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Se observa la arteria mamaria interna y el hematoma que se origina de la rotura de la misma.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Kevin Álvarez, López Jordi, Hernández Jose Angel" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Kevin" "apellidos" => "Álvarez" ] 1 => array:2 [ "nombre" => "López" "apellidos" => "Jordi" ] 2 => array:2 [ "nombre" => "Hernández" "apellidos" => "Jose Angel" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2173574319301121" "doi" => "10.1016/j.reumae.2017.08.011" "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/S2173574319301121?idApp=UINPBA00004M" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1699258X17302334?idApp=UINPBA00004M" 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"aff0010" ] ] ] 2 => array:3 [ "nombre" => "Jose Angel" "apellidos" => "Hernández" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Cardiología, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Las Palmas, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neumología, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Las Palmas, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Reumatología, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Las Palmas, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Hemotórax en síndrome de Ehlers-Danlos tipo vascular" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1216 "Ancho" => 1267 "Tamanyo" => 114300 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">CT image of the thorax, sagittal view. The internal mammary artery and the haematoma that originates from its rupture can be observed.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Type IV or vascular (EDS IV) Ehlers-Danlos syndrome is characterised by alterations in type III collagen metabolism by mutations of the COL3A1 gene. It usually starts at a young age with spontaneous ruptures of medium-calibre arteries. Its diagnosis requires a high level of suspicion in order to correctly direct the clinical history. Its management involves a series of measures to prevent trauma or risk situations. There is no effective pharmacological treatment, only β-blockers have been tried to prevent catastrophic bleeding.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical observation</span><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 50-year-old male who was admitted to the pneumology department with a diagnosis of pneumonia associated with significant parapneumonic effusion; the patient reported prior symptoms of influenza, a coughing spell that triggered severe pain in the right haemothorax and presyncope symptoms.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Personal history of note included: smoker of 30 packs/year, hypertension under treatment, an episode of left carotid-cavernous fistula aged 25 treated by embolisation. At 37 years he presented with symptoms of sudden abdominal pain resulting in spontaneous haemoperitoneum secondary to rupture of the round ligament, confirmed in emergency surgery. During the surgery, splenic rupture occurred which required splenectomy.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Family history of a father who died aged 30 years due to sudden death from an unexplained case. Paternal grandmother died aged 50 from internal bleeding secondary to a ruptured axillary artery from mild trauma.</p><p id="par0025" class="elsevierStylePara elsevierViewall">On physical examination: cutis marmorata, abundant haematomas on the upper limbs and thorax, secondary to banal trauma, slight hyperlaxity in metacarpophalangeal joints, without typical facies.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Pleural drainage of dense haematic content was performed, after which a computed tomography (CT) of the thorax was performed which revealed the presence of a ruptured aneurysm of the right internal mammary artery, causing ipsilateral haemothorax (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). He also had an aneurysm in the common hepatic artery. Embolisation of the ruptured aneurysm was performed as treatment.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">When EDS is suspected, a confirmatory genetic study is carried out for the presence of heterozygosis in the splicing variant c.636+1G>T in gene COL3A1. The variant described is not recorded on the databases consulted, but similar variants in the same point that are associated with type IV EDS are. It is transmitted with autosomal dominant inheritance. Once the suspected diagnosis has been confirmed, the study is continued and the family is given genetic counselling, and treatment is started with β-blockers and controls in outpatient clinics. After one year of follow-up, the patient remains asymptomatic with no new bleeding episodes, and under treatment with β-blockers.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">EDS is a hereditary disease caused by mutations of the collagen gene with different subtypes.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> EDS IV is the most severe form. It is inherited as an autosomal dominant trait and involves procollagen type III deficiency by mutation of the COL3A1 gene.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> It constitutes between 5% and 10% of all cases of EDS, with a prevalence of 1/50 000–1/200 000 inhabitants. It is a potentially fatal variant, increasing the risk of visceral and vascular rupture. The absence of hyperelasticity of large joints stands out, although the small distal joints of the hand may show moderate elasticity.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Diagnosis is based on the sum of clinical criteria and is confirmed by genetic study.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> There is no effective treatment, although the administration of β-blockers has been trialled previously to reduce catastrophic bleeding.