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Un estudio de casos y controles" ] ] "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" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 700 "Ancho" => 1675 "Tamanyo" => 91752 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Receiver operating characteristic curve. (A) Receiver operating characteristic curve shows the specificity and sensitivity of inflammatory markers (CRP, IL-6, and TNF) and HbA1c for diabetic foot prediction. CRP, C-reactive protein; TNF, tumor necrosis factor; IL-6, interleukin 6; HbA1c, glycated hemoglobin. (B) ROC curve for model performance.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Amal Ahmed Mohamed, Mona Abd Elmotaleb Hussein, Ihab Nabil Hanna, Abdulqadir Jeprel Japer Nashwan, Mohamed Saleh, Wafaa Yousif Abdel Wahed, Asmaa Mohamed Mohamed Mansour, Mohamed Ramadan Ezz Al Arab, Naglaa Fawzy, Yasser Sakr, Hassan Shalby, Eman AlHussain, Marwa Kamal Darwish, Heba El-Osaily, Mervat Naguib, Ahmed Ali Mohamed, Waleed Farouk Mohamed, Wael Hafez" "autores" => array:18 [ 0 => array:2 [ "nombre" => "Amal Ahmed" "apellidos" => "Mohamed" ] 1 => array:2 [ "nombre" => "Mona Abd" "apellidos" => "Elmotaleb Hussein" ] 2 => array:2 [ "nombre" => "Ihab" "apellidos" => "Nabil Hanna" ] 3 => array:2 [ "nombre" => "Abdulqadir Jeprel" "apellidos" => "Japer Nashwan" ] 4 => array:2 [ "nombre" => "Mohamed" "apellidos" => "Saleh" ] 5 => array:2 [ "nombre" => "Wafaa Yousif" "apellidos" => "Abdel Wahed" ] 6 => array:2 [ "nombre" => "Asmaa Mohamed" "apellidos" => "Mohamed Mansour" ] 7 => array:2 [ "nombre" => "Mohamed Ramadan" "apellidos" => "Ezz Al Arab" ] 8 => array:2 [ "nombre" => "Naglaa" "apellidos" => "Fawzy" ] 9 => array:2 [ "nombre" => "Yasser" "apellidos" => "Sakr" ] 10 => array:2 [ "nombre" => "Hassan" "apellidos" => "Shalby" ] 11 => array:2 [ "nombre" => "Eman" "apellidos" => "AlHussain" ] 12 => array:2 [ "nombre" => "Marwa" "apellidos" => "Kamal Darwish" ] 13 => array:2 [ "nombre" => "Heba" "apellidos" => "El-Osaily" ] 14 => array:2 [ "nombre" => "Mervat" "apellidos" => "Naguib" ] 15 => array:2 [ "nombre" => "Ahmed Ali" "apellidos" => "Mohamed" ] 16 => array:2 [ "nombre" => "Waleed" "apellidos" => "Farouk Mohamed" ] 17 => array:2 [ "nombre" => "Wael" "apellidos" => "Hafez" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775324000812?idApp=UINPBA00004N" "url" => "/00257753/0000016200000009/v1_202405080526/S0025775324000812/v1_202405080526/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S0025775323007029" "issn" => "00257753" "doi" => "10.1016/j.medcli.2023.09.029" "estado" => "S300" "fechaPublicacion" => "2024-05-17" "aid" => "6488" "copyright" => "Elsevier España, S.L.U." 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"apellidos" => "Valle" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">f</span>" "identificador" => "aff0030" ] ] ] 11 => array:3 [ "nombre" => "Carlos Casasnovas" "apellidos" => "Pons" "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">g</span>" "identificador" => "aff0035" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">i</span>" "identificador" => "aff0045" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">j</span>" "identificador" => "aff0050" ] ] ] 12 => array:3 [ "nombre" => "Arturo Fraga" "apellidos" => "Bau" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">e</span>" "identificador" => "aff0025" ] ] ] 13 => array:3 [ "nombre" => "Eugenia Cisneros" "apellidos" => "Barroso" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 14 => array:3 [ "nombre" => "Inés Losada" "apellidos" => "López" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 15 => array:4 [ "nombre" => "Juan" "apellidos" => "González-Moreno" "email" => array:1 [ 0 => "jgonzalez4@hsll.es" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:10 [ 0 => array:3 [ "entidad" => "Servicio de Medicina Interna, Unidad Amiloidosis por Trastirretina, Hospital Universitario Son Llàtzer, Instituto de Investigación Sanitaria Illes Balears (idISBA), Palma de Mallorca, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neurología, Unidad de Neuromuscular, IdISSC, Hospital Clínico San Carlos, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitari i Politècnic La Fe/IISLAFE, Universitat de Valencia, CIBERER (ERN EURO-NMD), Valencia, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Servicio de Medicina Interna, UMAH, Hospital Universitario Juan Ramón Jiménez, Huelva, Spain" "etiqueta" => "d" "identificador" => "aff0020" ] 4 => array:3 [ "entidad" => "Servicio de Medicina Interna, Hospital Álvaro Cunqueiro, Vigo, Spain" "etiqueta" => "e" "identificador" => "aff0025" ] 5 => array:3 [ "entidad" => "Servicio de Medicina Interna, Hospital Vall d’Hebron, Barcelona, Spain" "etiqueta" => "f" "identificador" => "aff0030" ] 6 => array:3 [ "entidad" => "Neuromuscular Unit, Neurology Department, Bellvitge University Hospital-IDIBELL, Spain" "etiqueta" => "g" "identificador" => "aff0035" ] 7 => array:3 [ "entidad" => "Servicio de Medicina Interna, Hospital Ribera-Povisa, Vigo, Spain" "etiqueta" => "h" "identificador" => "aff0040" ] 8 => array:3 [ "entidad" => "Multidisciplinary Unit of Familial Amyloidosis, Bellvitge University Hospital-IDIBELL, Neurometabolic Diseases Group, Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain" "etiqueta" => "i" "identificador" => "aff0045" ] 9 => array:3 [ "entidad" => "Biomedical Research Network Center in Rare Diseases (CIBERER), Valencia, Spain" "etiqueta" => "j" "identificador" => "aff0050" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Experiencia en vida real en España del uso de tafamidis para el tratamiento de pacientes con amiloidosis por transtirretina VAL30MET con polineuropatía" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1523 "Ancho" => 1508 "Tamanyo" => 83408 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">ROC curve for NIS and treatment response. NIS: lower neuropathy impairment score.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Variant transthyretin amyloidosis (A-ATTRv) is a rare systemic autosomal dominant neurodegenerative disease.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">1</span></a> More than 140 pathogenic variants in the transthyretin (TTR) gene have been described,<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">1</span></a> the most common being V30M, which is also the most prevalent in Spain where two major endemic foci have been identified, Valverde del Camino (Huelva) and Mallorca.<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">2</span></a> A-ATTRV30M usually manifests as a rapidly progressive axonal polyneuropathy that is long-dependent, typically accompanied by autonomic nervous system dysfunction.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Liver transplantation was the first effective treatment to halt neuropathy progression in V30M A-ATTR patients.