Cardiomyopathy in Patients With Hereditary Bullous Epidermolysis

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Batalla, A. Vicente, J. Bartrons, F. Prada, C. Fortuny, M.A. González-Enseñat" "autores" => array:6 [ 0 => array:3 [ "nombre" => "A." "apellidos" => "Batalla" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "A." "apellidos" => "Vicente" "email" => array:1 [ 0 => "avicente@hsjdbcn.org" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "J." "apellidos" => "Bartrons" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "F." "apellidos" => "Prada" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] 4 => array:3 [ "nombre" => "C." "apellidos" => "Fortuny" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "c" "identificador" => "aff0015" ] ] ] 5 => array:3 [ "nombre" => "M.A." "apellidos" => "González-Enseñat" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Sant Joan de Déu, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Cardiología, Hospital Sant Joan de Déu, Barcelona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Pediatría, Hospital Sant Joan de Déu, Barcelona, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Miocardiopatía en pacientes con epidermólisis ampollosa hereditaria" ] ] "textoCompleto" => "IntroductionThere are 3 main subtypes of cardiomyopathy: dilated, hypertrophic, and restrictive. Dilated cardiomyopathy (DCM) is defined as progressive dilation of the heart with contractile dysfunction of the left or the left and right ventricles.<a class="elsevierStyleCrossRef" href="#bib0105">1</a> One characteristic of DCM is congestive heart failure.<a class="elsevierStyleCrossRef" href="#bib0110">2</a> DCM is uncommon in the pediatric population, with an incidence of 0.6 to 1.2 cases per 10 000 children.<a class="elsevierStyleCrossRefs" href="#bib0105">1,2</a> Diverse factors have been implicated in its etiology, such as idiopathic, familial or genetic, viral, and autoimmune factors, micronutrient deficiency, iron overload, chronic anemia, and toxic or pharmacological agents.<a class="elsevierStyleCrossRefs" href="#bib0105">1,2</a>Inherited epidermolysis bullosa, which we will refer to throughout this article as epidermolysis bullosa (EB), comprises a group of genetic disorders characterized by skin fragility and blistering. These disorders as classified as EB simplex, junctional EB (Herlitz and non-Herlitz), dystrophic EB (dominant or recessive), and Kindler syndrome. The classification of EB according to the 2008 consensus report is shown in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.<a class="elsevierStyleCrossRefs" href="#bib0115">3,4</a><elsevierMultimedia ident="tbl0005"></elsevierMultimedia>An association between EB and DCM has been observed in recent decades, particularly in patients with recessive dystrophic EB. The cases published to date are summarized in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a><a class="elsevierStyleCrossRefs" href="#bib0110">2,5–14</a> and additional details are provided in Supplementary <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.<elsevierMultimedia ident="tbl0010"></elsevierMultimedia>The aim of this study was to determine the prevalence of DCM in pediatric patients with EB.MethodsWe performed a descriptive cross-sectional study of all cases of EB seen at Hospital de San Joan de Déu, a pediatric referral hospital in Barcelona, Spain, between May 1986 and April 2015. Patients with a clinical diagnosis of EB were identified through a database providing access to medical records and photographs. Based on the consensus recommendations for the classification of EB,<a class="elsevierStyleCrossRef" href="#bib0115">3</a> patients were divided into the different EB subtypes based on clinical findings and test results (standard histology, antigen mapping, electron microscopy, genetic studies). Information was also noted on reasons for referral to cardiology, presence or absence of echocardiographic alterations, presence or absence of DCM (diagnosed by a pediatric cardiologist and defined as ventricular dilation and reduction in systolic function), and laboratory findings possibly associated with DCM. All the data were recorded retrospectively and based only on information contained in the patients’ charts (history, physical examination, and other tests).ResultsSeventy-two patients with a diagnosis of EB were identified through the database. After exclusion of those with a doubtful diagnosis or lacking the test results required for correct classification, 57 patients were included. Nineteen had EB simplex, 10 had junctional EB (4 Herlitz and 6 non-Herlitz), 27 had dystrophic EB (14 dominant and 13 recessive), and 1 had Kindler syndrome.Cardiological and echocardiographic assessment had been performed in 19 patients (5 with EB simplex, 1 with Herlitz junctional EB, 4 with non-Herlitz junctional EB, 2 with dominant dystrophic EB, and 7 with recessive dystrophic EB). Among them, 17 patients showed no signs or symptoms of cardiac involvement and had been referred to the cardiologist to investigate early DCM. Two patients had been referred with a clinical suspicion of cardiomyopathy (confirmed in 1 case).DCM was diagnosed in just 2 patients, both with recessive dystrophic EB. This corresponds to a prevalence of 3.5% (2 of the 57 children analyzed). The prevalence of DCM by EB subtype was 0% for EB simplex, junctional EB, and Kindler syndrome (0/19, 0/10, and 0/1, respectively) and 7.4% (2/27) for dystrophic EB. Considering only the patients with recessive dystrophic EB, the prevalence of DCM was 15.4% (2/13).In the first of the 2 cases in which a diagnosis of DCM was confirmed, the condition was detected when the patient was 13 years old due to signs and symptoms of heart failure and compatible echocardiographic findings (ventricular dilation and ejection fraction [EF] <50%). The patient was treated with carvedilol at doses adjusted to symptoms and EF. Initially, the EF remained at around 50%, but it decreased significantly (to 20%) during flares. Four years after diagnosis of DCM, the patient showed progressive worsening and developed kidney failure due to systemic AA amyloidosis. He died at the age of 21 years, with severely deteriorated general health and multiple organ failure. Factors that may have contributed to the development of DCM are chronic anemia, multiple regular blood transfusions, hypoalbuminemia, hypoaminoacidemia, and zinc and selenium deficiency.The second patient was diagnosed with DCM during a routine echocardiographic examination when she was 3 years of age. She has since been receiving treatment with carvedilol (2mg/12h). The EF has remained at around 45% and there have been no signs of heart failure. Factors that could have contributed to the development of DCM in this patient include anemia, regular blood transfusions, hypoalbuminemia, hypoaminoacidemia, and carnitine deficiency.<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a> shows the presence of factors potentially associated with the development of DCM in the patients in our series grouped by EB subtype. Twenty-three patients had at least 1 alteration that may have an etiological role in DCM. Eleven of these patients (48%) had recessive dystrophic EB.<elsevierMultimedia ident="tbl0015"></elsevierMultimedia>DiscussionAn increased risk of heart disease has been observed in patients with EB. Fine et al.<a class="elsevierStyleCrossRef" href="#bib0110">2</a> published a report on 15 patients with EB and congestive heart failure, and considered that most of the patients also had DCM. The subtypes associated with heart failure were recessive dystrophic EB and non-Herlitz junctional EB. The condition was most common in patients with severe generalized dystrophic recessive EB. The authors found the risk of congestive heart failure to be significantly greater in these patients than in the general population and even greater still in patients with concomitant chronic kidney failure. More recently, Ryan et al.<a class="elsevierStyleCrossRef" href="#bib0170">14</a> investigated the presence of subclinical heart damage and other vascular disorders in patients with recessive dystrophic EB. They found that 40 of the 45 patients analyzed had echocardiographic abnormalities and that 18% had a dilated aortic arch.The factors that have been most often linked to the development of cardiopathy in patients with EB are chronic anemia, iron overload, low carnitine and selenium levels, drugs, and concomitant viral infection.<a class="elsevierStyleCrossRef" href="#bib0105">1</a>Chronic anemia generally has multiple causes. It responds poorly to dietary supplementation and requires regular blood transfusions. Both chronic anemia and iron overload secondary to repeated transfusions may have a role in the onset of DCM.<a class="elsevierStyleCrossRef" href="#bib0105">1</a>In relation to nutritional deficiencies, EB patients with DCM have been found to have significantly lower levels of carnitine than those without.<a class="elsevierStyleCrossRef" href="#bib0105">1</a> Selenium supplementation in cases of deficiency has been found to reduce morbidity and mortality in DCM. Nevertheless, no significant differences have been observed between selenium levels in patients with EB and DCM and patients with EB without DCM.<a class="elsevierStyleCrossRef" href="#bib0140">8</a> Thiamine deficiency is another documented cause of DCM.<a class="elsevierStyleCrossRef" href="#bib0140">8</a> Although there have been reports of DCM being reversed with restoration of carnitine levels<a class="elsevierStyleCrossRef" href="#bib0140">8</a> and of improved symptoms with selenium or thiamine supplementation, micronutrient deficiency alone does not appear to be a causal factor in DCM, but it could be a contributory factor.<a class="elsevierStyleCrossRefs" href="#bib0105">1,13</a>Viral infections can also cause DCM.