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array:24 [ "pii" => "S2173579422000470" "issn" => "21735794" "doi" => "10.1016/j.oftale.2022.03.001" "estado" => "S300" "fechaPublicacion" => "2022-05-01" "aid" => "1968" "copyright" => "Sociedad Española de Oftalmología" "copyrightAnyo" => "2021" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Arch Soc Esp Oftalmol. 2022;97:276-80" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "Traduccion" => array:1 [ "es" => array:19 [ "pii" => "S0365669121002549" "issn" => "03656691" "doi" => "10.1016/j.oftal.2021.04.005" "estado" => "S300" "fechaPublicacion" => "2022-05-01" "aid" => "1968" "copyright" => "Sociedad Española de Oftalmología" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Arch Soc Esp Oftalmol. 2022;97:276-80" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "es" => array:12 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Comunicación corta</span>" "titulo" => "Manifestaciones oftalmológicas en 6 pacientes con síndrome de Williams-Beuren y revisión de la literatura" "tienePdf" => "es" "tieneTextoCompleto" => "es" "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "276" "paginaFinal" => "280" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Ophthalmological manifestations in 6 patients diagnosed with Williams-Beuren syndrome and literature review" ] ] "contieneResumen" => array:2 [ "es" => true "en" => true ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "C. Calleja-García, A. Muñoz-Gallego, A. Dorado-López-Rosado, C. López-López, P. Tejada-Palacios" "autores" => array:5 [ 0 => array:2 [ "nombre" => "C." "apellidos" => "Calleja-García" ] 1 => array:2 [ "nombre" => "A." "apellidos" => "Muñoz-Gallego" ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Dorado-López-Rosado" ] 3 => array:2 [ "nombre" => "C." "apellidos" => "López-López" ] 4 => array:2 [ "nombre" => "P." "apellidos" => "Tejada-Palacios" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2173579422000470" "doi" => "10.1016/j.oftale.2022.03.001" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579422000470?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669121002549?idApp=UINPBA00004N" "url" => "/03656691/0000009700000005/v2_202205042123/S0365669121002549/v2_202205042123/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2173579422000482" "issn" => "21735794" "doi" => "10.1016/j.oftale.2022.03.002" "estado" => "S300" "fechaPublicacion" => "2022-05-01" "aid" => "1966" "copyright" => "Sociedad Española de Oftalmología" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Arch Soc Esp Oftalmol. 2022;97:281-5" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Short communication</span>" "titulo" => "Acute Horner syndrome secondary to glandular fever due to Epstein Barr virus infection" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "281" "paginaFinal" => "285" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Horner agudo secundario a mononucleosis infecciosa por virus de Epstein Barr" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 479 "Ancho" => 1505 "Tamanyo" => 136444 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Photographs showing the patient’s anisocoria and ptosis, and the changes after the eye drops test.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A: Condition prior to diagnostic test with 0.5% apraclonidine eye drops. Anisocoria can be observed with a pupillary diameter of 4 mm in RE and 2 mm in LE.</p> <p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">B: Examination 30 min after applying a drop of 0.5% apraclonidine eye drops in each eye. Pupillary dilatation of the LE with decreased ptosis and slight miosis of the RE can be observed.</p> <p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">C: State prior to the localisation test with 1% phenylephrine eye drops. Anisocoria can be observed with a pupillary diameter of 4 mm in RE and 2 mm in LE.</p> <p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">D: Examination 30 min after applying a drop of phenylephrine 1% eye drops to each eye. It shows a similar right pupil and a more dilated left pupil, with a slight decrease in ptosis, suggesting Horner’s syndrome with a probable postganglionic location.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M.M. Schilt-Catafal, L. 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"apellidos" => "Duch-Samper" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0365669121002525" "doi" => "10.1016/j.oftal.2021.05.002" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669121002525?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579422000482?idApp=UINPBA00004N" "url" => "/21735794/0000009700000005/v1_202205061311/S2173579422000482/v1_202205061311/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S2173579422000172" "issn" => "21735794" "doi" => "10.1016/j.oftale.2021.01.012" "estado" => "S300" "fechaPublicacion" => "2022-05-01" "aid" => "1954" "copyright" => "Sociedad Española de Oftalmología" "documento" => "article" "crossmark" => 1 "subdocumento" => "rev" "cita" => "Arch Soc Esp Oftalmol. 2022;97:264-75" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:12 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review</span>" "titulo" => "The association between intestinal microbiome and autoimmune uveitis" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "264" "paginaFinal" => "275" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "La asociación entre microbioma intestinal y uveítis autoinmune" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "D. Kalogeropoulos, R. Barry, C. Kalogeropoulos" "autores" => array:3 [ 0 => array:2 [ "nombre" => "D." "apellidos" => "Kalogeropoulos" ] 1 => array:2 [ "nombre" => "R." "apellidos" => "Barry" ] 2 => array:2 [ "nombre" => "C." "apellidos" => "Kalogeropoulos" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0365669121002409" "doi" => "10.1016/j.oftal.2021.01.019" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669121002409?