratory of Catalonia, Hospital Clinic, Barcelona" "etiqueta" => "aq" "identificador" => "aff0100" ] 17 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Miguel Servet, Zaragoza" "etiqueta" => "ar" "identificador" => "aff0105" ] 18 => array:3 [ "entidad" => "Hospital Universitario Nuestra Señora de la Candelaria, Santa Cruz de Tenerife" "etiqueta" => "as" "identificador" => "aff0110" ] 19 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Vall d’Hebron, Barcelona" "etiqueta" => "at" "identificador" => "aff0115" ] 20 => array:3 [ "entidad" => "Cystic Fibrosis Unit, Hospital Universitario del Niño Jesús-Hospital Universitario Gregorio Marañón, Madrid" "etiqueta" => "au" "identificador" => "aff0120" ] 21 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Virgen de la Arrixaca, Murcia" "etiqueta" => "av" "identificador" => "aff0125" ] 22 => array:3 [ "entidad" => "Paediatric Pulmonology Unit, Hospital Materno Infantil, Complexo Hospitalario Universitario A Coruña, A Coruña" "etiqueta" => "aw" "identificador" => "aff0130" ] 23 => array:3 [ "entidad" => "Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Cruces, Bilbao" "etiqueta" => "ax" "identificador" => "aff0135" ] 24 => array:3 [ "entidad" => "Pulmonology and Cystic Fibrosis Unit, Hospital Universitario del Niño Jesús, Madrid" "etiqueta" => "ay" "identificador" => "aff0140" ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Protocolo de seguimiento de pacientes con fibrosis quística diagnosticados por cribado neonatal" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1374 "Ancho" => 2167 "Tamanyo" => 237176 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Protocols for diagnosis of cystic fibrosis through neonatal screening proposed by the European Best Practice Guidelines for Cystic Fibrosis Neonatal Screening<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">7</span></a> applied in Spain.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Cystic fibrosis (CF) is the most frequent severe genetic disorder with an autosomal recessive inheritance pattern in the Caucasian population, with an incidence of 1 per 1800 to 25<span class="elsevierStyleHsp" style=""></span>000 live births depending on the geographical region or ethnicity. In 2016, the Canadian Cystic Fibrosis Foundation reported a median survival of 53.3 years.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">1</span></a> The substantial increase in survival in these patients is due to a series of factors, among which the implementation of neonatal screening programmes for early diagnosis have played a significant role.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Cystic fibrosis meets the criteria warranting early detection and NS for the purpose of determining the actual incidence of the disease, early genetic counselling and immediate initiation of treatment with the purpose of preventing or minimising lung damage, as molecules that can target and correct the defect in the abnormal cystic fibrosis transmembrane conductance regulator (CFTR) protein are currently available.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Since 2015, neonatal screening is performed in every autonomous community in Spain.</p><p id="par0020" class="elsevierStylePara elsevierViewall">We think it would be very convenient to have an updated consensus-based guideline for the diagnosis of CF and the follow-up and treatment of patients that meet the diagnostic criteria. This document summarises all the multidisciplinary aspects involved in the management of these patients.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Benefits and risks of neonatal screening for cystic fibrosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">At present, most professionals devoted to the care of patients with CF agree that a well-designed neonatal screening programme involves a positive risk-benefit ratio where the benefits (nutritional, respiratory, of early eradication of microorganisms, genetic counselling and participation in early intervention clinical trials) outweigh the disadvantages (especially in the psychosocial dimension) and are cost-effective in the long term.<a class="elsevierStyleCrossRefs" href="#bib0200"><span class="elsevierStyleSup">2–5</span></a> To guarantee the beneficial effect of neonatal screening, patients with CF should be managed appropriately (following standard care protocols) immediately after diagnosis in specialised CF units.<a class="elsevierStyleCrossRefs" href="#bib0220"><span class="elsevierStyleSup">6,7</span></a> Most of the risks of neonatal screening are minimised by taking the following measures<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">6</span></a>: (a) establishing a neonatal screening programme adapted to the characteristics of the population, (b) communicating effectively with parents and giving them useful information throughout the diagnostic process, both in case of affected patients and of patients who are carriers or that received a false positive result, trying to keep the time elapsed to establishment of the definitive diagnosis to a minimum; (c) offering medical follow-up to affected children.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Neonatal screening protocols in Spain</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Immunoreactive trypsinogen</span><p id="par0030" class="elsevierStylePara elsevierViewall">Serum levels of immunoreactive trypsinogen (IRT) are higher in newborns with CF and remain elevated longer compared to newborns who are not affected by the disease.<a class="elsevierStyleCrossRefs" href="#bib0230"><span class="elsevierStyleSup">8,9</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">An elevation of IRT at birth is not specific for CF, as there are healthy newborns that experience a transient elevation of this enzyme. The specificity of elevation of IRT in a single sample is low, so protocols have been developed that use a DNA test in the first neonatal sample (IRT<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>DNA), the protocol most frequently used in Spain, or that request a second sample for repeat measurement of IRT followed by a DNA test (IRT<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>IRT<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>DNA), or a sweat test (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). It is important to remember that up to 30% of newborns with meconium ileus have normal IRT levels. The IRT+IRT strategy is applied in only a few autonomous communities.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">In Spain, there are 3 different strategies currently in use: IRT<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>IRT, IRT<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>IRT<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>DNA and IRT<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>IRT.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Genetic testing for cystic fibrosis</span><p id="par0045" class="elsevierStylePara elsevierViewall">Genetic testing is part of most protocols for neonatal screening for CF and is performed in samples with IRT levels exceeding the established threshold. In each population, the applied protocol should be adapted to its mutation spectrum.</p><p id="par0050" class="elsevierStylePara elsevierViewall">There are 4 possible outcomes of genetic testing in a newborn with elevated IRT:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">a)</span><p id="par0055" class="elsevierStylePara elsevierViewall">If the test detects 2 CF-causing mutations, the newborn should undergo a sweat test and DNA segregation analysis should be performed to confirm that each mutation comes from a different parent.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">b)</span><p id="par0060" class="elsevierStylePara elsevierViewall">In case of a single mutation, a sweat test should be performed. If the results of the sweat test are inconclusive, genetic testing should be expanded to attempt to identify a second mutation. If the sweat test is negative, the newborn is considered a carrier.</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">c)</span><p id="par0065" class="elsevierStylePara elsevierViewall">Cases where no mutation is found and the sweat test is normal are considered false positives of screening.