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">3</span></a> However, the high peri-procedure morbidity and mortality led to the development of antiamyloidogenic drugs for A-ATTRv. Tafamidis, a safe orally bioavailable TTR stabiliser, became the first specific drug approved for A-ATTR related polyneuropathy in Coutinho stage (FAP) I in 2011.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">4</span></a> Since then, three other treatment options (all TTR liver synthesis silencers) have been accepted for use in A-ATTRv.<a class="elsevierStyleCrossRefs" href="#bib0170"><span class="elsevierStyleSup">5,6</span></a> Thus, the natural history of the disease has entirely changed.<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">7</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The efficacy of tafamidis in A-ATTRV30M with polyneuropathy was largely evaluated in both the pivotal clinical trial<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">8</span></a> and the long-term extension studies.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">9</span></a> These studies, which had a follow-up period of 6 years, demonstrated that tafamidis was correlated with reduced neurological decline and maintained an excellent safety profile.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">9</span></a> Additionally, tafamidis was linked to improved survival rates.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">9</span></a> Furthermore, an open-label single-arm trial in Japanese patients with predominantly V30M A-ATTRv showed the effectiveness of tafamidis.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">10</span></a> Additional post hoc analysis of the key clinical trials have been published,<a class="elsevierStyleCrossRefs" href="#bib0200"><span class="elsevierStyleSup">11,12</span></a> but there is limited real-life experience with tafamidis in V30M A-ATTR-related polyneuropathy, particularly outside of Portugal.<a class="elsevierStyleCrossRefs" href="#bib0210"><span class="elsevierStyleSup">13,14</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The aim of this study is to analyse the effectiveness of tafamidis in a real-world setting for patients with V30M A-ATTR-related polineuropathy treated at the main referral centres for the disease in Spain.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Material and methods</span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Study population</span><p id="par0025" class="elsevierStylePara elsevierViewall">Patients with V30M A-ATTR amyloidosis treated with tafamidis meglumine (20<span class="elsevierStyleHsp" style=""></span>mg once daily) for at least 1 year (as suggested elsewhere<a class="elsevierStyleCrossRefs" href="#bib0215"><span class="elsevierStyleSup">14,15</span></a> between January 2012 and December 2022 in 7 centres in Spain, including endemic and non endemic areas, were included. All patients were older than 18 years and diagnosed with Coutinho stage I of the V30M A-ATTR-related polyneuropathy based on the presence of the V30M TTR variant and symptomatic, sensory, sensorimotor and/or autonomic neuropathy. Sensory and sensorimotor polyneuropathy (PNP) was defined by clinical and electrophysiological studies. Autonomic neuropathy was diagnosed clinically with or without specific autonomic test. Clinical phenotype was defined as previously: early-onset (when disease starts before 50 years-old), late-onset (after 50 years-old) and mixed (if A-ATTR related cardiomyopathy is also present). Patients were excluded if they had been diagnosed with: multiple myeloma, non-TTR amyloidosis, lymphoma, leukaemia or macroglobulinemia. Patients were also excluded if they started tafamidis with basal Coutinho stage<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>I. Patients were assessed at baseline, when treatment was initiated, and every 6 months thereafter.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The study was conducted in accordance with the standards of the Good Clinical Practice and Guidelines of the International Conference on Harmonisation (ICH). The study obtained the approval of the research commission of the Son Llàtzer University Hospital and the Ethics Committee of the Balearic Islands (IB 4760/21 EOm). All data were anonymised and treated confidentially in accordance with current legislation on the protection of personal data.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Outcome measurements</span><p id="par0035" class="elsevierStylePara elsevierViewall">A-ATTR-PN was assessed at baseline and during follow-up using the following outcome measures: Coutinho (FAP) stage (ambulatory A-ATTRv-PNP stage: stage I; fully ambulant; stage II, need of 1 or 2 walking aids; stage III, wheelchair-bound or bedridden) PND stage (ambulatory A-ATTRv-PNP stage: stage I, fully ambulant without difficulty; stage II, fully ambulant with some walking impairment; stage IIIa, of 1 walking aid needed; stage IIIb, 2 walking aids needed); stage IV, wheelchair-bound or bedridden), Norfolk QoL (47-item PNP related QoL questionnaire, ranging from −2, best QoL, to 138, worst QoL), NIS (Neuropathy Impairment Score, summed scores of standard items of the neuromuscular examination of weakness, reflex loss, and sensation loss, ranging from 0, normal examination, to 244), ESC (electrochemical skin conductance) measured by Sudoscan® (non-invasive quantitative electrophysiological that assess ESC by the application of a pulsating direct current on the skin) and nerve conduction studies (NCS). ESC was expressed here as the sum of hand and feet ESC. SNAP of the ulnar and sural nerves and CMAP of the ulnar and peroneal nerves were measured as suggested elsewhere.<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">16</span></a> Average SNAPs and CMAPs were calculated.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Other measurements</span><p id="par0040" class="elsevierStylePara elsevierViewall">Clinical and demographic variables were also collected. These included age and sex, maternal/paternal inheritance, age at diagnosis, clinical phenotype (early onset, late onset A-ATTR-PN, mixed phenotype), date of tafamidis initiation and laboratory measurements (glomerular filtration rate, albumin, TTR, retinol binding protein).</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Responder classification</span><p id="par0045" class="elsevierStylePara elsevierViewall">Responder classification was considered according to that previously published by Monteiro et al. [B]. Thus, patients were classified as responders if they showed no progression of sensory, autonomic and/or motor neuropathic symptoms and signs, considering a stable NIS, if the change in total score was less than 2 points/year?; as non-responders if they showed a continuous and rapid worsening of sensory, motor and/or autonomic neuropathy not different from the expected progression without therapy, with an annual increase in NIS of more than 10 points; and as partial responders if they were not classified as responders or non-responders, with no change in dysautonomia.