<a class="elsevierStyleCrossRef" href="#bib0105">1</a> The heart damage in such cases is generally due to a postinfectious autoimmune process.<a class="elsevierStyleCrossRef" href="#bib0140">8</a> Prodromal viral infection frequently goes unnoticed, as it can precede the onset of DCM by several months. Diagnosis is even more complicated in patients with aggressive forms of EB, who, for varying reasons, may exhibit symptoms such as fever or dyspnea of noncardiac origin.<a class="elsevierStyleCrossRef" href="#bib0105">1</a>Cardiotoxic drugs can also exacerbate DCM in patients with EB and should therefore be withdrawn on detection of symptoms suggestive of cardiopathy.<a class="elsevierStyleCrossRef" href="#bib0175">15</a>Apart from mutations in the PLEC1 gene<a class="elsevierStyleCrossRefs" href="#bib0160">12,16,17</a> (coding for plectin) in EB simplex with muscular dystrophy associated with DCM<a class="elsevierStyleCrossRefs" href="#bib0160">12,16,17</a> and mutations in the DSP gene (coding for desmoplakin) in lethal acantholytic EB,<a class="elsevierStyleCrossRefs" href="#bib0190">18,19</a> no other genetic alterations have been linked to the development of cardiomyopathy in EB. There is an interesting case of a patient with recessive dystrophic EB who had left ventricular noncompaction cardiomyopathy (LVNC) without DCM.<a class="elsevierStyleCrossRef" href="#bib0200">20</a> The authors indicated that LVNC can progress to DCM in certain cases, and suggested that it might precede DCM in recessive dystrophic EB.Patients with EB have an increased risk of DCM, and those who develop the disease have a mortality of between 30% and 60%. In patients with recessive dystrophic EB, DCM tends to be detected at around 10 years of age, when it is already at an advanced stage. Such cases are associated with low EF and high mortality, with death generally occurring within 3 months of diagnosis. These data suggest delayed diagnosis—and more complicated treatment—of DCM in this setting.<a class="elsevierStyleCrossRef" href="#bib0105">1</a>Diagnostic tests that have been proposed for investigating DCM in asymptomatic patients with EB include annual blood tests with complete blood count, albumin levels, total and free carnitine levels, selenium and zinc levels, and cardiac assessment (electrocardiogram and echocardiogram). Patients with symptoms or alterations in any of the above tests should be urgently referred for cardiac assessment. In addition, thyroid hormone measurement, serology, culture of parvovirus and Coxsackie virus, metabolic tests, and myocardial biopsy when appropriate, should be performed.ConclusionsDCM must be contemplated as a possible complication of EB, especially in patients with recessive dystrophic or junctional EB. Regular testing at a young age is therefore necessary, as is the correction of alterations that have been linked to heart damage in these patients.<a class="elsevierStyleCrossRef" href="#bib0105">1</a> Early detection of anomalies will allow prompt initiation of the necessary treatment, thereby slowing disease progress and prolonging survival.Ethical DisclosuresProtection of humans and animalsThe authors declare that no tests were carried out in humans or animals for the purpose of this study.Confidentiality of dataThe authors declare that they have followed their hospital's protocol on the publication of data concerning patients.Right to privacy and informed consentThe authors declare that no private patient data appear in this article.Conflicts of InterestThe authors declare that they have no conflicts of interest." "textoCompletoSecciones" => array:1 [ "secciones" => array:12 [ 0 => array:3 [ "identificador" => "xres858578" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Background and objective" ] 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" => "xpalclavsec852701" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres858579" "titulo" => "Resumen" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introducción y objetivos" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusión" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec852700" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Methods" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Results" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Discussion" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conclusions" ] 9 => array:3 [ "identificador" => "sec0030" "titulo" => "Ethical Disclosures" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0035" "titulo" => "Protection of humans and animals" ] 1 => array:2 [ "identificador" => "sec0040" "titulo" => "Confidentiality of data" ] 2 => array:2 [ "identificador" => "sec0045" "titulo" => "Right to privacy and informed consent" ] ] ] 10 => array:2 [ "identificador" => "sec0050" "titulo" => "Conflicts of Interest" ] 11 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2016-09-20" "fechaAceptado" => "2017-01-31" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec852701" "palabras" => array:4 [ 0 => "Echocardiography" 1 => "Epidermolysis bullosa" 2 => "Micronutrients" 3 => "Dilated cardiomyopathy" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec852700" "palabras" => array:4 [ 0 => "Ecocardiograma" 1 => "Epidermólisis ampollosa" 2 => "Micronutrientes" 3 => "Miocardiopatía dilatada" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "Background and objectiveIn recent decades, an association has been reported between epidermolysis bullosa (EB) and dilated cardiomyopathy (DC). DC is typically in an advanced phase when detected, leading to a poorer prognosis. Our objective was to determine the prevalence of DC in patients with EB seen in Hospital San Joan de Déu in Barcelona, Spain, between May 1986 and April 2015. MethodsThis was a descriptive, cross-sectional chart-review study in which we recorded the type and main subtypes of EB and the presence or absence of DC. ResultsFifty-seven patients with EB were found, 19 with EB simplex, 10 with junctional EB, 27 with dystrophic EB (14 dominant dystrophic and 13 recessive dystrophic), and just 1 with Kindler syndrome. DC was detected in only 2 patients with recessive dystrophic EB. Twenty-three patients had presented factors that could have had a causal relationship with the potential onset of DC. ConclusionsDC is a possible complication of EB, particularly in recessive dystrophic EB. Periodic follow-up should be performed to make an early diagnosis and start treatment." "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Background and objective" ] 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" => "Introducción y objetivosEn las últimas décadas se ha descrito la asociación entre epidermólisis ampollosa (EA) y miocardiopatía dilatada (MD). Generalmente esta última enfermedad se detecta en fases avanzadas, implicando un peor pronóstico.Nuestro objetivo consistió en determinar la prevalencia de MD en los pacientes con EA vistos en el Hospital San Joan de Déu (Barcelona) desde mayo de 1986 a abril de 2015. MétodosEstudio descriptivo transversal mediante revisión de las historias clínicas con atención al tipo y subtipos mayores de EA y la existencia o no de MD. ResultadosSe recogieron 57 pacientes con diagnóstico de EA. De ellos 19 presentaban EA simple, 10 EA juntural, 27 EA distrófica (14 EA distrófica dominante y 13 EA distrófica recesiva) y existió un caso de síndrome de Kindler. Solo 2 de los pacientes con EA distrófica recesiva presentaron MD. En 23 de los pacientes con EA existieron factores que podrían tener una relación causal con el potencial desarrollo de MD. ConclusiónLa MD puede ser una complicación en los pacientes con EA, mayoritariamente del subtipo de EA distrófica recesiva, por lo que deben hacerse controles periódicos para su temprano diagnóstico y tratamiento." "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Introducción y objetivos" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusión" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "Please cite this article as: Batalla A, Vicente A, Bartrons J, Prada F, Fortuny C, González-Enseñat MA. Miocardiopatía en pacientes con epidermólisis ampollosa hereditaria. Actas Dermosifiliogr. 2017;108:544–549." ] ] "apendice" => array:1 [ 0 => array:1 [ "seccion" => array:1 [ 0 => array:4 [ "apendice" => "<elsevierMultimedia ident="upi0005"></elsevierMultimedia>" "etiqueta" => "Appendix A" "titulo" => "Supplementary data" "identificador" => "sec0060" ] ] ] ] "multimedia" => array:4 [ 0 => 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" => "Source: Fine et al.<a class="elsevierStyleCrossRef" href="#bib0115">3</a>; Hernandez-Martin et al.