</p></li></ul></p><p id="par0070" class="elsevierStylePara elsevierViewall">There is another group comprising those newborns in whom a conclusive diagnosis cannot be made, even with use of ancillary tests, for which the Working Group on CF of the European Cystic Fibrosis Society has proposed the label <span class="elsevierStyleItalic">cystic fibrosis screen positive, inconclusive diagnosis</span> (CFSPID).<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">10</span></a> This group includes:<ul class="elsevierStyleList" id="lis0010"><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">a)</span><p id="par0075" class="elsevierStylePara elsevierViewall">Newborns with a CF mutation in only one allele and intermediate sweat chloride values (30–59<span class="elsevierStyleHsp" style=""></span>mmol/L).</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">b)</span><p id="par0080" class="elsevierStylePara elsevierViewall">Newborns with a CFTR mutation in each allele, of which only 1 is classified as causing CF, and normal sweat chloride values (<30<span class="elsevierStyleHsp" style=""></span>mmol/L).</p></li></ul></p><p id="par0085" class="elsevierStylePara elsevierViewall">This group of patients requires ongoing follow-up, as in some cases CF is diagnosed at a later age due to onset of symptoms with elevation of sweat chloride levels.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Sweat chloride test</span><p id="par0090" class="elsevierStylePara elsevierViewall">The sweat test is the cornerstone of diagnosis of CF. Newborns with a positive neonatal screen test for CF must be referred for a sweat test exclusively to specialised CF units accredited for diagnosis.</p><p id="par0095" class="elsevierStylePara elsevierViewall">The sweat test is divided in 3 stages: (1) Stimulation by pilocarpine iontophoresis. (2) Collection of a sample over 30<span class="elsevierStyleHsp" style=""></span>min by either of the following methods: Macroduct collection system or the original Gibson and Cooke method (collection in pre-weighed filter paper or gauze). The sample should have a volume of at least 15<span class="elsevierStyleHsp" style=""></span>μL (Macroduct) or weigh at least 75<span class="elsevierStyleHsp" style=""></span>mg (gauze). (3) Chloride determination by coulometric titration with a chloridometer.<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">11</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">The diagnosis cannot be confirmed based exclusively on the sweat conductivity measurements obtained by the <span class="elsevierStyleItalic">nanoduc</span>t or <span class="elsevierStyleItalic">sweat check</span> systems (Macroduct). Samples must be analysed as soon as they are collected.</p><p id="par0105" class="elsevierStylePara elsevierViewall">The test result is classified as “normal” if the chloride concentration is less than 30<span class="elsevierStyleHsp" style=""></span>mmol/L; “intermediate” if it is between 30 and 59<span class="elsevierStyleHsp" style=""></span>mmol/L and “positive” if it is of 60<span class="elsevierStyleHsp" style=""></span>mmol/L or higher.<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">12</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">Patients with persistent intermediate values should undergo a diagnostic evaluation, including extended DNA testing and repetition of the sweat test at 6 months.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Appointment for reporting the results of neonatal screening</span><p id="par0115" class="elsevierStylePara elsevierViewall">Neonatal screening elicits a varying degree of anxiety in parents. Thus, it is advisable to report the results as soon as possible, preferably on the same day that testing is performed.</p><p id="par0120" class="elsevierStylePara elsevierViewall">If the newborn has CF, parents will be given information on all the tests performed to diagnose the disease and of the encouraging outcomes of new treatments and the extensive research that is underway, thanks to which it is now possible to have an optimistic perspective on the disease.</p><p id="par0125" class="elsevierStylePara elsevierViewall">At the end of the visit, if the newborn is not affected, parents will receive a report stating that the child does not have CF or that the child is a carrier and offering genetic counselling to the parents.<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">13</span></a></p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Initial appointment with a newborn with cystic fibrosis</span><p id="par0130" class="elsevierStylePara elsevierViewall">The diagnosis of CF through a neonatal screening test comes as a surprise to parents, since in most instances the newborn does not have any symptoms. For this reason, the psychosocial impact on the family of the new diagnosis must be managed with care.<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">14</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">The most important aspects to address are the following:<ul class="elsevierStyleList" id="lis0015"><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">•</span><p id="par0140" class="elsevierStylePara elsevierViewall">Provide an overview of the disease.</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">•</span><p id="par0145" class="elsevierStylePara elsevierViewall">Introduce the concept of the “care team”.</p></li><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">•</span><p id="par0150" class="elsevierStylePara elsevierViewall">Manage the expectations of the family.</p></li><li class="elsevierStyleListItem" id="lsti0045"><span class="elsevierStyleLabel">•</span><p id="par0155" class="elsevierStylePara elsevierViewall">Offer psychological support.</p></li></ul></p><p id="par0160" class="elsevierStylePara elsevierViewall">One of the key goals of the visit is to provide basic information in a positive light with a sensitive and empathic attitude and using simple language.<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">15</span></a></p><p id="par0165" class="elsevierStylePara elsevierViewall"><a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> specifies the actions to be taken during the first visit.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Initial management of the infant with cystic fibrosis</span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Gastrointestinal</span><p id="par0170" class="elsevierStylePara elsevierViewall">Follow-up by a paediatric gastroenterologist is recommended, with visits every 1–2 weeks immediately after diagnosis to ensure adequate nutrition and thereon every month for the first 3 months. If the patient is in good condition, subsequent visits may be held at 2- to 3-month intervals.</p><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Nutrition</span><p id="par0175" class="elsevierStylePara elsevierViewall">The main goal of nutritional interventions in CF is to promote normal growth and development for age (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>).</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0180" class="elsevierStylePara elsevierViewall">We recommend encouraging breastfeeding on demand. Whatever the feeding modality, patients with pancreatic insufficiency (PI) will receive pancreatic enzymes.<a class="elsevierStyleCrossRefs" href="#bib0270"><span class="elsevierStyleSup">16,17</span></a></p><p id="par0185" class="elsevierStylePara elsevierViewall">The introduction of complementary foods should conform to the guidelines for the general population, starting at age 6 months in exclusively breastfed infants and at age 4 to 6 months in formula-fed infants.<a class="elsevierStyleCrossRefs" href="#bib0280"><span class="elsevierStyleSup">18,19</span></a></p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Pancreatic and liver function</span><p id="par0190" class="elsevierStylePara elsevierViewall">As early as the neonatal period, children with CF may exhibit hypovitaminosis and PI.</p><p id="par0195" class="elsevierStylePara elsevierViewall">In case of clinical manifestations suggestive of PI or the presence of 2 mutations associated with PI, the patient should receive pancreatic enzyme replacement therapy and vitamin supplementation (<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>), and while treatment should not be delayed unduly, it is preferable and recommended to have the results of a faecal elastase test before starting treatment.