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Statistical analysis</span><p id="par0050" class="elsevierStylePara elsevierViewall">After assessing the normality of the data, continuous variables were expressed as median and range or mean and standard deviation. Numbers and percentages were used for categorical variables. Continuous variables were compared between the three groups using the non-parametric Kruskal–Wallis test, and bivariate comparisons were performed using the Mann–Whitney <span class="elsevierStyleItalic">U</span> test. Comparisons with categorical variables were performed with the Chi-squared test.</p><p id="par0055" class="elsevierStylePara elsevierViewall">To estimate the discriminatory ability of NIS between responders and non-responders, receiver operating characteristic (ROC) curves were plotted and area under the curve (AUC) was calculated. We calculated the sensitivity and specificity and both positive and negative predicted values to predict progression using a NIS cutoff value of NIS<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>10, and using the cutoff value provided by the Youden index.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Data analysis was performed using IBM® SPSS® Statistics v23.0 software. A <span class="elsevierStyleItalic">p</span>-value<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.05 was considered statistically significant.</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Results</span><p id="par0065" class="elsevierStylePara elsevierViewall">One hundred patients were included in the analysis. The main characteristics of the population are described in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>. As can be seen, the majority of patients were male (56%). The median age at diagnosis and start of tafamidis was 65 years (range: 24–86). Most patients were classified as having a late-onset neurological phenotype (46%), and the median NIS (available in 92% of patients) at diagnosis and tafamidis initiation was 6 (range: 0–100). Most patients (64%) had dysautonomia at diagnosis. Finally, most patients (83%) had very early stage disease (PND 1).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0070" class="elsevierStylePara elsevierViewall">Overall, 47 patients (47%) were classified as complete responders, with a median (range) time on treatment of 36 (12–128) months; 30 (30%) were classified as partial responders, with a median (range) time on treatment of 36 (12–75) months; 21 (21%) were non-responders with a median (range) time on treatment of 23 (12–89) months.</p><p id="par0075" class="elsevierStylePara elsevierViewall">Overall, the median duration of treatment with tafamidis was 35 months (range: 12–128). Treatment was discontinued in 48 patients (48%). The main reason for discontinuation was neurological progression, which occurred in 42 patients (42%), 21 non-responders and 21 partial responders. Of those, after tafamidis treatment, 21 (50%) started treatment with patisiran, 12 (28.6%) started inotersen, 6 (14.3%) were enrolled in clinical trials and 3 (7.1%) underwent liver transplant. In addition, one patient decided to discontinue tafamidis to undergo liver transplantation. Another patient discontinued tafamidis to participate in a clinical trial. Four patients discontinued tafamidis for other unspecified reasons. No significant adverse events were reported.</p><p id="par0080" class="elsevierStylePara elsevierViewall">Very early stages of the disease (defined PND<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>I) were associated with better treatment response (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001). Furthermore, PND and Coutinho stages remained stable in 82 patients (82%) along the study. A lower baseline NIS (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.008), CMAP (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.01) and Norfolk (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.003) were also associated with better treatment response (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). There was a non-significant trend of better treatment response in younger patients. Gender, dysautonomia, baseline SNAP and ESC were not associated with treatment response. Furthermore, Norfolk evaluation was available in 42 patients and it remained stable in 29 (69%).</p><p id="par0085" class="elsevierStylePara elsevierViewall">Main laboratory parameters are summarised in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>. Higher albumin levels (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.04) and lower NTproBNP levels (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.01) were both associated with better treatment response. However, eGFR, prealbumin, retinol binding protein and troponin I were not associated with treatment response.</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0090" class="elsevierStylePara elsevierViewall">When analyzing together complete and partial response vs non-response, mixed phenotype (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.026), older age (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.01), and higher NIS (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.01), Norfolk (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.04), NTproBNP (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.006), and PND (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001) were associated with non-response.</p><p id="par0095" class="elsevierStylePara elsevierViewall">ROC curve analysis was used to compare the ability of baseline NIS to discriminate responders (complete and partial) from non-responders (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The AUC was 0.70 (CI 95% 0.58–0.83; <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.006). A baseline NIS of more than 10 points had a sensitivity of 61% and a specificity of 71% to predict progression. The Youden index yielded a NIS value of 15 as the optimal cutoff point. A baseline NIS of 15 points or more had a sensitivity of 50% and a specificity of 82% to predict progression.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0100" class="elsevierStylePara elsevierViewall">Using NIS cut-offs, we can calculate the positive (PPV) and negative predictive value (NPV) for treatment response. A patient starting tafamidis with a baseline NIS<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>10 has a 33% probability of being a non-responder. On the other hand, using a NIS<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>15 we can predict that the patient could be a non-responder with a 60% probability.