<a class="elsevierStyleCrossRef" href="#bib0120">4</a>" "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=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">EB simplex (EBS) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Suprabasal \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Basal \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Lethal acantholytic EB Plakophilin syndrome EBS superficialis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">EBS, localized EBS, Dowling-Meara or herpetiform EBS, generalized (non-Dowling-Meara) EBS with mottled pigmentation EBS with pyloric atresia EBS, Ogna EBS, migratory circinate \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Junctional EB (JEB) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Herlitz type \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Non-Herlitz type \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">JEB, non-Herlitz, generalized JEB with pyloric atresia JEB, inversa JEB, late-onset Laryngo-onycho-cutaneous syndrome \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Dystrophic EB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Dominant dystrophic EB (DDEB) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Recessive dystrophic EB (RDEB) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">DDEB DDEB, acral DDEB, pretibial DDEB, pruriginosa DDEB, nails only DDEB, bullous dermolysis of the newborn \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">RDEB, severe generalized RDEB, generalized other RDEB, inversa RDEB, pretibial RDEB, pruriginosa RDEB, centripetalis RDEB, bullous dermolysis of the newborn \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mixed EB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Kindler Syndrome \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1450836.png" ] ] ] ] "descripcion" => array:1 [ "en" => "Current Classification of Inherited Epidermolysis Bullosa (EB)." ] ] 1 => 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:3 [ "leyenda" => "Abbreviations: F, female; M, male." "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=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mean age (SD), y \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " colspan="2" align="left" valign="top">12.27 (5.60)<a class="elsevierStyleCrossRef" href="#tblfn0010">b</a></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sex \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " colspan="2" align="left" valign="top">M:F=27:31<a class="elsevierStyleCrossRef" href="#tblfn0010">b</a></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ejection fraction, % \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " colspan="2" align="left" valign="top">9-45<a class="elsevierStyleCrossRef" href="#tblfn0015">c</a></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">EB subtype \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">EB simplex \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3/71 (4.2%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Non-Herlitz junctional EB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2/71 (2.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Dystrophic EB (not specified) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1/71 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Recessive dystrophic EB \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">65/71 (91.5%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Outcome \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Death \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">24/71 (33.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Survival \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">44/71 (62.0%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Not specified \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3/71 (4.2%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Associated factors mentioned in at least 1 case \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Anemia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">22/71 (31.0%)<a class="elsevierStyleCrossRef" href="#tblfn0020">d</a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Iron overload (secondary hemosiderosis) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2/71 (2.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Fluid overload \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1/71 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Mutation in PLEC \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1/71 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Mutation in DSP \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2/71 (2.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Chronic kidney failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6/71 (8.5%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Nutritional deficiency (not specified) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1/71 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Carnitine deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7/71 (9.9%)<a class="elsevierStyleCrossRef" href="#tblfn0020">d</a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Selenium deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7/71 (9.9%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Zinc deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2/71 (2.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hypoalbuminemia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">9/71 (12.7%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hypoaminoacidemia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2/71 (2.8%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Cardiotoxic drugs (amitriptyline, cisapride) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1/71 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Viral cause \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1/71 (1.4%) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1450834.png" ] ] ] "notaPie" => array:4 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "More detailed information can be consulted in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> of the supplementary material." ] 1 => array:3 [ "identificador" => "tblfn0010" "etiqueta" => "b" "nota" => "Sex and age were not specified in several of the articles." ] 2 => array:3 [ "identificador" => "tblfn0015" "etiqueta" => "c" "nota" => "Minimum and maximum values are given as most studies did not report exact values." ] 3 => array:3 [ "identificador" => "tblfn0020" "etiqueta" => "d" "nota" => "One article did not specify the exact number of patients with chronic anemia, hemoglobin<8mg/dL, or carnitine deficiency. In this table, it has been considered as one patient for each of the mentioned items." ] ] ] "descripcion" => array:1 [ "en" => "Summary of Epidermolysis Bullosa (EB) Cases Associated With Dilated Cardiomyopathy in the Literature<a class="elsevierStyleCrossRef" href="#tblfn0005">a</a>" ] ] 2 => array:8 [ "identificador" => "tbl0015" "etiqueta" => "Table 3" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at3" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "Abbreviations: DDEB, dominant dystrophic epidermolysis bullosa; EBS, epidermolysis bullosa simplex; H-JEB, Herlitz junctional epidermolysis bullosa; nH-JEB, non-Herlitz junctional epidermolysis bullosa; RDEB, recessive dystrophic epidermolysis bullosa." "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Factor \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">EBS \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">H-JEB \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">nH-JEB \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">DDEB \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">RDEB \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Kindler Syndrome<a class="elsevierStyleCrossRef" href="#tblfn0025">a</a> \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Anemia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10.5% (2/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">75% (3/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">66.7% (4/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">76.9% (10/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(1/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Iron overload \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5.3% (1/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">15.4% (2/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(0/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Selenium deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">25% (1/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">30.8% (4/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(0/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Carnitine deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">15.4% (2/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(0/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Zinc deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">25% (1/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">46.2% (6/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(0/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypoalbuminemia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5.3% (1/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">75% (3/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">16.7% (1/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">69.2% (9/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(0/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypoaminoacidemia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5.3% (1/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">25% (1/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7.1% (1/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">46.2% (6/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(0/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Kidney failure \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/19) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">0% (0/14) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7.7% (1/13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">(0/1) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1450835.png" ] ] ] "notaPie" => array:1 [ 0 => array:3 [ "identificador" => "tblfn0025" "etiqueta" => "a" "nota" => "Not expressed as a percentage as just 1 patient had Kindler syndrome." ] ] ] "descripcion" => array:1 [ "en" => "Factors That Could Play a Role in the Development of Dilated Cardiomyopathy in the Patients in the Present Series." ] ] 3 => array:5 [ "identificador" => "upi0005" "tipo" => "MULTIMEDIAECOMPONENTE" "mostrarFloat" => false "mostrarDisplay" => true "Ecomponente" => array:2 [ "fichero" => "mmc1.pdf" "ficheroTamanyo" => 63195 ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:20 [ 0 => array:3 [ "identificador" => "bib0105" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Dilated cardiomyopathy in epidermolysis bullosa: A retrospective, multicenter study" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "I. 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Cardiomyopathy in Patients With Hereditary Bullous Epidermolysis