</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0200" class="elsevierStylePara elsevierViewall">Different enzyme formulations are currently available (<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>). Pancreatic enzymes should be taken with every meal. Administration with water or acidic foods such as apple juice or sauce is recommended to prevent the microbeads from opening prematurely.<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">20</span></a> Routine supplementation with fat-soluble vitamins (A, D, E and, to a lesser degree, K) is indicated in all infants with PI (<a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a>).<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">16</span></a></p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia><elsevierMultimedia ident="tbl0025"></elsevierMultimedia><p id="par0205" class="elsevierStylePara elsevierViewall">These patients can experience liver impairment of a heterogeneous nature (<a class="elsevierStyleCrossRef" href="#tbl0030">Table 6</a>).</p><elsevierMultimedia ident="tbl0030"></elsevierMultimedia><p id="par0210" class="elsevierStylePara elsevierViewall">It is important to remember sodium chloride supplementation in hot weather.</p></span></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Respiratory management</span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Techniques to improve mucociliary clearance</span><p id="par0215" class="elsevierStylePara elsevierViewall">The dehydrated secretions of these patients hinder mucociliary clearance, giving rise to a vicious cycle of inflammation and chronic lung infection, which calls for early initiation of chest physical therapy.<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">21</span></a> There are several chest physical therapy techniques that can be applied in the paediatric age group, such as percussion, postural drainage and high-frequency chest compression. The evidence currently available is insufficient to establish which method is best.</p><p id="par0220" class="elsevierStylePara elsevierViewall">Treatment with recombinant human DNase reduces the viscosity of the mucus and facilitates mucociliary clearance. In children aged more than 6 years, administer 2.5<span class="elsevierStyleHsp" style=""></span>mL/day in nebulised form (following administration of a bronchodilator); in younger children, this treatment will only be considered in select cases.</p><p id="par0225" class="elsevierStylePara elsevierViewall">Hypertonic saline solution has an osmotic effect that restores fluid to the airway surfaces. Nebulisation of 4<span class="elsevierStyleHsp" style=""></span>mL of 7% hypertonic saline (following administration of a bronchodilator) in patients aged more than 6 years was found efficacious in improving lung function and pulmonary exacerbations.<a class="elsevierStyleCrossRefs" href="#bib0300"><span class="elsevierStyleSup">22,23</span></a></p></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Treatment of inflammation</span><p id="par0230" class="elsevierStylePara elsevierViewall">Azithromycin modulates cytokine production and plays an indirect role in biofilm formation. In cases where it is indicated (for instance, with persistent detection of <span class="elsevierStyleItalic">Pseudomonas</span> in culture), it should be given 3 times a week at the usual dose while monitoring liver function, taking into account that co-administration with inhaled tobramycin could impair the response to the latter.<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">24</span></a></p></span><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Respiratory infections</span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Prevention of infection</span><p id="par0235" class="elsevierStylePara elsevierViewall">Neonatal screening for CF provides an opportunity to educate the family and the child from a young age on the importance of hand hygiene in the household.<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">25</span></a></p><p id="par0240" class="elsevierStylePara elsevierViewall">In the clinic setting, it is recommended that waiting times are kept to a minimum and that the child wears a mask and plays with their own toys.<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">26</span></a> At present, there is absolute consensus that these patients should always be segregated. Vaccination according to the routine schedule is recommended, including vaccination against influenza (in children aged more than 6 months), while attendance to a child care centre is recommended against.</p></span><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Treatment of primary bacterial infections</span><p id="par0245" class="elsevierStylePara elsevierViewall">The presence of viscous secretions in the airways characteristic of these patients facilitates recurrent infections by different microorganisms. The most frequently involved pathogens are the following:</p><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Staphylococcus aureus</span><p id="par0250" class="elsevierStylePara elsevierViewall">(A) Methicillin-sensitive <span class="elsevierStyleItalic">S. aureus</span></p><p id="par0255" class="elsevierStylePara elsevierViewall">The current evidence is insufficient to recommend or discourage eradication of <span class="elsevierStyleItalic">S. aureus</span> or antibiotic prophylaxis.<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">27</span></a> The drugs used most frequently are amoxicillin-clavulanic acid, cefadroxil y trimethoprim-sulfamethoxazole (TMP-SMX) given orally for 14–21 days. Performance of a respiratory culture 1 week after completion of treatment is recommended (<a class="elsevierStyleCrossRef" href="#tbl0035">Table 7</a>).</p><elsevierMultimedia ident="tbl0035"></elsevierMultimedia><p id="par0260" class="elsevierStylePara elsevierViewall">(B) Methicillin-resistant <span class="elsevierStyleItalic">S. aureus</span></p><p id="par0265" class="elsevierStylePara elsevierViewall">In patients with no symptoms or mild symptoms: TMP-SMX, linezolid, 2–4 weeks.<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">28</span></a> In patients with severe symptoms, the recommended treatment is intravenous vancomycin or teicoplanin for 2–3 weeks. In case of chronic infection, one possible option is inhaled vancomycin for 2–3 weeks.</p></span><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Haemophilus influenzae</span><p id="par0270" class="elsevierStylePara elsevierViewall">If the patients have symptoms, the most frequently used drugs are amoxicillin-clavulanic acid and TMP-SMX (2–4 weeks).</p></span></span></span></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Pseudomonas aeruginosa</span><p id="par0275" class="elsevierStylePara elsevierViewall">Treatment for eradication must be initiated as soon as <span class="elsevierStyleItalic">P. aeruginosa</span> is detected for the first time. None of the eradication protocols published to date has proven superior to the others. Based on the Spanish consensus guidelines, the management of a first episode with isolation of <span class="elsevierStyleItalic">P. aeruginosa</span><a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">29</span></a> consist in:</p><p id="par0280" class="elsevierStylePara elsevierViewall">(1) <span class="elsevierStyleItalic">Asymptomatic patients</span>: oral ciprofloxacin for 2–4 weeks<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>nebulised antibiotics—colistin given daily (maximum 3–6 months) or 1–3 on-off cycles of tobramycin or aztreonam (maximum 6 months).</p><p id="par0285" class="elsevierStylePara elsevierViewall">(2) <span class="elsevierStyleItalic">Symptomatic patients</span>: systemic<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>inhaled antibiotherapy.</p><p id="par0290" class="elsevierStylePara elsevierViewall">- Mild: oral ciprofloxacin for 2–4 weeks.</p><p id="par0295" class="elsevierStylePara elsevierViewall">- Moderate–severe: intravenous ceftazidime<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>tobramycin (2–3 weeks).