</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Discussion</span><p id="par0105" class="elsevierStylePara elsevierViewall">In our analysis of real-world experience with tafamidis 20<span class="elsevierStyleHsp" style=""></span>mg in A-ATTR-V30M with polyneuropathy, we showed that early treatment (FAP I, PND I, lower NIS, higher CMAP, lower Norfolk) was associated with a better response. We also showed that older age, mixed phenotype and higher NT-proBNP levels were associated with a worse response to treatment.</p><p id="par0110" class="elsevierStylePara elsevierViewall">Personalised or precision medicine should be the scope of A-ATTR management as it is an exceptionally heterogeneous disease.<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">17</span></a> Furthermore, the number of treatment options with different pharmacological mechanisms is increasing over time. Therefore, the search for factors associated with treatment response is essential.</p><p id="par0115" class="elsevierStylePara elsevierViewall">The response to tafamidis should be assessed after at least one year of treatment, as an initial worsening could be observed during the first 6 months.<a class="elsevierStyleCrossRefs" href="#bib0215"><span class="elsevierStyleSup">14,15</span></a> On the other hand, there is no global consensus on the definition of disease progression. Response to treatment is mainly based on NIS or NIS<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>7, i.e. response to treatment is defined by clinical and neurophysiological studies. Serum biomarkers, particularly serum light neurofilaments (NfL), are promising markers of disease progression and treatment response. However, there's little data on their use during tafamidis treatment.</p><p id="par0120" class="elsevierStylePara elsevierViewall">We have defined treatment response in the same way as Monteiro et al.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">13</span></a> (no change in NIS values), as this is the largest study analysing response to tafamidis in real-world practice.</p><p id="par0125" class="elsevierStylePara elsevierViewall">It is well known that early treatment with tafamidis is associated with a better pharmacological response and clinical outcome.<a class="elsevierStyleCrossRefs" href="#bib0200"><span class="elsevierStyleSup">11,13,18,19</span></a> This observation has also been found for other treatments such as TTR silencers,<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">20,21</span></a> so early diagnosis of A-ATTR is mandatory for a better prognosis.</p><p id="par0130" class="elsevierStylePara elsevierViewall">Monteiro et al. reported a predictive model for tafamidis response based mainly on sex, neurophysiological score (based on average SNAP and CMAP) and tafamidis plasma concentration.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">13</span></a> Although it showed good performance in discriminating responders from non-responders, this predictive model could rarely be used in clinical practice because the neurophysiological score and especially tafamidis plasma levels are not widely available.</p><p id="par0135" class="elsevierStylePara elsevierViewall">Therefore, it is of great importance to find easily available clinical and biochemical markers to predict tafamidis response. Baseline NIS could be one option. According to the work of Monteiro et al., a baseline NIS<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>10 is associated with a worse treatment response.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">13</span></a> In this study, median NIS of non-responders was 14.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">13</span></a> On the same line, a French open-label study that included 36 V30<span class="elsevierStyleHsp" style=""></span>M patients and included patients with NIS<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>10, showed worse treatment results, with a very low rate of complete response (7%).<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">15</span></a> This finding is also supported by a post hoc analysis of the tafamidis pivotal trial and subsequent extension studies, which selected patients with basal NIS<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>10. In this subgroup, tafamidis treatment was associated with a mean change in NIS from baseline of only 5.3 points in 5.5 years.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">12</span></a> Baseline NIS has also been shown to be an important predictor of treatment response in non-V30M variants.<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">22</span></a> The differences seen in the literature regarding tafamidis treatment response between non-V30M and V30M patients may be related to the often higher baseline NIS in the former group. In our experience, a NIS of 15 points has the best positive predictive value for treatment response. In addition, PND<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>I may be associated with a worse treatment response,<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">18</span></a> as we have also shown. Taking together all these data, the optimal scenario for tafamidis treatment, in which could be considered the first line treatment, would be an A-ATTR-PN patient with PND stage I and a NIS<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>15 points. Thus, other treatment options such as gene silencers should be considered in PND stage<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>I patients or patients with a baseline NIS of more than 15 points.</p><p id="par0140" class="elsevierStylePara elsevierViewall">In our series, the median duration of treatment was 35 months, slightly shorter than in the Portuguese study (48 months),<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">13</span></a> which may justify an overall better response with tafamidis (complete response of 46.1% in our series vs. 34.3% in their series).</p><p id="par0145" class="elsevierStylePara elsevierViewall">It is noteworthy that the median duration of treatment with tafamidis in the non-responders in our cohort was 23 months, with a range of treatment duration of up to 89 months. This means that a significant number of non-responders were relatively stable for at least 2 years. This was also shown in the study led by Monteiro, as the differences in NIS between responders and non-responders increased over time.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">13</span></a></p><p id="par0150" class="elsevierStylePara elsevierViewall">A French analysis of long-term treatment in 43 patients (53% non-V30M) also showed that complete responses were often seen in the first 6–12 months. However, no complete responders were seen after 3 years of follow-up.<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">23</span></a> Thus, the response to tafamidis may not only be dose-dependent but also time-dependent. Why the disease can progress after years of responding to treatment may need to be studied.