Miocardiopatía en pacientes con epidermólisis ampollosa hereditaria

A. Batallaa, A. Vicentea,

Corresponding author

avicente@hsjdbcn.org

Corresponding author.

, J. Bartronsb, F. Pradab, C. Fortunyc, M.A. González-Enseñata

a Servicio de Dermatología, Hospital Sant Joan de Déu, Barcelona, Spain

b Servicio de Cardiología, Hospital Sant Joan de Déu, Barcelona, Spain

c Servicio de Pediatría, Hospital Sant Joan de Déu, Barcelona, Spain

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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">There are 3 main subtypes of cardiomyopathy&#58; dilated&#44; hypertrophic&#44; and restrictive&#46; Dilated cardiomyopathy &#40;DCM&#41; is defined as progressive dilation of the heart with contractile dysfunction of the left or the left and right ventricles&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a> One characteristic of DCM is congestive heart failure&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">2</span></a> DCM is uncommon in the pediatric population&#44; with an incidence of 0&#46;6 to 1&#46;2 cases per 10 000 children&#46;<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">1&#44;2</span></a> Diverse factors have been implicated in its etiology&#44; such as idiopathic&#44; familial or genetic&#44; viral&#44; and autoimmune factors&#44; micronutrient deficiency&#44; iron overload&#44; chronic anemia&#44; and toxic or pharmacological agents&#46;<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Inherited epidermolysis bullosa&#44; which we will refer to throughout this article as <span class="elsevierStyleItalic">epidermolysis bullosa</span> &#40;EB&#41;&#44; comprises a group of genetic disorders characterized by skin fragility and blistering&#46; These disorders as classified as EB simplex&#44; junctional EB &#40;Herlitz and non-Herlitz&#41;&#44; dystrophic EB &#40;dominant or recessive&#41;&#44; and Kindler syndrome&#46; The classification of EB according to the 2008 consensus report is shown in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">3&#44;4</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">An association between EB and DCM has been observed in recent decades&#44; particularly in patients with recessive dystrophic EB&#46; The cases published to date are summarized in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a><a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">2&#44;5&#8211;14</span></a> and additional details are provided in Supplementary <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#46;</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The aim of this study was to determine the prevalence of DCM in pediatric patients with EB&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Methods</span><p id="par0025" class="elsevierStylePara elsevierViewall">We performed a descriptive cross-sectional study of all cases of EB seen at Hospital de San Joan de D&#233;u&#44; a pediatric referral hospital in Barcelona&#44; Spain&#44; between May 1986 and April 2015&#46; Patients with a clinical diagnosis of EB were identified through a database providing access to medical records and photographs&#46; Based on the consensus recommendations for the classification of EB&#44;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">3</span></a> patients were divided into the different EB subtypes based on clinical findings and test results &#40;standard histology&#44; antigen mapping&#44; electron microscopy&#44; genetic studies&#41;&#46; Information was also noted on reasons for referral to cardiology&#44; presence or absence of echocardiographic alterations&#44; presence or absence of DCM &#40;diagnosed by a pediatric cardiologist and defined as ventricular dilation and reduction in systolic function&#41;&#44; and laboratory findings possibly associated with DCM&#46; All the data were recorded retrospectively and based only on information contained in the patients&#8217; charts &#40;history&#44; physical examination&#44; and other tests&#41;&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Results</span><p id="par0030" class="elsevierStylePara elsevierViewall">Seventy-two patients with a diagnosis of EB were identified through the database&#46; After exclusion of those with a doubtful diagnosis or lacking the test results required for correct classification&#44; 57 patients were included&#46; Nineteen had EB simplex&#44; 10 had junctional EB &#40;4 Herlitz and 6 non-Herlitz&#41;&#44; 27 had dystrophic EB &#40;14 dominant and 13 recessive&#41;&#44; and 1 had Kindler syndrome&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Cardiological and echocardiographic assessment had been performed in 19 patients &#40;5 with EB simplex&#44; 1 with Herlitz junctional EB&#44; 4 with non-Herlitz junctional EB&#44; 2 with dominant dystrophic EB&#44; and 7 with recessive dystrophic EB&#41;&#46; Among them&#44; 17 patients showed no signs or symptoms of cardiac involvement and had been referred to the cardiologist to investigate early DCM&#46; Two patients had been referred with a clinical suspicion of cardiomyopathy &#40;confirmed in 1 case&#41;&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">DCM was diagnosed in just 2 patients&#44; both with recessive dystrophic EB&#46; This corresponds to a prevalence of 3&#46;5&#37; &#40;2 of the 57 children analyzed&#41;&#46; The prevalence of DCM by EB subtype was 0&#37; for EB simplex&#44; junctional EB&#44; and Kindler syndrome &#40;0&#47;19&#44; 0&#47;10&#44; and 0&#47;1&#44; respectively&#41; and 7&#46;4&#37; &#40;2&#47;27&#41; for dystrophic EB&#46; Considering only the patients with recessive dystrophic EB&#44; the prevalence of DCM was 15&#46;4&#37; &#40;2&#47;13&#41;&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">In the first of the 2 cases in which a diagnosis of DCM was confirmed&#44; the condition was detected when the patient was 13 years old due to signs and symptoms of heart failure and compatible echocardiographic findings &#40;ventricular dilation and ejection fraction &#91;EF&#93; &#60;<span class="elsevierStyleHsp" style=""></span>50&#37;&#41;&#46; The patient was treated with carvedilol at doses adjusted to symptoms and EF&#46; Initially&#44; the EF remained at around 50&#37;&#44; but it decreased significantly &#40;to 20&#37;&#41; during flares&#46; Four years after diagnosis of DCM&#44; the patient showed progressive worsening and developed kidney failure due to systemic AA amyloidosis&#46; He died at the age of 21 years&#44; with severely deteriorated general health and multiple organ failure&#46; Factors that may have contributed to the development of DCM are chronic anemia&#44; multiple regular blood transfusions&#44; hypoalbuminemia&#44; hypoaminoacidemia&#44; and zinc and selenium deficiency&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The second patient was diagnosed with DCM during a routine echocardiographic examination when she was 3 years of age&#46; She has since been receiving treatment with carvedilol &#40;2<span class="elsevierStyleHsp" style=""></span>mg&#47;12<span class="elsevierStyleHsp" style=""></span>h&#41;&#46; The EF has remained at around 45&#37; and there have been no signs of heart failure&#46; Factors that could have contributed to the development of DCM in this patient include anemia&#44; regular blood transfusions&#44; hypoalbuminemia&#44; hypoaminoacidemia&#44; and carnitine deficiency&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall"><a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a> shows the presence of factors potentially associated with the development of DCM in the patients in our series grouped by EB subtype&#46; Twenty-three patients had at least 1 alteration that may have an etiological role in DCM&#46; Eleven of these patients &#40;48&#37;&#41; had recessive dystrophic EB&#46;</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Discussion</span><p id="par0060" class="elsevierStylePara elsevierViewall">An increased risk of heart disease has been observed in patients with EB&#46; Fine et al&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">2</span></a> published a report on 15 patients with EB and congestive heart failure&#44; and considered that most of the patients also had DCM&#46; The subtypes associated with heart failure were recessive dystrophic EB and non-Herlitz junctional EB&#46; The condition was most common in patients with severe generalized dystrophic recessive EB&#46; The authors found the risk of congestive heart failure to be