</p><p id="par0300" class="elsevierStylePara elsevierViewall">Performance of a respiratory culture is recommended 1 week after completion of treatment. If the culture is negative, we recommend maintenance of daily colistin (for a maximum of 3–6 months) or 1–3 on–off cycles of tobramycin or aztreonam (maximum 6 months).</p><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Treatment of pulmonary exacerbations</span><p id="par0305" class="elsevierStylePara elsevierViewall">A consensus definition of pulmonary exacerbation in infants and preschool-aged children with CF is necessary. Some groups have defined it as any change in the usual respiratory symptoms.</p><p id="par0310" class="elsevierStylePara elsevierViewall">The greater the number of exacerbations in the first 2 years of life, the more the disease progresses.<a class="elsevierStyleCrossRef" href="#bib0340"><span class="elsevierStyleSup">30</span></a> If case of respiratory infection, a respiratory culture must be ordered immediately along with initiation of antibiotherapy based on the age of the patient, the clinical presentation and previous microbiological results<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">15</span></a> for a duration of 2–4 weeks.</p><p id="par0315" class="elsevierStylePara elsevierViewall">If there is no improvement despite adequate treatment, it is possible that the infection is caused by microorganisms resistant to the prescribed antibiotics (in this case, assess the need for flexible bronchoscopy and bronchoalveolar lavage), or that other diseases are at play, such as gastro-oesophageal reflux, swallowing problems, etc.<a class="elsevierStyleCrossRefs" href="#bib0260"><span class="elsevierStyleSup">14,15,30</span></a> Chest physical therapy and adequate nutrition and hydration should never be neglected.</p></span></span><span id="sec0115" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Treatment of the underlying CFTR protein defect</span><p id="par0320" class="elsevierStylePara elsevierViewall">Treatments are currently available that target the underlying defect that causes the disease. These new therapies aim to improve CFTR function using compounds that act on the protein, which may be <span class="elsevierStyleItalic">correctors</span> (class <span class="elsevierStyleSmallCaps">II</span>) or <span class="elsevierStyleItalic">potentiators</span> (classes <span class="elsevierStyleSmallCaps">III</span> and <span class="elsevierStyleSmallCaps">IV</span>). Ivacaftor (Kalydeco<span class="elsevierStyleSup">®</span>) is a potentiator that has proven to be efficacious (improvements in lung function and body mass index, and reductions in the chloride concentration in sweat and of the number of exacerbations by up to 52%) and safe in patients with class III mutations.<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">31</span></a> The dosage consists of one 150<span class="elsevierStyleHsp" style=""></span>mg tablet every 12<span class="elsevierStyleHsp" style=""></span>h, to be ingested with fatty foods. In Spain, its used is approved starting at age 6 years, and in the United States starting at 12 months.</p><p id="par0325" class="elsevierStylePara elsevierViewall">The combination of a CFTR corrector and a CFTR potentiator, lumacaftor<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>ivacaftor (Orkambi<span class="elsevierStyleSup">®</span>), is a new strategy for treatment of patients homozygous for the F508del mutation (class <span class="elsevierStyleSmallCaps">II</span>) that has been shown to improve lung function, the frequency of exacerbations and quality of life.<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">32</span></a> The dosage consists of 2 tablets every 12<span class="elsevierStyleHsp" style=""></span>h. It is authorised for use starting at age 2 years in the United States, but is not yet available in Spain.</p></span><span id="sec0120" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Follow-up of the newborn with a diagnosis of cystic fibrosis through neonatal screening</span><span id="sec0125" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0145">Programmed visits and annual evaluation</span><p id="par0330" class="elsevierStylePara elsevierViewall"><a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a> shows the schedule of routine visits recommended in patients who are asymptomatic or clinically stable, although in practice it should be individualised based on the symptoms and the needs of the family.<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">14</span></a></p><p id="par0335" class="elsevierStylePara elsevierViewall">If something happens or questions arise, families should be able to contact the care team and be acquainted with the care protocol. Should there be any changes in symptoms suggestive of a pulmonary exacerbation, the care team should facilitate performance of a respiratory culture and determine whether empirical antibiotherapy is indicated.<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">33</span></a></p></span></span><span id="sec0130" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0150">Ancillary diagnostic tests</span><p id="par0340" class="elsevierStylePara elsevierViewall">A chest radiograph is currently recommended in case a complication is suspected or of moderate to severe pulmonary exacerbation.</p><p id="par0345" class="elsevierStylePara elsevierViewall">Chest computed tomography (CT) is the gold standard among imaging tests for the early detection of initial abnormalities (air trapping, bronchiectasis) and their progression, as it is more sensitive than chest radiography.</p><p id="par0350" class="elsevierStylePara elsevierViewall">Bronchiectasis can be found in up to 50% of CF patients by age 5 years.<a class="elsevierStyleCrossRefs" href="#bib0360"><span class="elsevierStyleSup">34,35</span></a> The CT protocol for assessment of CF should include inspiratory and expiratory views with low-dose radiation. Most hospitals perform a routine CT scan starting at age 2 years (without anaesthesia) and every 2 years thereafter at the time of the annual evaluation, while others do the initial scan at age 4 to 5 years with less frequent follow-up scans.</p><span id="sec0135" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0155">Lung function in early childhood</span><p id="par0355" class="elsevierStylePara elsevierViewall">Spirometry is the most commonly used technique to assess lung function. The forced expiratory volume in 1 second (FEV<span class="elsevierStyleInf">1</span>) is useful to define the presence of an exacerbation and the response to treatment. However, there is evidence that even in the presence of structural abnormalities, lung function may be normal in preschool-aged children.</p></span></span><span id="sec0140" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0160">Lung clearance index</span><p id="par0360" class="elsevierStylePara elsevierViewall">The lung clearance index is a new and very sensitive test that detects abnormalities earlier than spirometry and can be used to assess ventilation inhomogeneity.<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">36</span></a> At present it is used in clinical trials.<a class="elsevierStyleCrossRefs" href="#bib0375"><span class="elsevierStyleSup">37,38</span></a></p></span></span><span id="sec0145" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0165">Conclusions</span><p id="par0365" class="elsevierStylePara elsevierViewall">Neonatal screening for CF offers a unique opportunity for early diagnosis that has been proven to improve nutritional status, lung function and survival and to reduce hospital admissions, all of which may reduce health care costs. It is widely implemented worldwide and in Spain. Screening can only have beneficial effects if it is followed by strict adherence to the established standards of care, which is why we thought it was essential to publish these guidelines for the diagnosis and follow-up of patients with CF identified through neonatal screening.