Techniques and Procedures
Nasal Potential Difference Test to Diagnose Cystic Fibrosis
C. Domingo-Ribasa,
, M. Bosque-Garcíab
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cdomingo@cspt.es
Correspondence: Dr. C. Domingo-Ribas. Servicio de Neumología. Corporació Parc Taulí. Parc Taulí, s/n. 08208 Sabadell. Barcelona. España
Correspondence: Dr. C. Domingo-Ribas. Servicio de Neumología. Corporació Parc Taulí. Parc Taulí, s/n. 08208 Sabadell. Barcelona. España
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Cardona, I. Amat" "autores" => array:2 [ 0 => array:3 [ "preGrado" => "Dr." "nombre" => "P.J." "apellidos" => "Cardona" ] 1 => array:2 [ "nombre" => "I." "apellidos" => "Amat" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1579212906601109?idApp=UINPBA00003Z" "url" => "/15792129/0000004200000001/v1_201305150227/S1579212906601109/v1_201305150227/en/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Techniques and Procedures</span>" "titulo" => "Nasal Potential Difference Test to Diagnose Cystic Fibrosis" "tieneTextoCompleto" => 0 "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "33" "paginaFinal" => "38" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "C. Domingo-Ribas, M. Bosque-García" "autores" => array:2 [ 0 => array:5 [ "preGrado" => "Dr." "nombre" => "C." "apellidos" => "Domingo-Ribas" "email" => array:1 [ 0 => "cdomingo@cspt.es" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff1" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor1" ] ] ] 1 => array:3 [ "nombre" => "M." "apellidos" => "Bosque-García" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff2" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Neumología, Corporació Parc Taulí-Universidad Autónoma de Barcelona, Sabadell, Barcelona, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff1" ] 1 => array:3 [ "entidad" => "Unidad de Neumología, Servicio de Pediatría, Corporació Parc Taulí-Universidad Autónoma de Barcelona, Sabadell, Barcelona, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff2" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor1" "etiqueta" => "*" "correspondencia" => "Correspondence: Dr. C. Domingo-Ribas. Servicio de Neumología. Corporació Parc Taulí. Parc Taulí, s/n. 08208 Sabadell. Barcelona. España" ] ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2005-01-14" "fechaAceptado" => "2005-03-01" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key words" "identificador" => "xpalclavsec154179" "palabras" => array:3 [ 0 => "Cystic fibrosis" 1 => "Diagnosis" 2 => "Nasal potential difference" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec154178" "palabras" => array:3 [ 0 => "Fibrosis quística" 1 => "Diagnóstico" 2 => "Diferencia de potencial nasal" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:1 [ "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">Cystic fibrosis is usually diagnosed based on suspicion arising from a typical clinical picture and must be confirmed by either a finding of high chloride concentrations in sweat tests on 2 separate days or detection of 2 gene mutations. The nasal potential difference (NPD) test has been proposed to provide evidence of abnormal function of the cystic fibrosis transmembrane conductance regulator (CFTR), a receptor that forms a chloride ion channel. The test is especially useful for patients who have normal chloride concentrations in sweat tests and in whom 2 gene mutations related to cystic fibrosis have not been detected. The NPD test requires 2 electrodes connected