significantly greater in these patients than in the general population and even greater still in patients with concomitant chronic kidney failure&#46; More recently&#44; Ryan et al&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">14</span></a> investigated the presence of subclinical heart damage and other vascular disorders in patients with recessive dystrophic EB&#46; They found that 40 of the 45 patients analyzed had echocardiographic abnormalities and that 18&#37; had a dilated aortic arch&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">The factors that have been most often linked to the development of cardiopathy in patients with EB are chronic anemia&#44; iron overload&#44; low carnitine and selenium levels&#44; drugs&#44; and concomitant viral infection&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Chronic anemia generally has multiple causes&#46; It responds poorly to dietary supplementation and requires regular blood transfusions&#46; Both chronic anemia and iron overload secondary to repeated transfusions may have a role in the onset of DCM&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">In relation to nutritional deficiencies&#44; EB patients with DCM have been found to have significantly lower levels of carnitine than those without&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a> Selenium supplementation in cases of deficiency has been found to reduce morbidity and mortality in DCM&#46; Nevertheless&#44; no significant differences have been observed between selenium levels in patients with EB and DCM and patients with EB without DCM&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">8</span></a> Thiamine deficiency is another documented cause of DCM&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">8</span></a> Although there have been reports of DCM being reversed with restoration of carnitine levels<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">8</span></a> and of improved symptoms with selenium or thiamine supplementation&#44; micronutrient deficiency alone does not appear to be a causal factor in DCM&#44; but it could be a contributory factor&#46;<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">1&#44;13</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Viral infections can also cause DCM&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a> The heart damage in such cases is generally due to a postinfectious autoimmune process&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">8</span></a> Prodromal viral infection frequently goes unnoticed&#44; as it can precede the onset of DCM by several months&#46; Diagnosis is even more complicated in patients with aggressive forms of EB&#44; who&#44; for varying reasons&#44; may exhibit symptoms such as fever or dyspnea of noncardiac origin&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Cardiotoxic drugs can also exacerbate DCM in patients with EB and should therefore be withdrawn on detection of symptoms suggestive of cardiopathy&#46;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">15</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Apart from mutations in the <span class="elsevierStyleItalic">PLEC1</span> gene<a class="elsevierStyleCrossRefs" href="#bib0160"><span class="elsevierStyleSup">12&#44;16&#44;17</span></a> &#40;coding for plectin&#41; in EB simplex with muscular dystrophy associated with DCM<a class="elsevierStyleCrossRefs" href="#bib0160"><span class="elsevierStyleSup">12&#44;16&#44;17</span></a> and mutations in the <span class="elsevierStyleItalic">DSP</span> gene &#40;coding for desmoplakin&#41; in lethal acantholytic EB&#44;<a class="elsevierStyleCrossRefs" href="#bib0190"><span class="elsevierStyleSup">18&#44;19</span></a> no other genetic alterations have been linked to the development of cardiomyopathy in EB&#46; There is an interesting case of a patient with recessive dystrophic EB who had left ventricular noncompaction cardiomyopathy &#40;LVNC&#41; without DCM&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">20</span></a> The authors indicated that LVNC can progress to DCM in certain cases&#44; and suggested that it might precede DCM in recessive dystrophic EB&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Patients with EB have an increased risk of DCM&#44; and those who develop the disease have a mortality of between 30&#37; and 60&#37;&#46; In patients with recessive dystrophic EB&#44; DCM tends to be detected at around 10 years of age&#44; when it is already at an advanced stage&#46; Such cases are associated with low EF and high mortality&#44; with death generally occurring within 3 months of diagnosis&#46; These data suggest delayed diagnosis&#8212;and more complicated treatment&#8212;of DCM in this setting&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Diagnostic tests that have been proposed for investigating DCM in asymptomatic patients with EB include annual blood tests with complete blood count&#44; albumin levels&#44; total and free carnitine levels&#44; selenium and zinc levels&#44; and cardiac assessment &#40;electrocardiogram and echocardiogram&#41;&#46; Patients with symptoms or alterations in any of the above tests should be urgently referred for cardiac assessment&#46; In addition&#44; thyroid hormone measurement&#44; serology&#44; culture of parvovirus and <span class="elsevierStyleItalic">Coxsackie</span> virus&#44; metabolic tests&#44; and myocardial biopsy when appropriate&#44; should be performed&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Conclusions</span><p id="par0105" class="elsevierStylePara elsevierViewall">DCM must be contemplated as a possible complication of EB&#44; especially in patients with recessive dystrophic or junctional EB&#46; Regular testing at a young age is therefore necessary&#44; as is the correction of alterations that have been linked to heart damage in these patients&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a> Early detection of anomalies will allow prompt initiation of the necessary treatment&#44; thereby slowing disease progress and prolonging survival&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Ethical Disclosures</span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Protection of humans and animals</span><p id="par0110" class="elsevierStylePara elsevierViewall">The authors declare that no tests were carried out in humans or animals for the purpose of this study&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Confidentiality of data</span><p id="par0115" class="elsevierStylePara elsevierViewall">The authors declare that they have followed their hospital&#39;s protocol on the publication of data concerning patients&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Right to privacy and informed consent</span><p id="par0120" class="elsevierStylePara elsevierViewall">The authors declare that no private patient data appear in this article&#46;</p></span></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Conflicts of Interest</span><p id="par0125" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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            0 => "Ecocardiograma"
            1 => "Epiderm&#243;lisis ampollosa"
            2 => "Micronutrientes"
            3 => "Miocardiopat&#237;a dilatada"
          ]
        ]
      ]
    ]
    "tieneResumen" => true
    "resumen" => array:2 [
      "en" => array:3 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Background and objective</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">In recent decades&#44; an association has been reported between epidermolysis bullosa &#40;EB&#41; and dilated cardiomyopathy &#40;DC&#41;&#46; DC is typically in an advanced phase when detected&#44; leading to a poorer prognosis&#46; Our objective was to determine the prevalence of DC in patients with EB seen in Hospital San Joan de D&#233;u in Barcelona&#44; Spain&#44; between May 1986 and April 2015&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">This was a descriptive&#44; cross-sectional chart-review study in which we recorded the type and main subtypes of EB and the presence or absence of DC&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Fifty-seven patients with EB were found&#44; 19 with EB simplex&#44; 10 with junctional EB&#44; 27 with dystrophic EB &#40;14 dominant dystrophic and 13 recessive dystrophic&#41;&#44; and just 1 with Kindler syndrome&#46; DC was detected in only 2 patients with recessive dystrophic EB&#46; Twenty-three patients had presented factors that could have had a causal relationship with the potential onset of DC&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">DC is a possible complication of EB&#44; particularly in recessive dystrophic EB&#46; Periodic follow-up should be performed to make an early diagnosis and start treatment&#46;</p></span>"