</p></span><span id="sec0150" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0170">Conflicts of interest</span><p id="par0370" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:12 [ 0 => array:3 [ "identificador" => "xres1173373" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1097424" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1173372" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1097425" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Benefits and risks of neonatal screening for cystic fibrosis" ] 6 => array:3 [ "identificador" => "sec0015" "titulo" => "Neonatal screening protocols in Spain" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Immunoreactive trypsinogen" ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "Genetic testing for cystic fibrosis" ] 2 => array:2 [ "identificador" => "sec0030" "titulo" => "Sweat chloride test" ] 3 => array:2 [ "identificador" => "sec0035" "titulo" => "Appointment for reporting the results of neonatal screening" ] ] ] 7 => array:2 [ "identificador" => "sec0040" "titulo" => "Initial appointment with a newborn with cystic fibrosis" ] 8 => array:3 [ "identificador" => "sec0045" "titulo" => "Initial management of the infant with cystic fibrosis" "secciones" => array:7 [ 0 => array:3 [ "identificador" => "sec0050" "titulo" => "Gastrointestinal" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0055" "titulo" => "Nutrition" ] 1 => array:2 [ "identificador" => "sec0060" "titulo" => "Pancreatic and liver function" ] ] ] 1 => array:3 [ "identificador" => "sec0065" "titulo" => "Respiratory management" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0070" "titulo" => "Techniques to improve mucociliary clearance" ] 1 => array:2 [ "identificador" => "sec0075" "titulo" => "Treatment of inflammation" ] 2 => array:3 [ "identificador" => "sec0080" "titulo" => "Respiratory infections" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0085" "titulo" => "Prevention of infection" ] 1 => array:3 [ "identificador" => "sec0090" "titulo" => "Treatment of primary bacterial infections" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0095" "titulo" => "Staphylococcus aureus" ] 1 => array:2 [ "identificador" => "sec0100" "titulo" => "Haemophilus influenzae" ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "sec0105" "titulo" => "Pseudomonas aeruginosa" "secciones" => array:1 [ 0 => array:2 [ "identificador" => "sec0110" "titulo" => "Treatment of pulmonary exacerbations" ] ] ] 3 => array:2 [ "identificador" => "sec0115" "titulo" => "Treatment of the underlying CFTR protein defect" ] 4 => array:3 [ "identificador" => "sec0120" "titulo" => "Follow-up of the newborn with a diagnosis of cystic fibrosis through neonatal screening" "secciones" => array:1 [ 0 => array:2 [ "identificador" => "sec0125" "titulo" => "Programmed visits and annual evaluation" ] ] ] 5 => array:3 [ "identificador" => "sec0130" "titulo" => "Ancillary diagnostic tests" "secciones" => array:1 [ 0 => array:2 [ "identificador" => "sec0135" "titulo" => "Lung function in early childhood" ] ] ] 6 => array:2 [ "identificador" => "sec0140" "titulo" => "Lung clearance index" ] ] ] 9 => array:2 [ "identificador" => "sec0145" "titulo" => "Conclusions" ] 10 => array:2 [ "identificador" => "sec0150" "titulo" => "Conflicts of interest" ] 11 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-09-29" "fechaAceptado" => "2018-11-14" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1097424" "palabras" => array:3 [ 0 => "Cystic fibrosis" 1 => "Newborn screening" 2 => "Guidelines for diagnosis and treatment" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1097425" "palabras" => array:3 [ 0 => "Fibrosis quística" 1 => "Cribado neonatal" 2 => "Protocolo de diagnóstico y seguimiento" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Newborn screening (NBS) for cystic fibrosis (CF) is well-established in many countries and provides the opportunity for an early diagnosis and treatment before the development of irreversible structural lung damage.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">In 1999, Catalonia, Castilla-León, and the Balearic Islands started the NBS programme for CF. In the last 10 years its implementation rapidly spread and all the autonomies offer the NBS programme for CF since 2015. There are many different strategies across Spain. It is believed that it is very opportune to have an updated and consensual guide for the diagnosis, follow-up, and treatment of patients diagnosed by neonatal screening.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">El diagnóstico de fibrosis quística (FQ) a través del cribado neonatal (CN) está bien establecido en muchos países y brinda la oportunidad de un diagnóstico y tratamiento temprano antes del desarrollo de daño estructural pulmonar irreversible.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">En 1999, Cataluña, Castilla-León y las Islas Baleares iniciaron el programa CN para FQ. En los últimos 10 años su implementación se extendió rápidamente y todas las autonomías ofrecen el programa CN para FQ desde 2015. Hay varias estrategias diferentes en toda España. Creemos que es muy oportuno contar con una guía actualizada y consensuada para el diagnóstico, el seguimiento y el tratamiento de los pacientes diagnosticados de FQ mediante CN.</p></span>" ] ] "NotaPie" => array:2 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0105">Please cite this article as: Gartner S, Mondéjar-López P, Asensio de la Cruz Ó, Grupo de Trabajo de Fibrosis Quística de la Sociedad Española de Neumología Pediátrica. Protocolo de seguimiento de pacientes con fibrosis quística diagnosticados por cribado neonatal. An Pediatr (Barc). 2019;152:251.</p>" ] 1 => array:3 [ "etiqueta" => "1" "nota" => "<p class="elsevierStyleNotepara" id="npar0110">The names of the authors of the Cystic Fibrosis Working Group of the Spanish Society of Pediatric Pulmonology are listed in Appendix A.</p>" "identificador" => "fn0005" ] ] "apendice" => array:1 [ 0 => array:1 [ "seccion" => array:1 [ 0 => array:4 [ "apendice" => "<p id="par0375" class="elsevierStylePara elsevierViewall">María José Alonso: Instituto de Biología y Genética Molecular (IBGM), Universidad de Valladolid, Valladolid.</p> <p id="par0380" class="elsevierStylePara elsevierViewall">Marina Álvarez: Paediatric Gastroenterology, Hepatology and Nutritional Support Unit, Hospital Universitari Vall d’Hebron, Barcelona.</p> <p id="par0385" class="elsevierStylePara elsevierViewall">Anselmo Andrés Martín: Department of Paediatric Pulmonology, Department and Clinical Management Unit of Paediatrics, Hospital Universitario Virgen Macarena, Seville.</p> <p id="par0390" class="elsevierStylePara elsevierViewall">María Isabel Barrio Gómez de Agüero: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario La Paz, Madrid</p> <p id="par0395" class="elsevierStylePara elsevierViewall">María Jesús Cabero Pérez: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Marqués de Valdecilla, Santander.</p> <p id="par0400" class="elsevierStylePara elsevierViewall">Pilar Caro Aguilera: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Materno-Infantil Universitario de Málaga, Malaga.</p> <p id="par0405" class="elsevierStylePara elsevierViewall">María Cols Roig: Paediatric Pulmonology and Cystic Fibrosis Unit, Department of Paediatrics, Hospital Sant Joan de Déu, Barcelona.</p> <p id="par0410" class="elsevierStylePara elsevierViewall">Isidoro Cortell Aznar-Pérez: Department of Paediatric Allergy and Pulmonology, Hospital Universitario La Fe, Valencia.</p> <p id="par0415" class="elsevierStylePara elsevierViewall">Jordi Costa Colomer: Paediatric Pulmonology and Cystic Fibrosis Unit, Department of Paediatrics, Hospital Sant Joan de Déu, Barcelona.</p> <p id="par0420" class="elsevierStylePara elsevierViewall">Isabel Delgado Pecellín: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Virgen del Rocío, Seville.</p> <p id="par0425" class="elsevierStylePara elsevierViewall">Amparo Escribano Montaner: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Clínico Universitario de Valencia, Universitat de València, Valencia.</p> <p id="par0430" class="elsevierStylePara elsevierViewall">Joan Figuerola Mulet: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Son Espases, Palma de Mallorca.</p> <p id="par0435" class="elsevierStylePara elsevierViewall">Gloria García Hernández: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario 12 de Octubre, Madrid.