to a voltmeter (a Tholy-Medicap® device). One is placed on the nasal mucosa of the inferior turbinate and the other is placed subcutaneously on the forearm. A reading less than —40 mV is considered abnormal, as values under that cut point are never found in healthy individuals. Two abnormal NPD findings on separate days are required for a diagnosis of CFTR dysfunction. False negatives arise when the integrity of the epithelium is altered. After application of amiloride, NPD decreases more markedly in cystic fibrosis patients than in healthy individuals and applying isoproterenol or fenoterol after amiloride provokes no response in patients with the genetic defect that prevents chloride ion channel activation.</p>" ] "es" => array:1 [ "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">En la gran mayoría de los pacientes con fibrosis quística (FQ), el diagnóstico se sospecha por unos síntomas clínicos típicos y debe confirmarse mediante la determinación en su-dor de una concentración de cloro elevada en 2 días separa-dos o mediante la identificación de 2 mutaciones en un estu-dio genético. Para evidenciar el anormal comportamiento de la proteína de membrana CFTR (<span class="elsevierStyleItalic">cystic fibrosis transmembrane conductance regulator</span>), encargada del transporte de cloro, se ha ideado la prueba de la diferencia de potencial nasal (DPN), especialmente útil en pacientes con concentra-ciones de cloro normales y en los que no se identifican las 2 mutaciones del gen de la FQ. Para la realización de la DPN se requieren 2 electrodos conectados a un voltímetro (dispo-sitivo de medida Tholy-Medicap®), uno colocado sobre la mucosa nasal del cornete inferior, y otro en el tejido celular subcutáneo del antebrazo. Un valor inferior a —40 mV se considera patológico. Los valores obtenidos en sujetos sanos no sobrepasan nunca este valor. Se precisan 2 determinacio-nes anormales de DPN registradas en 2 días separados para aceptar la disfunción de la CFTR. Pueden observarse falsos negativos cuando la integridad del epitelio está alterada. En la FQ, tras la aplicación de amilorida la diferencia de potencial se reduce de modo más llamativo que en sanos, y la aplicación de isoproterenol o fenoterol después de amilorida no provoca respuesta debido al defecto genético que impide la activación de los canales de cloro.</p>" ] ] "NotaPie" => array:1 [ 0 => array:1 [ "nota" => "<p class="elsevierStyleNotepara">This study was funded through an Òscar Ravà grant from the Catalan Foundation of Pneumology (FUCAP), 1999.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "REFERENCES" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:18 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Discinesia ciliar primaria" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "C Domingo Ribas" 1 => "J Roig Cutillas" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Med Clin (Barc)" "fecha" => "1991" "volumen" => "97" "paginaInicial" => "144" "paginaFinal" => "146" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib2" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "What is cystic fibrosis diagnosis?" 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 2 | 2 | 4 |
| 2024 October | 34 | 22 | 56 |
| 2024 September | 40 | 21 | 61 |
| 2024 August | 54 | 38 | 92 |
| 2024 July | 33 | 23 | 56 |
| 2024 June | 32 | 28 | 60 |
| 2024 May | 34 | 28 | 62 |
| 2024 April | 21 | 29 | 50 |
| 2024 March | 24 | 18 | 42 |
| 2024 February | 29 | 25 | 54 |
| 2023 March | 1 | 1 | 2 |