</p><p id="par0155" class="elsevierStylePara elsevierViewall">In our study, patients with a mixed phenotype and those with higher NT-proBNP levels were more likely to be non-responders. Although tafamidis has been shown to be effective in the treatment of cardiac amyloidosis,<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">24</span></a> it is likely that this subgroup of patients in our cohort, all V30<span class="elsevierStyleHsp" style=""></span>M, have more advanced disease. In addition, higher doses of tafamidis have been shown to be more effective in treating cardiac amyloidosis.<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">25</span></a></p><p id="par0160" class="elsevierStylePara elsevierViewall">Finally, we did not find gender, ESC nor TTR plasma levels differences in treatment response, as others did.<a class="elsevierStyleCrossRefs" href="#bib0210"><span class="elsevierStyleSup">13,23,26</span></a></p><p id="par0165" class="elsevierStylePara elsevierViewall">TTR stabilisation with tafamidis treatment has also been studied. Ando et al. showed that although the percentage of TTR stabilisation was 80% at week 78, it did not correlate with NIS-LL stabilisation.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">10</span></a> Alternative mechanisms other than plasma TTR stabilisation should be associated with treatment response, such as continued deposition of TTR or toxicity of amyloid precursors.<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">14</span></a></p><p id="par0170" class="elsevierStylePara elsevierViewall">Real-world data analysis of tafamidis response is scarce and studies have shown heterogeneus results. In an Italian multicentre study involving 61 patients, worse tafamidis response was observed.<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">14</span></a> This could be due to the low percentage of V30M patients and more advanced disease at baseline.<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">14</span></a> However, this study showed that tafamidis may have a role in non-V30M patients. Similar results were shown in a French cohort of 43 patients, also with a late age of onset (59 years) and predominantly non-V30M variants.<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">23</span></a> An analysis of 252 tafamidis-treated patients from the Transthyretin Amyloidosis Outcomes Survey (THAOS) confirmed the efficacy of tafamidis in a real-world setting. However, the total number of responders or factors associated with treatment response were not reported.<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">27</span></a> Finally, a German retrospective study of a large A-ATTR cohort included 77 patients treated with tafamidis. Although 32 different variants were included in the overall cohort (42.9% V30M), the authors did not show which pathological variants were present in the treated group. In this group, 36 (56.3%) patients experienced disease progression after 28.6 months, with a longer time to progression (34.6 months) in the patients treated early (PND 1). In this study, only basal PND was independently associated with disease progression.<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">18</span></a></p><p id="par0175" class="elsevierStylePara elsevierViewall">In our study, walking ability, measured by both PND and Coutinho stages, remained stable in the vast majority of the population (80%), even in cases where clinical progression was considered. This percentage was higher than in other studies.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">10</span></a> Ambulation status is widely used to classify neurological disease status. However, it has several limitations. NIS and electrophysiological changes are more sensitive to disease progression. In addition, both scales do not assess other important clinical outcomes such as dysautonomia or extraneurological involvement. Changes or progression of the disease at other levels could also lead to treatment change.</p><p id="par0180" class="elsevierStylePara elsevierViewall">Tafamidis treatment has been associated with improved survival in A-ATTR-PN in the short and long term compared with liver transplantation and natural disease progression.<a class="elsevierStyleCrossRefs" href="#bib0180"><span class="elsevierStyleSup">7,28</span></a> Tafamidis has also shown a good safety profile in various studies. Urinary tract infections and diarrhoea were the most commonly reported adverse events.<a class="elsevierStyleCrossRefs" href="#bib0290"><span class="elsevierStyleSup">15,29</span></a> In our study, no serious adverse events were recorded. This ensures its place as a first-line treatment if started promptly.</p><p id="par0185" class="elsevierStylePara elsevierViewall">It would be interesting to know if a higher dose of tafamidis (i.e. tafamidis 61<span class="elsevierStyleHsp" style=""></span>mg) would achieve a better treatment response in A-ATTRv-PN. As tafamidis plasma levels appear to be related to treatment response, it is intuitive to think that a higher dose would result in better clinical outcomes.</p><p id="par0190" class="elsevierStylePara elsevierViewall">Our study has several limitations. Firstly, we were unable to measure tafamidis plasma concentration, which has been shown to be related to treatment response.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">13</span></a> However, this technique is not widely available, making its use in clinical practice difficult. Secondly, we did not include body mass index in our analysis, which has been associated with treatment response in other articles.<a class="elsevierStyleCrossRefs" href="#bib0290"><span class="elsevierStyleSup">15,23</span></a> Nevertheless, we show differences in other nutritional variables such as serum albumin, also suggesting that nutritional status may play a role in treatment response. Thirdly, patients in our study were treated and followed in referral centres, so results in real life scenarios out of this kind of centres could vary. Finally, our study includes only patients with the V30M variant, so the results cannot be extrapolated to other variants.</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Conclusions</span><p id="par0195" class="elsevierStylePara elsevierViewall">Our results reinforce the tafamidis efficacy to treat A-ATTRv-PN if started early in the disease course. Patients with the V30M variant, NIS<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>15 and PND I are the most appropriate subjects for this treatment, and treatment alternatives should be considered in more advanced disease. Treatment failure may occur late after starting tafamidis.