        "secciones" => array:4 [
          0 => array:2 [
            "identificador" => "abst0005"
            "titulo" => "Background and objective"
          ]
          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">Introducci&#243;n y objetivos</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">En las &#250;ltimas d&#233;cadas se ha descrito la asociaci&#243;n entre epiderm&#243;lisis ampollosa &#40;EA&#41; y miocardiopat&#237;a dilatada &#40;MD&#41;&#46; Generalmente esta &#250;ltima enfermedad se detecta en fases avanzadas&#44; implicando un peor pron&#243;stico&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Nuestro objetivo consisti&#243; en determinar la prevalencia de MD en los pacientes con EA vistos en el Hospital San Joan de D&#233;u &#40;Barcelona&#41; desde mayo de 1986 a abril de 2015&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">M&#233;todos</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Estudio descriptivo transversal mediante revisi&#243;n de las historias cl&#237;nicas con atenci&#243;n al tipo y subtipos mayores de EA y la existencia o no de MD&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Se recogieron 57 pacientes con diagn&#243;stico de EA&#46; De ellos 19 presentaban EA simple&#44; 10 EA juntural&#44; 27 EA distr&#243;fica &#40;14 EA distr&#243;fica dominante y 13 EA distr&#243;fica recesiva&#41; y existi&#243; un caso de s&#237;ndrome de Kindler&#46; Solo 2 de los pacientes con EA distr&#243;fica recesiva presentaron MD&#46; En 23 de los pacientes con EA existieron factores que podr&#237;an tener una relaci&#243;n causal con el potencial desarrollo de MD&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusi&#243;n</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">La MD puede ser una complicaci&#243;n en los pacientes con EA&#44; mayoritariamente del subtipo de EA distr&#243;fica recesiva&#44; por lo que deben hacerse controles peri&#243;dicos para su temprano diagn&#243;stico y tratamiento&#46;</p></span>"
        "secciones" => array:4 [
          0 => array:2 [
            "identificador" => "abst0025"
            "titulo" => "Introducci&#243;n y objetivos"
          ]
          1 => array:2 [
            "identificador" => "abst0030"
            "titulo" => "M&#233;todos"
          ]
          2 => array:2 [
            "identificador" => "abst0035"
            "titulo" => "Resultados"
          ]
          3 => array:2 [
            "identificador" => "abst0040"
            "titulo" => "Conclusi&#243;n"
          ]
        ]
      ]
    ]
    "NotaPie" => array:1 [
      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0030">Please cite this article as&#58; Batalla A&#44; Vicente A&#44; Bartrons J&#44; Prada F&#44; Fortuny C&#44; Gonz&#225;lez-Ense&#241;at MA&#46; Miocardiopat&#237;a en pacientes con epiderm&#243;lisis ampollosa hereditaria&#46; Actas Dermosifiliogr&#46; 2017&#59;108&#58;544&#8211;549&#46;</p>"
      ]
    ]
    "apendice" => array:1 [
      0 => array:1 [
        "seccion" => array:1 [
          0 => array:4 [
            "apendice" => "<p id="par0135" class="elsevierStylePara elsevierViewall"><elsevierMultimedia ident="upi0005"></elsevierMultimedia></p>"
            "etiqueta" => "Appendix A"
            "titulo" => "Supplementary data"
            "identificador" => "sec0060"
          ]
        ]
      ]
    ]
    "multimedia" => array:4 [
      0 => 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">Source&#58; Fine et al&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">3</span></a>&#59; Hernandez-Martin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">4</span></a></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=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">EB simplex &#40;EBS&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Suprabasal</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Basal</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Lethal acantholytic EB<br>Plakophilin syndrome<br>EBS superficialis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">EBS&#44; localized<br>EBS&#44; Dowling-Meara or herpetiform<br>EBS&#44; generalized &#40;non-Dowling-Meara&#41;<br>EBS with mottled pigmentation<br>EBS with pyloric atresia<br>EBS&#44; Ogna<br>EBS&#44; migratory circinate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Junctional EB &#40;JEB&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Herlitz type</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Non-Herlitz type</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">JEB&#44; non-Herlitz&#44; generalized<br>JEB with pyloric atresia<br>JEB&#44; inversa<br>JEB&#44; late-onset<br>Laryngo-onycho-cutaneous syndrome&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Dystrophic EB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Dominant dystrophic EB &#40;DDEB&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top"><span class="elsevierStyleItalic">Recessive dystrophic EB &#40;RDEB&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">DDEB<br>DDEB&#44; acral<br>DDEB&#44; pretibial<br>DDEB&#44; pruriginosa<br>DDEB&#44; nails only<br>DDEB&#44; bullous dermolysis of the newborn&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">RDEB&#44; severe generalized<br>RDEB&#44; generalized other<br>RDEB&#44; inversa<br>RDEB&#44; pretibial<br>RDEB&#44; pruriginosa<br>RDEB&#44; centripetalis<br>RDEB&#44; bullous dermolysis of the newborn&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mixed EB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Kindler Syndrome&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab1450836.png"
              ]
            ]
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Current Classification of Inherited Epidermolysis Bullosa &#40;EB&#41;&#46;</p>"
        ]
      ]
      1 => 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:3 [
          "leyenda" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Abbreviations&#58; F&#44; female&#59; M&#44; male&#46;</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=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Mean age &#40;SD&#41;&#44; y</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">12&#46;27 &#40;5&#46;60&#41;<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Sex</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">M&#58;F<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>27&#58;31<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Ejection fraction&#44; &#37;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " colspan="2" align="left" valign="top">9-45<a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">EB subtype</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">EB simplex&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&#47;71 &#40;4&#46;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Non-Herlitz junctional EB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&#47;71 &#40;2&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Dystrophic EB &#40;not specified&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&#47;71 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Recessive dystrophic EB&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">65&#47;71 &#40;91&#46;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Outcome</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Death&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">24&#47;71 &#40;33&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Survival&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">44&#47;71 &#40;62&#46;0&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Not specified&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&#47;71 &#40;4&#46;2&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Associated factors mentioned in at least 1 case</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Anemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">22&#47;71 &#40;31&#46;0&#37;&#41;<a class="elsevierStyleCrossRef" href="#tblfn0020"><span class="elsevierStyleSup">d</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Iron overload &#40;secondary hemosiderosis&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&#47;71 &#40;2&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Fluid overload&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&#47;71 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Mutation in <span class="elsevierStyleItalic">PLEC</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&#47;71 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Mutation in <span class="elsevierStyleItalic">DSP</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&#47;71 &#40;2&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Chronic kidney failure&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">6&#47;71 &#40;8&#46;5&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Nutritional deficiency &#40;not specified&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&#47;71 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Carnitine deficiency&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&#47;71 &#40;9&#46;9&#37;&#41;<a class="elsevierStyleCrossRef" href="#tblfn0020"><span class="elsevierStyleSup">d</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Selenium deficiency&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&#47;71 &#40;9&#46;9&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Zinc deficiency&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&#47;71 &#40;2&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Hypoalbuminemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">9&#47;71 &#40;12&#46;7&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Hypoaminoacidemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">2&#47;71 &#40;2&#46;8&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Cardiotoxic drugs &#40;amitriptyline&#44; cisapride&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&#47;71 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Viral cause&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">1&#47;71 &#40;1&#46;4&#37;&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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              "nota" => "<p class="elsevierStyleNotepara" id="npar0015">Minimum and maximum values are given as most studies did not report exact values&#46;</p>"
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          "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Summary of Epidermolysis Bullosa &#40;EB&#41; Cases Associated With Dilated Cardiomyopathy in the Literature<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a></p>"
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          "leyenda" => "<p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">Abbreviations&#58; DDEB&#44; dominant dystrophic epidermolysis bullosa&#59; EBS&#44; epidermolysis bullosa simplex&#59; H-JEB&#44; Herlitz junctional epidermolysis bullosa&#59; nH-JEB&#44; non-Herlitz junctional epidermolysis bullosa&#59; RDEB&#44; recessive dystrophic epidermolysis bullosa&#46;</p>"
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                  <table border="0" frame="\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">75&#37; &#40;3&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">66&#46;7&#37; &#40;4&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">76&#46;9&#37; &#40;10&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;1&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Iron overload&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&#46;3&#37; &#40;1&#47;19&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&#46;4&#37; &#40;2&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;0&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Selenium deficiency&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;19&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&#37; &#40;1&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">30&#46;8&#37; &#40;4&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;0&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Carnitine deficiency&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&#46;4&#37; &#40;2&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;0&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Zinc deficiency&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;19&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&#37; &#40;1&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">46&#46;2&#37; &#40;6&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;0&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypoalbuminemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&#46;3&#37; &#40;1&#47;19&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">75&#37; &#40;3&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">16&#46;7&#37; &#40;1&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">69&#46;2&#37; &#40;9&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;0&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypoaminoacidemia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&#46;3&#37; &#40;1&#47;19&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">25&#37; &#40;1&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&#46;1&#37; &#40;1&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">46&#46;2&#37; &#40;6&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;0&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Kidney failure&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;4&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">0&#37; &#40;0&#47;14&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">7&#46;7&#37; &#40;1&#47;13&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#40;0&#47;1&#41;&nbsp;\t\t\t\t\t\t\n
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                      "titulo" => "Dilated cardiomyopathy in epidermolysis bullosa&#58; A retrospective&#44; multicenter study"
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                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "I&#46; Lara-Corrales"
                            1 => "J&#46;E&#46; Mellerio"
                            2 => "A&#46;E&#46; Martinez"
                            3 => "A&#46; Green"
                            4 => "A&#46;W&#46; Lucky"
                            5 => "R&#46;G&#46; Azizkhan"
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                      "doi" => "10.1111/j.1525-1470.2010.01127.x"
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                        "tituloSerie" => "Pediatr Dermatol"
                        "fecha" => "2010"
                        "volumen" => "27"
                        "paginaInicial" => "238"
                        "paginaFinal" => "243"
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                      "titulo" => "The risk of cardiomyopathy in inherited epidermolysis bullosa"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "J&#46;D&#46; Fine"
                            1 => "M&#46; Hall"
                            2 => "M&#46; Weiner"
                            3 => "K&#46;P&#46; Li"
                            4 => "C&#46; Suchindran"
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                    0 => array:2 [
                      "doi" => "10.1111/j.1365-2133.2008.08697.x"
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                        "tituloSerie" => "Br J Dermatol"
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                0 => array:2 [
                  "contribucion" => array:1 [
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                      "titulo" => "The classification of inherited epidermolysis bullosa &#40;EB&#41;&#58; Report of the Third International Consensus Meeting on Diagnosis and Classification of EB"
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                          "etal" => true
                          "autores" => array:6 [
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                            2 => "E&#46;A&#46; Bauer"
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                  "contribucion" => array:1 [
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                      "titulo" => "Inherited epidermolysis bullosa&#58; From diagnosis to reality"
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                        0 => array:2 [
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                          "autores" => array:2 [
                            0 => "A&#46; Hernandez-Martin"
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Article information