| 2023 February | 29 | 25 | 54 |
| 2023 January | 18 | 36 | 54 |
| 2022 December | 26 | 34 | 60 |
| 2022 November | 30 | 22 | 52 |
| 2022 October | 19 | 38 | 57 |
| 2022 September | 24 | 27 | 51 |
| 2022 August | 22 | 28 | 50 |
| 2022 July | 17 | 43 | 60 |
| 2022 June | 15 | 35 | 50 |
| 2022 May | 17 | 30 | 47 |
| 2022 April | 15 | 27 | 42 |
| 2022 March | 17 | 32 | 49 |
| 2022 February | 18 | 31 | 49 |
| 2022 January | 16 | 34 | 50 |
| 2021 December | 13 | 39 | 52 |
| 2021 November | 22 | 38 | 60 |
| 2021 October | 20 | 43 | 63 |
| 2021 September | 21 | 53 | 74 |
| 2021 August | 13 | 32 | 45 |
| 2021 July | 17 | 25 | 42 |
| 2021 June | 19 | 36 | 55 |
| 2021 May | 35 | 27 | 62 |
| 2021 April | 60 | 21 | 81 |
| 2021 March | 44 | 16 | 60 |
| 2021 February | 17 | 12 | 29 |
| 2021 January | 20 | 12 | 32 |
| 2020 December | 11 | 12 | 23 |
| 2020 November | 18 | 15 | 33 |
| 2020 October | 8 | 6 | 14 |
| 2020 September | 4 | 6 | 10 |
| 2020 August | 18 | 10 | 28 |
| 2020 July | 14 | 13 | 27 |
| 2020 June | 6 | 4 | 10 |
| 2020 May | 15 | 19 | 34 |
| 2020 April | 14 | 11 | 25 |
| 2020 March | 15 | 11 | 26 |
| 2020 February | 16 | 8 | 24 |
| 2020 January | 19 | 16 | 35 |
| 2019 December | 20 | 30 | 50 |
| 2019 November | 6 | 18 | 24 |
| 2019 October | 6 | 28 | 34 |
| 2019 September | 14 | 35 | 49 |
| 2019 August | 10 | 28 | 38 |
| 2019 July | 13 | 38 | 51 |
| 2019 June | 12 | 23 | 35 |
| 2019 May | 21 | 29 | 50 |
| 2019 April | 27 | 48 | 75 |
| 2019 March | 17 | 37 | 54 |
| 2019 February | 13 | 20 | 33 |
| 2019 January | 4 | 30 | 34 |
| 2018 December | 8 | 32 | 40 |
| 2018 November | 27 | 26 | 53 |
| 2018 October | 20 | 36 | 56 |
| 2018 September | 12 | 43 | 55 |
| 2018 May | 6 | 0 | 6 |
| 2018 April | 8 | 8 | 16 |
| 2018 March | 4 | 3 | 7 |
| 2018 February | 5 | 7 | 12 |
| 2018 January | 2 | 9 | 11 |
| 2017 December | 4 | 14 | 18 |
| 2017 November | 8 | 20 | 28 |
| 2017 October | 4 | 26 | 30 |
| 2017 September | 3 | 13 | 16 |
| 2017 August | 8 | 22 | 30 |
| 2017 July | 6 | 26 | 32 |
| 2017 June | 16 | 32 | 48 |
| 2017 May | 9 | 25 | 34 |
| 2017 April | 2 | 23 | 25 |
| 2017 March | 8 | 27 | 35 |
| 2017 February | 4 | 17 | 21 |
| 2017 January | 5 | 11 | 16 |
| 2016 December | 17 | 8 | 25 |
| 2016 November | 13 | 9 | 22 |
| 2016 October | 19 | 20 | 39 |
| 2016 September | 13 | 12 | 25 |
| 2016 August | 9 | 5 | 14 |
| 2016 July | 12 | 5 | 17 |
| 2016 March | 2 | 0 | 2 |
| 2015 December | 2 | 0 | 2 |
| 2015 October | 175 | 1 | 176 |
| 2015 September | 162 | 14 | 176 |
| 2015 August | 132 | 16 | 148 |
| 2015 July | 159 | 17 | 176 |
| 2015 June | 153 | 16 | 169 |
| 2015 May | 173 | 25 | 198 |
| 2015 April | 154 | 14 | 168 |
| 2015 March | 145 | 13 | 158 |
| 2015 February | 133 | 9 | 142 |
| 2015 January | 111 | 9 | 120 |
| 2014 December | 182 | 6 | 188 |
| 2014 November | 217 | 11 | 228 |
| 2014 October | 235 | 21 | 256 |
| 2014 September | 241 | 15 | 256 |
| 2014 August | 172 | 17 | 189 |
| 2014 July | 200 | 13 | 213 |
| 2014 June | 208 | 18 | 226 |
| 2014 May | 196 | 17 | 213 |
| 2014 April | 259 | 14 | 273 |
| 2014 March | 201 | 18 | 219 |
| 2014 February | 178 | 13 | 191 |
| 2014 January | 146 | 14 | 160 |
| 2013 December | 123 | 16 | 139 |
| 2013 November | 155 | 11 | 166 |
| 2013 October | 128 | 20 | 148 |
| 2013 September | 134 | 14 | 148 |
| 2013 August | 96 | 23 | 119 |
| 2013 July | 94 | 18 | 112 |
| 2013 June | 102 | 19 | 121 |
| 2013 May | 94 | 12 | 106 |
| 2013 April | 80 | 14 | 94 |
| 2013 March | 21 | 5 | 26 |
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