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579422000172?idApp=UINPBA00004N" "url" => "/21735794/0000009700000005/v1_202205061311/S2173579422000172/v1_202205061311/en/main.assets" ] "en" => array:19 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Short communication</span>" "titulo" => "Ophthalmological manifestations in 6 patients diagnosed with Williams-Beuren syndrome and literature review" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "276" "paginaFinal" => "280" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "C. Calleja-García, A. Muñoz-Gallego, A. Dorado-López-Rosado, C. López-López, P. Tejada-Palacios" "autores" => array:5 [ 0 => array:3 [ "nombre" => "C." "apellidos" => "Calleja-García" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "A." "apellidos" => "Muñoz-Gallego" "email" => array:1 [ 0 => "ali_munozg@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "A." "apellidos" => "Dorado-López-Rosado" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "C." "apellidos" => "López-López" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "P." "apellidos" => "Tejada-Palacios" "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Oftalmología, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Instituto de investigación i+12, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Departamento de Inmunología, Oftalmología y ORL, Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Manifestaciones oftalmológicas en 6 pacientes con síndrome de Williams-Beuren y revisión de la literatura" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The Williams-Beuren syndrome (WBS) or Williams syndrome is a congenital disorder caused by a 1.5–1.8 Mb hemizygous microdeletion of 26–28 contiguous genes located on the long arm of chromosome 7 (7q11.23), including the elastin gene.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> Atypical deletions smaller than 1.5 Mb present in 1–2% of individuals with WBS have been described and have been associated with specific clinical, cognitive and behavioural features,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> although this genotype-phenotype correlation has yet to be studied for meaningful results.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Most cases of WBS are sporadic (99%), although an autosomal dominant inheritance pattern<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> has also been described. Its incidence is one in 10,000–20,000 live births<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and it presents systemic alterations including characteristic facial features (elfin face), cardiovascular (most frequently supravalvular aortic stenosis),<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> neurological, ophthalmological, connective tissue, personality, infantile hypercalcemia, etc.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical cases</span><p id="par0010" class="elsevierStylePara elsevierViewall">The clinical characteristics (systemic and ophthalmological) of 6 patients with genetic diagnosis (by in situ hybridisation technique) aged between 1 and 5 years, 5 of them male, were reviewed. All had phenotypic features consistent with WBS (broad forehead, epicanthus, narrow palpebral fissure, anteverted nose, pendulous cheeks, thickened lips, skin hyperlaxity, hoarse cry and joint hyperlaxity). In the cardiovascular field, five patients had stenosis of one or two pulmonary branches of greater or lesser severity, of which two cases also had supravalvular aortic stenosis and one case had an atrial septal defect of the <span class="elsevierStyleItalic">ostium secundum</span> type. Only one case had supravalvular aortic stenosis without other cardiac abnormalities. Four patients required cardiovascular surgical treatment. Other findings included two cases of hernias (inguinal and ventral), one case of diaphragmatic Morgagni hernia, two cases of phimosis (one with scrotal implantation anomaly), one case of obstructive hydrocephalus secondary to subarachnoid hemorrhage requiring ventriculoperitoneal shunt placement and one case of idiopathic hypercalciuria with nephrocalcinosis.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The ophthalmological examination included visual acuity (VA) (in preverbal age [up to 2 years] with the LEA paddle preferential gaze test, and in verbal age [2–5 years] with the Pigassou test and later with the Snellen test), cycloplegic refraction (30 min after instillation of 3 drops of 1% cyclopentolate every 15 min) using a portable autorefractometer and retinoscopy, slit lamp examination, fundus examination with indirect ophthalmoscopy, ocular motility examination, stereopsis test (TNO test) and visuoperceptual examination using the PreVias questionnaire, Mailbox test and Ishihara test. The ophthalmological findings are summarised in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Regarding VA, only one case of amblyopia was found, which was corrected with optical correction and occlusions. The most frequent refractive error was hypermetropia (n = 6) and astigmatism (n = 5). Anterior pole examination ruled out the presence of lens opacities and stellate pattern of the iris in all cases. Iris colour was brown in 5 patients and blue in one case. No alterations were found in the fundus either. Ocular motility disturbances were observed in 2 cases: 1 case of right eye amblyopia with near exophoria of -8 prismatic dioptres and mild right inferior oblique hyperfunction (IOHF) which was treated with optical correction and convergence exercises; and another case of congenital endotropia (measured with correction) of +30 dioptres prismatic with mild IOHF of both eyes, which was treated with 2 sessions of botulinum toxin injection (5 UI) in both medial rectus with orthotropia result. The TNO test showed the absence of stereopsis in 5 patients. Only one patient showed unquantifiable stereopsis at the first examination (due to lack of cooperation), which is currently 480'' of arc.</p><p id="par0025" class="elsevierStylePara elsevierViewall">All had epicanthus and one case had bilateral congenital lacrimal duct obstruction which resolved after bilateral probing.</p><p id="par0030" class="elsevierStylePara elsevierViewall">At the visuoperceptual level, 4 patients showed visual maturity delay with compromised visuomotor coordination and visual processing, one patient showed normal visual cognitive development and another did not cooperate with testing. All patients exhibited intellectual developmental delay requiring school support. Three patients showed a tendency towards inattention and hyperactivity. In one patient there was also a neglect in the right lower temporal quadrant assessed by Stycar test due to an episode of prepontine and prebulbar subarachnoid hemorrhage, which improved completely after visuospatial stimulation exercises.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">BWS is a rare congenital disorder with multilevel involvement. This article describes the different systemic and ophthalmological alterations present in a series of 6 patients.