</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Ethical considerations</span><p id="par0200" class="elsevierStylePara elsevierViewall">The study was conducted in accordance with the standards of the Good Clinical Practice and Guidelines of the International Conference on Harmonisation (ICH). The study obtained the approval of the research commission of the Son Llàtzer University Hospital and the Ethics Committee of the Balearic Islands (IB 4760/21 EOm). Due to its retrospective nature, no confirmed informed consent was collected. However, all data were anonymised and treated confidentially in accordance with current legislation on the protection of personal data.</p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Funding</span><p id="par0205" class="elsevierStylePara elsevierViewall">This study was done without any public or private funding.</p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Conflicts of interest</span><p id="par0210" class="elsevierStylePara elsevierViewall">MARS, ARR, LMV, AAV, MMP, LGV, FMV, AFB and ECB have no conflicts to declare. TS has disclosed that he has received speaking and consultancy fees from Pfizer, Alnylam, Sobi and Sanofi. CBG has disclosed that he has received speaking fees from Pfizer, Alnylam, Sobi and Akcea. JFM has disclosed that he has received speaking and consultancy fees from Pfizer, Alnylam and Sobi. LGD has disclosed that he received speaker and consultant fees from Pfizer, Alnylam, Akcea and Grunenthal. FMV has disclosed that he has received speaking fees from Pfizer, Alnylam, Horizon and Sobi. CCP has disclosed that he has received speaking and consultancy fees from Alexion-AstraZeneca, Alnylam, Sobi, CSL Behring, Pfizer, Argnex Inc, UCB Pharma, Ferrer Inc and PharmaNext. ILL and JGM have disclosed that he has received speaking and consultancy fees from Pfizer, Alnylam, Sobi and Akcea.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:13 [ 0 => array:3 [ "identificador" => "xres2141280" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1817803" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres2141279" "titulo" => "Resumen" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1817802" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:3 [ "identificador" => "sec0010" "titulo" => "Material and methods" "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0015" "titulo" => "Study population" ] 1 => array:2 [ "identificador" => "sec0020" "titulo" => "Outcome measurements" ] 2 => array:2 [ "identificador" => "sec0025" "titulo" => "Other measurements" ] 3 => array:2 [ "identificador" => "sec0030" "titulo" => "Responder classification" ] 4 => array:2 [ "identificador" => "sec0035" "titulo" => "Statistical analysis" ] ] ] 6 => array:2 [ "identificador" => "sec0040" "titulo" => "Results" ] 7 => array:2 [ "identificador" => "sec0045" "titulo" => "Discussion" ] 8 => array:2 [ "identificador" => "sec0050" "titulo" => "Conclusions" ] 9 => array:2 [ "identificador" => "sec0055" "titulo" => "Ethical considerations" ] 10 => array:2 [ "identificador" => "sec0060" "titulo" => "Funding" ] 11 => array:2 [ "identificador" => "sec0065" "titulo" => "Conflicts of interest" ] 12 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2023-10-02" "fechaAceptado" => "2024-01-25" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1817803" "palabras" => array:5 [ 0 => "Transthyretin amyloidosis" 1 => "ATTR" 2 => "Polyneuropathy" 3 => "Tafamidis" 4 => "Real-life" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1817802" "palabras" => array:5 [ 0 => "Amiloidosis por transtirretina" 1 => "ATTR" 2 => "Polineuropatía" 3 => "Tafamidis" 4 => "Vida real" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Tafamidis is the only approved transthyretin stabiliser approved for the treatment of variant transthyretin amyloidosis (A-ATTRv) related polyneuropathy (PNP). The aim of this study is to analyse the effectiveness of tafamidis in a real-world setting in Spain.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">This is a national multicenter study in which patients with V30M A-ATTR related PN treated with tafamidis for at least 1 year were included. Clinical, demographic, analytical and neurophysiological variables were analysed.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">100 patients were recruited. Overall, 47 patients (47%) were classified as complete responders, 32 (32%) as partial responders and 21 (21%) as non-responders. The median duration of treatment with tafamidis was 35 months. Better treatment response was shown in patients with in polyneuropathy disability score (PND) I, lower neuropathy impairment score (NIS), compound muscle action potential (CMAP) and Norfolk QoL questionnaire. Higher albumin levels and lower NTproBNP levels were also associated with better treatment response. A basal NIS<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>15 predicts that the patient could be a non-responder with a 60% probability.</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Our results reinforce the tafamidis efficacy to treat A-ATTRv-PNP if started early in the disease course. Patients with the V30M variant, NIS<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>15 and PND I are the most appropriate subjects for this treatment.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Introducción</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Tafamidis es el único estabilizador de la transtirretina aprobado para el tratamiento de la polineuropatía (PN) relacionada con la amiloidosis por transtirretina variante (A-ATTRv). El objetivo de este estudio es analizar la eficacia de tafamidis en vida real en España.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Métodos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Se trata de un estudio multicéntrico nacional en el que se incluyó a pacientes con PN relacionada con V30M A-ATTR tratados con tafamidis durante al menos un año. Se analizaron variables clínicas, demográficas, analíticas y neurofisiológicas.</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Se reclutó a 100 pacientes. En conjunto, 47 pacientes (47%) fueron clasificados como respondedores completos, 32 (32%) como respondedores parciales y 21 (21%) como no respondedores. La duración media del tratamiento con tafamidis fue de 35 meses. Se observó una mejor respuesta al tratamiento en los pacientes con <span class="elsevierStyleItalic">polyneuropathy disability score</span> (PND) 1 y con <span class="elsevierStyleItalic">neuropathy impairment score</span> (NIS), <span class="elsevierStyleItalic">compound muscle action potential</span> (CMAP) y cuestionario Norfolk QoL más bajos. Los niveles más altos de albúmina y los niveles más bajos de NTproBNP también se asociaron a una mejor respuesta al tratamiento. Un NIS basal ≥ 15 predice que el paciente podría ser no respondedor con una probabilidad del 60%.