</p> <p id="par0440" class="elsevierStylePara elsevierViewall">Pilar Guayarte: Paediatric Gastroenterology Unit, Hospital Universitario Parc Taulí, Sabadell.</p> <p id="par0445" class="elsevierStylePara elsevierViewall">David Gil Ortega: Paediatric Gastroenterology, Hepatology and Nutrition Unit, Hospital Universitario Virgen de la Arrixaca, Murcia.</p> <p id="par0450" class="elsevierStylePara elsevierViewall">David Gómez Pastrana: Paediatric Pulmonology Unit, Hospital de Jerez, Jerez de la Frontera.</p> <p id="par0455" class="elsevierStylePara elsevierViewall">Adelaida Lamas Ferreiro: Cystic Fibrosis Unit, Hospital Universitario Ramón y Cajal, Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Madrid.</p> <p id="par0460" class="elsevierStylePara elsevierViewall">José Luis Marín Soria: Neonatal Screening Laboratory of Catalonia, Hospital Clinic, Barcelona.</p> <p id="par0465" class="elsevierStylePara elsevierViewall">Carlos Martín de Vicente: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Miguel Servet, Zaragoza.</p> <p id="par0470" class="elsevierStylePara elsevierViewall">Martín Navarro Merino: Department of Paediatric Pulmonology, Department and Clinical Management Unit of Paediatrics, Hospital Universitario Virgen Macarena, Seville.</p> <p id="par0475" class="elsevierStylePara elsevierViewall">Concepción Oliva Hernández: Hospital Universitario Nuestra Señora de la Candelaria, Santa Cruz de Tenerife.</p> <p id="par0480" class="elsevierStylePara elsevierViewall">Javier Pérez Frías: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Materno-Infantil Universitario de Málaga, Malaga.</p> <p id="par0485" class="elsevierStylePara elsevierViewall">Estela Pérez Ruiz: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Materno-Infantil Universitario de Málaga, Malaga.</p> <p id="par0490" class="elsevierStylePara elsevierViewall">Sandra Rovira Amigo: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Vall d’Hebron, Barcelona.</p> <p id="par0495" class="elsevierStylePara elsevierViewall">Antonio Salcedo Posadas: Cystic Fibrosis Unit, Hospital Universitario del Niño Jesús-Hospital Universitario Gregorio Marañón, Madrid.</p> <p id="par0500" class="elsevierStylePara elsevierViewall">Manuel Sánchez-Solís: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Virgen de la Arrixaca, Murcia.</p> <p id="par0505" class="elsevierStylePara elsevierViewall">Josep Sirvent Gómez: Paediatric Pulmonology Unit, Hospital Materno Infantil, Complexo Hospitalario Universitario A Coruña, A Coruña.</p> <p id="par0510" class="elsevierStylePara elsevierViewall">Carlos Vázquez Cordero: Paediatric Pulmonology and Cystic Fibrosis Unit, Hospital Universitario Cruces, Bilbao.</p> <p id="par0515" class="elsevierStylePara elsevierViewall">José Ramón Villa Asensi: Pulmonology and Cystic Fibrosis Unit, Hospital Universitario del Niño Jesús, Madrid.</p>" "etiqueta" => "Appendix A" "titulo" => "List of authors of the Working Group on Cystic Fibrosis of the Sociedad de Neumología Pediátrica (Spanish Sociology of Paediatric Pulmonology)" "identificador" => "sec0155" ] ] ] ] "multimedia" => array:8 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1374 "Ancho" => 2167 "Tamanyo" => 237176 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Protocols for diagnosis of cystic fibrosis through neonatal screening proposed by the European Best Practice Guidelines for Cystic Fibrosis Neonatal Screening<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">7</span></a> applied in Spain.</p>" ] ] 1 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">C, consider performing in this visit; LFT, lung function test; PERT, pancreatic enzyme replacement therapy; V, perform in any of these visits; X, perform in this visit.</p>" "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"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Age in months \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Diagnosis <a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">24–48<span class="elsevierStyleHsp" style=""></span>h<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">1</span></a> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">1<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">2 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">3 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">4 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">5 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">6 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">8 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">10 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">12 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Every 2–3 \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">24 \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " colspan="14" align="left" valign="top"><span class="elsevierStyleItalic">Care</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Discuss diagnosis<a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">c \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Breastfeeding \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Start PERT in patients with PI<a class="elsevierStyleCrossRef" href="#tblfn0020"><span class="elsevierStyleSup">d</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Salt supplementation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Start vitamins \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>History and physical with, anthropometric measurements \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Chest physical therapy<a class="elsevierStyleCrossRef" href="#tblfn0025"><span class="elsevierStyleSup">e</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Assess nutrient intake and dosage of enzymes in patients with PI \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">- Vaccinations<a class="elsevierStyleCrossRef" href="#tblfn0030"><span class="elsevierStyleSup">f</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="14" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="14" align="left" valign="top"><span class="elsevierStyleItalic">Diagnostic tests</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Sweat test \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X<a class="elsevierStyleCrossRef" href="#tblfn0035"><span class="elsevierStyleSup">g</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X<a class="elsevierStyleCrossRef" href="#tblfn0035"><span class="elsevierStyleSup">g</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Pancreatic function<a class="elsevierStyleCrossRef" href="#tblfn0040"><span class="elsevierStyleSup">h</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Respiratory culture<a class="elsevierStyleCrossRef" href="#tblfn0045"><span class="elsevierStyleSup">i</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Chest X-ray \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Chest CT<a class="elsevierStyleCrossRef" href="#tblfn0050"><span class="elsevierStyleSup">j</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Vitamin levels<a class="elsevierStyleCrossRef" href="#tblfn0055"><span class="elsevierStyleSup">k</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>CBC and chemistry panel<a class="elsevierStyleCrossRef" href="#tblfn0060"><span class="elsevierStyleSup">l</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Bronchoscopy<a class="elsevierStyleCrossRef" href="#tblfn0065"><span class="elsevierStyleSup">m</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>LFTs<a class="elsevierStyleCrossRef" href="#tblfn0070"><span class="elsevierStyleSup">n</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Faecal elastase \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X (SP) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Faecal fat \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Abdominal ultrasound \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="14" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Education</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Infection control \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Written information<a class="elsevierStyleCrossRef" href="#tblfn0075"><span class="elsevierStyleSup">o</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>CF register<a class="elsevierStyleCrossRef" href="#tblfn0080"><span class="elsevierStyleSup">p</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Genetic counselling \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">C \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Physical therapy \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Dietary habits \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">V \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2V<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">16</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Smoking \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">X \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2002695.png" ] ] ] "notaPie" => array:16 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Day of diagnosis (sweat test) and new appointment 24–48<span class="elsevierStyleHsp" style=""></span>h from diagnosis.