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Follow-up includes minimising trauma, identifying patients and creating a care group around them, forming and facilitating individualised emergency plans, centralising treatment in referral centres, maintaining normal blood pressure and, in the event of high blood pressure, treating it strictly and monitoring the vascular tree by means of ultrasound, CT-arteriography or nuclear magnetic resonance on an annual basis.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusions</span><p id="par0050" class="elsevierStylePara elsevierViewall">This is a rare disease, difficult to diagnose. With regard to genetic counselling and emergency management of the patient in the emergency department, it is advisable to know the different possible manifestations to reach a quick suspicion and avoid complications. It is important to avoid trauma, and diagnostic or therapeutic vascular techniques that are not strictly necessary.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that neither human nor animal testing has been carried out in this research.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that they have complied with their work centre protocols for the publication of patient data.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflict of interests</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors have no conflict of interests to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:11 [ 0 => array:3 [ "identificador" => "xres1268388" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1173987" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1268387" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1173988" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinical observation" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusions" ] 8 => array:3 [ "identificador" => "sec0025" "titulo" => "Ethical disclosures" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0030" "titulo" => "Protection of human and animal subjects" ] 1 => array:2 [ "identificador" => "sec0035" "titulo" => "Confidentiality of data" ] 2 => array:2 [ "identificador" => "sec0040" "titulo" => "Right to privacy and informed consent" ] ] ] 9 => array:2 [ "identificador" => "sec0045" "titulo" => "Conflict of interests" ] 10 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-07-11" "fechaAceptado" => "2017-08-10" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1173987" "palabras" => array:3 [ 0 => "Ehlers-Danlos syndrome" 1 => "EDS IV" 2 => "Vascular Ehlers-Danlos syndrome" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1173988" "palabras" => array:3 [ 0 => "Síndrome de Ehlers-Danlos" 1 => "SED IV" 2 => "Síndrome de Ehlers-Danlos tipo vascular" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Vascular Ehlers-Danlos syndrome (EDS <span class="elsevierStyleSmallCaps">IV</span>) is a rare genetic disorder characterised by an alteration in the COL3A1 gene which encodes type <span class="elsevierStyleSmallCaps">III</span> collagen. It is the most common type of collagen in vessels of medium size and certain organs such as the intestines and the uterus. The alteration of this type of collagen produces aneurisms and ruptures of vessels and organs. A high level of clinical suspicion is required for diagnosis. It is a complex disease whose management requires a multidisciplinary team to treat the different complications that may occur.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report the case of a 50-year-old man diagnosed with EDS <span class="elsevierStyleSmallCaps">IV</span> detected incidentally after haemothorax secondary to a coughing spell.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Ehlers-Danlos de tipo vascular (SED <span class="elsevierStyleSmallCaps">IV</span>) se trata de una rara alteración genética causada por una alteración del gen COL3A1 que codifica el colágeno tipo <span class="elsevierStyleSmallCaps">III</span>. Este es el tipo de colágeno más frecuente en los vasos de mediano calibre y en algunos órganos como intestino y útero. Su alteración produce, entre otros, aneurismas y roturas de vasos y órganos. Para su diagnóstico se requiere de un alto nivel de sospecha clínica. Se trata de una enfermedad de manejo complejo que requiere de un equipo multidisciplinar para tratar las diferentes complicaciones que pueden acontecer.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Se presenta el caso de un paciente varón de 50 años diagnosticado de SED <span class="elsevierStyleSmallCaps">IV</span> de forma incidental tras hemotórax secundario a acceso de tos.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Álvarez K, López J, Hernández JA. Hemotórax en síndrome de Ehlers-Danlos tipo vascular. Reumatol Clin. 2019;15:e128–e129.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1216 "Ancho" => 1267 "Tamanyo" => 114300 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">CT image of the thorax, sagittal view. The internal mammary artery and the haematoma that originates from its rupture can be observed.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "COL3A1 mutations cause variable clinical phenotypes including acrogeria and vascular rupture" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "F.M. Pope" 1 => "P. Narcisi" 2 => "A.C. Nicholls" 3 => "D.P. Germain" 4 => "G. Pals" 5 => "A.J. 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Year/Month | Html | Total | |
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2024 November | 3 | 5 | 8 |
2024 October | 43 | 39 | 82 |
2024 September | 52 | 17 | 69 |
2024 August | 48 | 42 | 90 |
2024 July | 39 | 28 | 67 |
2024 June | 56 | 20 | 76 |
2024 May | 72 | 26 | 98 |
2024 April | 38 | 16 | 54 |
2024 March | 45 | 25 | 70 |
2024 February | 35 | 29 | 64 |
2024 January | 38 | 21 | 59 |
2023 December | 23 | 22 | 45 |
2023 November | 31 | 25 | 56 |
2023 October | 50 | 30 | 80 |
2023 September | 71 | 33 | 104 |
2023 August | 43 | 12 | 55 |
2023 July | 32 | 16 | 48 |
2023 June | 31 | 26 | 57 |
2023 May | 29 | 19 | 48 |
2023 April | 18 | 6 | 24 |
2023 March | 50 | 18 | 68 |
2023 February | 38 | 22 | 60 |
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2020 December | 23 | 29 | 52 |
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2020 January | 1 | 0 | 1 |