ISSN: 15782190
Original language: English

DOI:10.1016/j.adengl.2017.05.014

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2024 January	58	34	92
2023 December	58	14	72
2023 November	80	24	104
2023 October	98	24	122
2023 September	63	34	97
2023 August	48	17	65
2023 July	67	32	99
2023 June	57	24	81
2023 May	49	26	75
2023 April	36	22	58
2023 March	55	39	94
2023 February	55	31	86
2023 January	45	38	83
2022 December	51	39	90
2022 November	24	35	59
2022 October	68	28	96
2022 September	21	35	56
2022 August	31	35	66
2022 July	19	34	53
2022 June	22	30	52
2022 May	39	36	75
2022 April	48	48	96
2022 March	53	63	116
2022 February	45	45	90
2022 January	40	55	95
2021 December	35	44	79
2021 November	33	58	91
2021 October	61	55	116
2021 September	39	37	76
2021 August	33	44	77
2021 July	39	29	68
2021 June	45	42	87
2021 May	54	68	122
2021 April	116	110	226
2021 March	95	39	134
2021 February	73	53	126
2021 January	63	47	110
2020 December	46	35	81
2020 November	53	37	90
2020 October	36	21	57
2020 September	52	25	77
2020 August	67	43	110
2020 July	39	43	82
2020 June	42	46	88
2020 May	21	9	30
2020 April	23	21	44
2020 March	19	14	33
2020 February	4	0	4
2019 January	1	0	1
2018 December	3	0	3
2018 November	1	0	1
2018 October	2	0	2
2018 September	6	0	6
2018 May	1	0	1
2018 February	13	8	21
2018 January	22	14	36
2017 December	42	11	53
2017 November	31	9	40
2017 October	19	9	28
2017 September	41	16	57
2017 August	43	24	67
2017 July	97	26	123
2017 June	22	14	36

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