</p>" ] 1 => array:3 [ "identificador" => "tblfn0010" "etiqueta" => "b" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Checkup 1 week after diagnosis or at age approximately 1 month.</p>" ] 2 => array:3 [ "identificador" => "tblfn0015" "etiqueta" => "c" "nota" => "<p class="elsevierStyleNotepara" id="npar0015">Explain how it was determined that the child has CF and provide details regarding the diagnosis, genetics and impact on other siblings and family members.</p>" ] 3 => array:3 [ "identificador" => "tblfn0020" "etiqueta" => "d" "nota" => "<p class="elsevierStyleNotepara" id="npar0020">Initiate PERT if patient has symptoms of malabsorption or a faecal elastase concentration <200<span class="elsevierStyleHsp" style=""></span>mcg/g or 2 CFTR mutations associated with PI.</p>" ] 4 => array:3 [ "identificador" => "tblfn0025" "etiqueta" => "e" "nota" => "<p class="elsevierStyleNotepara" id="npar0025">Train on techniques that facilitate mucociliary clearance: percussion and postural drainage. There is controversy as to which of the 2 is most appropriate in asymptomatic children.</p>" ] 5 => array:3 [ "identificador" => "tblfn0030" "etiqueta" => "f" "nota" => "<p class="elsevierStyleNotepara" id="npar0030">Coordination with routine primary care visits. Include vaccination against varicella, <span class="elsevierStyleItalic">S. pneumoniae</span> and rotavirus. Vaccinate children aged more than 6 months against influenza and administer palivizumab to infants aged less than 1 year.</p>" ] 6 => array:3 [ "identificador" => "tblfn0035" "etiqueta" => "g" "nota" => "<p class="elsevierStyleNotepara" id="npar0035">Sweat testing of all siblings.</p>" ] 7 => array:3 [ "identificador" => "tblfn0040" "etiqueta" => "h" "nota" => "<p class="elsevierStyleNotepara" id="npar0040">If PI is not present at the time of diagnosis, repeat the faecal elastase test at least twice in the first year of life and annually thereafter, as PI may develop at a later time.</p>" ] 8 => array:3 [ "identificador" => "tblfn0045" "etiqueta" => "i" "nota" => "<p class="elsevierStyleNotepara" id="npar0045">Consider more frequent performance of respiratory culture if the infant is symptomatic.</p>" ] 9 => array:3 [ "identificador" => "tblfn0050" "etiqueta" => "j" "nota" => "<p class="elsevierStyleNotepara" id="npar0050">Routine performance recommended from age 2 years in asymptomatic children, or before if the patient has symptoms. Expiratory CT scan with low-dose radiation and expiratory views to assess peripheral airway disease.</p>" ] 10 => array:3 [ "identificador" => "tblfn0055" "etiqueta" => "k" "nota" => "<p class="elsevierStyleNotepara" id="npar0055">Repeat at 1–2 months from initiation of supplementation. Measure more frequently in case of abnormal results.</p>" ] 11 => array:3 [ "identificador" => "tblfn0060" "etiqueta" => "l" "nota" => "<p class="elsevierStyleNotepara" id="npar0060">Complete blood count, electrolytes, urea, creatinine, albumin, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyl transferase, bilirubin, alkaline phosphatase.</p>" ] 12 => array:3 [ "identificador" => "tblfn0065" "etiqueta" => "m" "nota" => "<p class="elsevierStyleNotepara" id="npar0065">Some hospitals perform a bronchoalveolar lavage at approximately 3 months and 1 year of age for early detection of infection, inflammation markers or aspiration/reflux, and a bronchial biopsy for clinical investigation.</p>" ] 13 => array:3 [ "identificador" => "tblfn0070" "etiqueta" => "n" "nota" => "<p class="elsevierStyleNotepara" id="npar0070">Lung function tests in infants: consider performance if available in facility.</p>" ] 14 => array:3 [ "identificador" => "tblfn0075" "etiqueta" => "o" "nota" => "<p class="elsevierStyleNotepara" id="npar0075">Provide brochure with instructions on how and when to contact the CF unit (with a diary of symptoms and treatments prescribed or received outside routine visits).</p>" ] 15 => array:3 [ "identificador" => "tblfn0080" "etiqueta" => "p" "nota" => "<p class="elsevierStyleNotepara" id="npar0080">Obtained informed consent to include child in CF register.</p> <p class="elsevierStyleNotepara" id="npar0085"><span class="elsevierStyleItalic">Source</span>: Adapted from Borowitz et al.<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">14</span></a></p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Routine visit schedule.</p>" ] ] 2 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at2" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">BMI, body mass index calculated as weight (kg)/height square (m<span class="elsevierStyleSup">2</span>); <span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">10</span>, 10th percentile; <span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">50</span>, 50th percentile.</p>" "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"><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black"><2 years \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">2–18 years \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Optimal nutritional status: preventive nutritional counselling \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Weight for height ≥<span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">50</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">BMI<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">50</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Suboptimal status: dietary modification and oral nutrient supplementation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Weight for height <span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">10</span><span class="elsevierStyleHsp" style=""></span>−<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">50</span><br>Weight loss<br>Faltering growth \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">BMI<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">10</span><span class="elsevierStyleHsp" style=""></span>−<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">50</span><br>Weight loss in 2–4 months or absence of weight gain in 2 months \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Persistent malnutrition: enteral nutrition (nasogastric or gastrostomy tube) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Weight for height <span class="elsevierStyleItalic">a</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">10</span><br>Persistent weight loss or faltering growth despite oral supplementation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">BMI<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">P</span><span class="elsevierStyleInf">10</span><br>Drop of 2 percentile points despite oral supplementation \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2002697.