</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusiones</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Nuestros resultados refuerzan la eficacia de tafamidis para tratar la A-ATTRv-PN si se inicia precozmente en el curso de la enfermedad. Los pacientes con la variante V30M, NIS<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>15 y PND I son los sujetos más apropiados para este tratamiento.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1523 "Ancho" => 1508 "Tamanyo" => 83408 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">ROC curve for NIS and treatment response. NIS: lower neuropathy impairment score.</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:2 [ "leyenda" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">CMAP: compound muscle action potential. ESC: electrochemical skin conductance. NIS: neuropathy impairment score. NS: non-significant. PND: polyneuropathy disability scoring system. SNAP: sensory nerve action potential.</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="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Overall (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>100) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Responders (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>47) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Partial responders (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>32) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Non responders (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>21) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Men (%)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">56 (56%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">23 (48.9%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">20 (62.5%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">13 (54.2%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Median age</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">65 (24–86) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">61 (31–83) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">60.5 (24–86) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">76 (42–85) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="6" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Clinical phenotype</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="4" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Neurological early onset \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">26 (26%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11 (23.4%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11 (34.4%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 (19%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Neurological late onset \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">46 (46%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">26 (55.3%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">15 (46.9%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5 (23.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Mixed \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">28 (28%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 (21.3%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 (18.8%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">12 (57.1%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="6" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Median NIS (n</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">92)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 (0–100) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3 (0–47) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 (0–100) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">14 (2–64) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.008 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Dysautonomia</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">64 (64%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">25 (53.2%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">24 (75%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">15 (71.4%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="6" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Coutinho</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="4" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">100 (98.0%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">47 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">32 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">21 (100%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>II \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>III \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="6" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">PND</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="6" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.03</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>I \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">83 (83%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">45 (95.7%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">27 (84.4%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11 (52.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>II \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">17 (17%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2 (4.3%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5 (15.6%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 (47.6%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>IIIa \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>IIIb \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>IV \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " colspan="6" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Median Norfolk (n</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">42)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">27.5 (0–123) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">14 (0–80) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">55 (0–123) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">51 (23–93) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.003 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Median CMAP (μV) (n</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">70)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8.9 (1.2–20.6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11.3 (1.6–20.6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7.7 (1.2–15) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8.4 (3.2–12.8) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.01 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Median SNAP (μV) (n</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">69)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">12.1 (0–63) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">17.5 (0–63) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8.6 (0–41.4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10.1 (0–53) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">ESC (μS) (n</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">=</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">33)</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">144 (73–176) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">146.5 (73–176) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">144.5 (92–164) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">144 (134–174) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3529839.