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Facial features include findings such as broad forehead, low nasal bridge, epicanthus, anteverted nose, narrow palpebral fissure, prominent cheeks and thickened lips.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4</span></a> All our patients had these features. Hoarse voice, presence of intestinal and inguinal hernias, bladder diverticula and joint hyperlaxity<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> are also characteristic as shown by the patients in our study. Other phenotypic features that may be present in SWB and were not present in our cases are malar hypoplasia, micrognathia, dental malocclusion, dental enamel hypoplasia and long nasal philtrum.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">According to the literature, cardiovascular anomalies are present in 80% of cases, with supravalvular aortic stenosis being the most frequent alteration, present in 64% of our cases.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In the present study all patients had cardiovascular abnormalities, the most frequent abnormality being pulmonary branch stenosis (present in 5 patients), followed by supravalvular aortic stenosis present in 3. Hypercalcemia has been associated with this syndrome, although it is present in only 15% of patients.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In our study, one patient had hypercalcemia with associated nephrocalcinosis.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Weber et al. described in their case series that 70% of patients required at least one surgical intervention, the most frequent being inguinal hernia surgery (37%), followed by cardiovascular surgery (13%) and strabismus surgery (13%).<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> All our patients required at least one surgical intervention, the most frequent being cardiovascular surgery (50%) and hernia repair surgery (50%), followed by phimosis surgery (30%). Two patients required ophthalmological intervention: in one case botulinum toxin injection and in another case bilateral lacrimal duct probing.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Regarding ophthalmological manifestations, strabismus is probably the most frequent finding. Winter and Greenberg found 54% and 29% strabismus in their series respectively. Other authors found an incidence of 78%.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In 92% of patients endotropia is the most frequent form.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,5,6</span></a> In the present study the incidence of strabismus is 33.3% (n = 2), with one case of congenital endotropia and one case of exophoria. Weber et al. described the presence of dissociated vertical and horizontal deviation associated with strabismus in these patients.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> We found one case with bilateral and one unilateral IOHF.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Regarding VA, the literature shows that most cases have good VA. Weber et al. describe only one case of amblyopia<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> in their case series. In the present study only one case of unilateral amblyopia was found. The most frequent refractive defect is hypermetropia according to pediatric age, present in 50–87% of cases,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> followed by astigmatism present in 13%–50% of patients.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In our study the most frequent refractive defect was hyperopia followed by astigmatism. There are some reported cases of keratoconus associated with SWB<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> and it is proposed that there may be a genetic association between both. In the only patient with astigmatism greater than 3 dioptres unilaterally, corneal topography could not be performed due to lack of cooperation, although no alterations of the anterior segment were found.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Other ophthalmological alterations include the presence of a stellate pattern of the iris in 50–74% of cases,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> being easier to detect in blue irises. In our study, five patients had brown irises and one had blue irises, but in none of the cases was this pattern identified. Fundus abnormalities include vascular tortuosity in 22–27% of cases.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,4,5</span></a> Other abnormalities include optic disc hypoplasia, peripheral retinal degenerations<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> or cone and rod dystrophy.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> None of these disorders were found in our series. Weber et al. reported a 23% incidence of epiphora with nasolacrimal duct obstruction,<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a> cataracts and ptosis.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> One case of congenital obstruction of the lacrimal duct was found in our series and no patient showed lens opacifications or ptosis.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Patients with WBS exhibit neurodevelopmental disturbances characterised by cognitive-behavioural defects.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Visuoperceptual disorders include abnormalities of visuospatial perception and difficulty in figure-background discrimination due to disturbances in the dorsal longitudinal fasciculus of the brain.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Impaired binocular function together with poor visuospatial skills have been reported, which may influence the development of the occipitoparietal cortex.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> In our series, 4 patients showed visuoperceptive disturbances and visual maturation delay and 5 of them did not have stereopsis. Weber et al. described psychomotor retardation in all patients evaluated and attention deficit in 20% of the cases. In our study all patients showed school retardation requiring learning support. In addition, 3 showed attention deficit with hyperactivity and in 2 cases emotional disturbances such as overreactions, low self-esteem and irritability.