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Guide for prescription of nutritional interventions<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">18</span></a></p>" ] ] 3 => array:8 [ "identificador" => "tbl0015" "etiqueta" => "Table 3" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at3" "detalle" => "Table " "rol" => "short" ] ] "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"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Infants 0–12 months \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">2000–4000<span class="elsevierStyleHsp" style=""></span>IU lipase/120<span class="elsevierStyleHsp" style=""></span>mL or per breastfeeding episode \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Children 12 months to 4 years \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1000<span class="elsevierStyleHsp" style=""></span>IU lipase/kg per feeding,<a class="elsevierStyleCrossRef" href="#tblfn0085"><span class="elsevierStyleSup">a</span></a> maximum 2500<span class="elsevierStyleHsp" style=""></span>IU/kg/day or 10<span class="elsevierStyleHsp" style=""></span>000<span class="elsevierStyleHsp" style=""></span>IU/kg/day or 4000 UI/g fat in diet \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Children >4 years through adulthood \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">500<span class="elsevierStyleHsp" style=""></span>IU lipase/kg per feeding,<a class="elsevierStyleCrossRef" href="#tblfn0085"><span class="elsevierStyleSup">a</span></a> maximum 2.500<span class="elsevierStyleHsp" style=""></span>IU/kg/day or 10<span class="elsevierStyleHsp" style=""></span>000<span class="elsevierStyleHsp" style=""></span>IU/kg/day or 4000<span class="elsevierStyleHsp" style=""></span>IU/g fat in diet \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2002698.png" ] ] ] "notaPie" => array:1 [ 0 => array:3 [ "identificador" => "tblfn0085" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0090">A half dose is recommended with snacks.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Recommendations for pancreatic enzyme replacement therapy in children with pancreatic insufficiency.</p>" ] ] 4 => array:8 [ "identificador" => "tbl0020" "etiqueta" => "Table 4" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at4" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "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"><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Kreon 5000<span class="elsevierStyleSup">®</span> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Kreon 10<span class="elsevierStyleHsp" style=""></span>000<span class="elsevierStyleSup">®</span> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Kreon 25<span class="elsevierStyleHsp" style=""></span>000<span class="elsevierStyleSup">®</span> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Kreon 40<span class="elsevierStyleHsp" style=""></span>000<span class="elsevierStyleSup">®</span> \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Lipase activity (units) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">5000 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">10<span class="elsevierStyleHsp" style=""></span>000 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">25<span class="elsevierStyleHsp" style=""></span>000 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">40<span class="elsevierStyleHsp" style=""></span>000 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Amylase activity (units) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">4000 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">8000 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">18<span class="elsevierStyleHsp" style=""></span>000 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">25<span class="elsevierStyleHsp" style=""></span>000 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Protease activity (units) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">300 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">600 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1000 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1600 \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2002696.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Enzyme replacement formulations currently available in Spain.</p>" ] ] 5 => array:8 [ "identificador" => "tbl0025" "etiqueta" => "Table 5" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at5" "detalle" => "Table " "rol" => "short" ] ] "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"><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Recommended daily dose<a class="elsevierStyleCrossRef" href="#tblfn0090"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Monitoring \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Precautions \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Vitamin A \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1500<span class="elsevierStyleHsp" style=""></span>IU \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Serum value/RBP 0.8–2.0<br>Normal beta carotene levels \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Precaution in patients with renal insufficiency, osteopenia, liver disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Vitamin E \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">40–50<span class="elsevierStyleHsp" style=""></span>IU \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Serum vitamin E >30 mcg/mL<br>Vitamin E/total lipids: >0.8<span class="elsevierStyleHsp" style=""></span>mg/g \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">May exacerbate coagulopathy secondary to vitamin K deficiency \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Vitamin D \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">400–800<span class="elsevierStyleHsp" style=""></span>IU \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">25-OH vitamin D<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>30<span class="elsevierStyleHsp" style=""></span>ng/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Abnormally low levels in patients with pancreatic sufficiency \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Vitamin K \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0.3–1<span class="elsevierStyleHsp" style=""></span>mg \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PIVKA-<span class="elsevierStyleSmallCaps">II</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>2<span class="elsevierStyleHsp" style=""></span>ng/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Dosage not established. Recommend especially in case of cholestasis, liver disease, intestinal resection, antibiotherapy, haemoptysis, osteopenia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sodium chloride \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2<span class="elsevierStyleHsp" style=""></span>mEq/kg \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Consider in all patients (with or without pancreatic insufficiency). Increase dose in case of hot weather, intense exercise or fluid loss (fever, ileostomy, diarrhoea) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2002700.png" ] ] ] "notaPie" => array:1 [ 0 => array:3 [ "identificador" => "tblfn0090" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0095">Based on the recommendations of the European Cystic Fibrosis Society.<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">16</span></a></p> <p class="elsevierStyleNotepara" id="npar0100">Serum retinol (mg/dL)/retinol binding protein (RBP) (mg/dL)<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>0.0734.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Recommended doses of fat-soluble vitamins and minerals in infants aged less than 1 year with pancreatic insufficiency.</p>" ] ] 6 => array:8 [ "identificador" => "tbl0030" "etiqueta" => "Table 6" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at6" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "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"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Manifestation \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Relative frequency as % \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Isolated abnormalities in blood tests \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Focal biliary cirrhosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">20–30 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Multilobular biliary cirrhosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Portal hypertension \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2–5 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Neonatal cholestasis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><10 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sclerosing cholangitis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Frequently silent \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Biliary microlithiasis/biliary sludge \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">30 \t\t\t\t\t\t\n