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Main baseline characteristics of the cohort.</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="spar0065" class="elsevierStyleSimplePara elsevierViewall">GFR: glomerular filtration rate. NS: non significant. RBP: retinol binding protein. TpI: troponin I. TTR: transthyretin.</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="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Overall (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>100) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Responders (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>47) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Partial responders (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>32) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Non responders (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>21) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Median NTproBNP (pg/mL) (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>46) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">178 (3–1117) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">132 (8–419) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">193 (3–1024) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">407 (73–1117) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.01 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Median eGFR (mL/min) (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>90) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">87.5 (26–159.4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">85.5 (36–110) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">90 (26–113) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">88 (36–112) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Median albumin (g/L) (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>80) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4.3 (3.2–4.9) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4.4 (3.2–4.9) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4.2 (3.5–4.9) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4.2 (3.9–4.6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.04 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Median TTR (mg/dL) (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>43) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">25 (3–34.8) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">23 (12–31.2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">24.1 (12–34.8) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">26.9 (3–31) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Median RBP (mg/dL) (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>27) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5.2 (1.8–8.5) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4.8 (1.8–7.8) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5.2 (1.9–8.3) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5.2 (4.4–8.5) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Median TpI (ng/L) (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>9) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7 (1.7–36.3) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6.5 (1.7–36.3) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="char" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8.2 (3.5–19.1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NA \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">NS \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab3529840.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Main baseline laboratory characteristics.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:29 [ 0 => array:3 [ "identificador" => "bib0150" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Familial amyloid polyneuropathy" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "V. Planté-Bordeneuve" 1 => "G. Said" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/S1474-4422(11)70246-0" "Revista" => array:5 [ "tituloSerie" => "Lancet Neurol" "fecha" => "2011" "volumen" => "12" "paginaInicial" => "1086" "paginaFinal" => "1097" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0155" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A descriptive analysis of ATTR amyloidosis in Spain from the transthyretin amyloidosis outcomes survey" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. González-Moreno" 1 => "I. Losada-López" 2 => "E. Cisneros-Barroso" 3 => "P. Garcia-Pavia" 4 => "J. González-Costello" 5 => "F. 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Journal Information
Original article
Real life experience of tafamidis for the treatment of Spanish patients with Val30Met transthyretin amyloidosis with polyneuropathy
Experiencia en vida real en España del uso de tafamidis para el tratamiento de pacientes con amiloidosis por transtirretina VAL30MET con polineuropatía
Maria Antonia Ribot Sansoa, Adrián Rodriguez Rodrigueza, Laura Martínez Vicenteb, Teresa Sevillac, Cristina Borrachero Garrod, Julian Fernández Martíne, Adrián Antón Vicentef, Moises Morales de la Pridag, Lucía Galán Dávilab, Laura González Vázquezh, Ferran Martínez Vallef, Carlos Casasnovas Ponsg,i,j, Arturo Fraga Baue, Eugenia Cisneros Barrosoa, Inés Losada Lópeza, Juan González-Morenoa,
Corresponding author
a Servicio de Medicina Interna, Unidad Amiloidosis por Trastirretina, Hospital Universitario Son Llàtzer, Instituto de Investigación Sanitaria Illes Balears (idISBA), Palma de Mallorca, Spain
b Servicio de Neurología, Unidad de Neuromuscular, IdISSC, Hospital Clínico San Carlos, Madrid, Spain
c Servicio de Neurología, Hospital Universitari i Politècnic La Fe/IISLAFE, Universitat de Valencia, CIBERER (ERN EURO-NMD), Valencia, Spain
d Servicio de Medicina Interna, UMAH, Hospital Universitario Juan Ramón Jiménez, Huelva, Spain
e Servicio de Medicina Interna, Hospital Álvaro Cunqueiro, Vigo, Spain
f Servicio de Medicina Interna, Hospital Vall d’Hebron, Barcelona, Spain
g Neuromuscular Unit, Neurology Department, Bellvitge University Hospital-IDIBELL, Spain
h Servicio de Medicina Interna, Hospital Ribera-Povisa, Vigo, Spain
i Multidisciplinary Unit of Familial Amyloidosis, Bellvitge University Hospital-IDIBELL, Neurometabolic Diseases Group, Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
j Biomedical Research Network Center in Rare Diseases (CIBERER), Valencia, Spain
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