</p><p id="par0075" class="elsevierStylePara elsevierViewall">Another important aspect of SWB patients is that they are at increased risk of serious adverse effects due to sedation or general anesthesia. An increased risk of coronary ischemia has been described in cases of supravalvular aortic stenosis, as well as an increased risk of malignant hyperthermia, which in some cases has led to sudden death.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0080" class="elsevierStylePara elsevierViewall">BWS is a rare disorder, with systemic alterations at different levels, some of which are of vital importance. Ophthalmologically they can present different affectations and early evaluation and long-term follow-up is necessary to ensure the best development of these patients.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflict of interest</span><p id="par0085" class="elsevierStylePara elsevierViewall">No conflicts of interest were declared by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1704074" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1508576" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1704075" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1508575" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinical cases" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusion" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflict of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2020-11-08" "fechaAceptado" => "2021-04-06" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1508576" "palabras" => array:5 [ 0 => "Williams-Beuren syndrome" 1 => "Williams syndrome" 2 => "Ophthalmology" 3 => "Strabismus" 4 => "Visuospatial alterations" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1508575" "palabras" => array:5 [ 0 => "Síndrome de Williams-Beuren" 1 => "Síndrome de Williams" 2 => "Oftalmología" 3 => "Estrabismo" 4 => "Alteraciones visoespaciales" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Medical history of 6 patients diagnosed with Williams-Beuren Syndrome (SWB) in pediatric age was revised.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">All the patients presented characteristic elf facies and cardiovascular abnormalities. All presented good visual acuity, except one case of unilateral amblyopia. The most frequent refractive error was hyperopia (n = 6; 100%) and astigmatism (n = 5; 83.3%). Ocular motility alterations were found in 2 patients (1 case of exophoria with hyperfunction of right inferior oblique and another of congenital endotropia with bilateral hyperfunction of inferior oblique). On the cognitive function, 66.7% (n = 4) had visoperceptive disorders. Other findings were epicanthus (n = 6; 100%) and congenital obstruction of the nasolacrimal duct with unilateral epiphora (n = 1; 16.7%).</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">SWB is a rare disorder with complex ophthalmological and systemic manifestations. For this reason, ophthalmological follow-up of these children is recommended.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Se revisó la historia clínica de 6 pacientes diagnosticados de síndrome de Williams-Beuren en la edad pediátrica.</p><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Todos los pacientes presentaron la facies de elfo característica y anomalías cardiovasculares. Todos presentaron buena agudeza visual, excepto un caso de ambliopía unilateral. El error refractivo más frecuente fue la hipermetropía (n = 6; 100%) y el astigmatismo (n = 5; 83,3%). En 2 pacientes se encontraron alteraciones de la motilidad ocular (un caso de exoforia con hiperfunción de oblicuo inferior derecho y otro de endotropía congénita con hiperfunción del oblicuo inferior bilateral). Cognitivamente un 66,7% (n = 4) tenía alteraciones visoperceptivas. Otros hallazgos fueron epicantus (n = 6; 100%) y obstrucción congénita del conducto nasolagrimal con epífora unilateral (n = 1; 16,7%).</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Williams-Beuren es un trastorno poco frecuente con manifestaciones oftalmológicas y sistémicas complejas. Por ello, es recomendable realizar un seguimiento oftalmológico a estos niños.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Calleja-García C, Muñoz-Gallego A, Dorado-López-Rosado A, López-López C, Tejada-Palacios P. Manifestaciones oftalmológicas en 6 pacientes con síndrome de Williams-Beuren y revisión de la literatura. Arch Soc Esp Oftalmol. 2022. <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.oftal.2021.04.005">https://doi.org/10.1016/j.oftal.2021.04.005</span></p>" ] ] "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">c.p.g.: cycles per degree; PD: prismatic dioptres; IOHF: inferior oblique hyperfocal; LEA: preferential gaze test; BCVA: best corrected visual acuity; RE: right eye; LE: left eye.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Patient \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Sex \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Age 1st ophthalmological examination \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Age at last ophthalmological examination \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Initial BCVA \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Current BCVA \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Refraction with cycloplegia \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Strabismus \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Degree of stereopsis with TNO test \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Cognitive-behavioural assessment (pre-test. Ishihara test. letter-box test). \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="center" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Other \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleBold">1</span></td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Male</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 years</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 years and 10 months</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pigassou \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Snellen \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: +3.50−1.25 × 2° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Exophoria-8 DP near RE \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">480" arc</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Uncooperative</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Initial amblyopia RE recovered \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/32 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/16 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: +3.25−1.00 × 163° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mild IOHF RE \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Epicanthus</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RO: 20/16 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleBold">2</span></td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Male</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1 year and 8 months</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5 years and 7 months</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">READ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pigassou \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: +3.75−3.00 × 6° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Endotropia +30 DP congenital \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No stereopsis</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Visuoperceptual disorder</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Epicanthus</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 12 c.p.g. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: +6.00−4.50 × 168° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mild bilateral IOHF \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 12 c.p.g. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleBold">3</span></td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Male</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1 year and 1 month</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5 years and 10 months</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">READ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pigassou \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: +1.25−2.23 × 105° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No stereopsis</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Visuoperceptual disorder</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Bilateral congenital lacrimal duct obstruction \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 12 c.p.g. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/16 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: +3.00−2.50 × 55° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Epicanthus</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 12 c.p.g. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RO: 20/16 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleBold">4</span></td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Woman</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2 years and 6 months</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7 years and 9 months</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">READ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Snellen \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: +2.75 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No stereopsis</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Visuoperceptual disorder</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Epicanthus</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 32 c.p.g \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/16 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: +2.25 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 32 c.p.g \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RO: 20/16 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleBold">5</span></td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Male</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2 years</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 years and 10 months</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">READ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pigassou \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: +2.75−1.00 × 65° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No stereopsis</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Normal visual cognitive development</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Epicanthus \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 12 c.p.g. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/16 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: +3.25−2.25 × 100° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Negligence of the lower right quadrant of the visual field</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 12 c.p.g. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead rowgroup " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleBold">6</span></td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Woman</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4 years</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 years</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pigassou \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Snellen \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: +2.50−1.50 × 175° \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No stereopsis</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Visuoperceptual disorder</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="3" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Epicanthus</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">RE: 20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: +2.25−1.23 × 175° \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE:20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">LE: 20/20 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2896187.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Ophthalmological characteristics of 6 patients with Williams-Beuren Syndrome.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Ocular features in 16 Brazilian patients with Williams-Beuren syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M.M. Viana" 1 => "M. Frasson" 2 => "H. Galvão" 3 => "L.L. Leão" 4 => "M. Stofanko" 5 => "H. Gonçalves-Dornelas" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3109/13816810.2013.873941" "Revista" => array:6 [ "tituloSerie" => "Ophthalmic Genet" "fecha" => "2015" "volumen" => "36" "paginaInicial" => "234" "paginaFinal" => "238" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24417560" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Williams syndrome: ophthalmological examination and review of systemic manifestations" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "S.L. Weber" 1 => "R.B. Souza" 2 => "L.G. Ribeiro" 3 => "M.F. Tavares" 4 => "M. 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