Clinical characteristics of children with scleroderma in a referral hospital
Características clínicas de niños con esclerodesmia en un hospital de referencia
Zoilo Morel
, Eduardo Benadón, Enrique Faugier, María del Rocío Maldonado
Corresponding author
Departamento de Reumatología Pediátrica, Hospital Infantil de México Federico Gómez, México DF, Mexico
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array:2 [ "en" => true "es" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Daniel Roig Vilaseca, Montserrat Núñez Juárez, Esther Núñez Juárez, José Luis del Val García, Àlex Sánchez Plà, Maria Bonet Llorach" "autores" => array:7 [ 0 => array:2 [ "nombre" => "Daniel Roig" "apellidos" => "Vilaseca" ] 1 => array:2 [ "nombre" => "Montserrat Núñez" "apellidos" => "Juárez" ] 2 => array:2 [ "nombre" => "Esther Núñez" "apellidos" => "Juárez" ] 3 => array:2 [ "nombre" => "José Luis del" "apellidos" => "Val García" ] 4 => array:2 [ "nombre" => "Àlex Sánchez" "apellidos" => "Plà" ] 5 => array:2 [ "nombre" => "Maria Bonet" "apellidos" => "Llorach" ] 6 => array:1 [ "colaborador" => "on behalf of the ARQUALIS group" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S217357430970111X?idApp=UINPBA00004M" "url" => "/21735743/0000000500000004/v1_201305061557/S217357430970111X/v1_201305061557/en/main.assets" ] "en" => array:14 [ "idiomaDefecto" => true "titulo" => "Clinical characteristics of children with scleroderma in a referral hospital" "tieneTextoCompleto" => 0 "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "158" "paginaFinal" => "162" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Zoilo Morel, Eduardo Benadón, Enrique Faugier, María del Rocío Maldonado" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Zoilo" "apellidos" => "Morel" "email" => array:1 [ 0 => "zoiloma@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Eduardo" "apellidos" => "Benadón" ] 2 => array:2 [ "nombre" => "Enrique" "apellidos" => "Faugier" ] 3 => array:2 [ "nombre" => "María del" "apellidos" => "Rocío Maldonado" ] ] "afiliaciones" => array:1 [ 0 => array:1 [ "entidad" => "Departamento de Reumatología Pediátrica, Hospital Infantil de México Federico Gómez, México DF, Mexico" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Características clínicas de niños con esclerodesmia en un hospital de referencia" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2008-08-09" "fechaAceptado" => "2008-11-19" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec112132" "palabras" => array:5 [ 0 => "Systemic scleroderma" 1 => "Localized scleroderma" 2 => "Morphea" 3 => "Raynaud's phenomenon" 4 => "Methotrexate" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec112131" "palabras" => array:5 [ 0 => "Esclerodermia sistémica" 1 => "Esclerodermia localizada" 2 => "Morfea" 3 => "Fenómeno de Raynaud" 4 => "Metotrexato" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span class="elsevierStyleSectionTitle">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Scleroderma is an autoimmune disease that involves the connective tissue characterized by skin fibrosis, classified as localized and systemic (participation of one or more internal organs). The primary objective of this study is to describe and analyze the clinical and laboratory findings in a group of children diagnosed with scleroderma at a referral hospital.</p> <span class="elsevierStyleSectionTitle">Material and methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Extraction of data from clinical charts of children with scleroderma in the rheumatology department at the Hospital Infantil de México Federico Gómez, between January 2000 and December 2007.</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Sixty-two patients were included in the group. All of them completed the classification criteria for juvenile sclerodema, both systemic and localized. The mean age at diagnosis was 7.8 (1–14) years. The mean time from disease onset to diagnosis, based on clinical manifestations, was 23 months. The lesions found were: linear scleroderma (42%), mixed morphea (22%), circumscribed morphea (19%), generalized morphea (13%), and panclerotic morphea (4%). Involvement associated with systemic scleroderma was gastrointestinal 100% (18 patients), pulmonary 100% (18/18), Raynaud's phenomenon 89% (16/18), proximal sclerosis 89% (16/18), sclerodactilia 67% (12/18), joint pain 28% (5/18), calcinosis 56% (10/18). Positive antinuclear antibodies (ANA) were present in 14/62 (23%) patients (10 with systemic range and 4 localized), antiSCL 70 in 2/62 (4%) cases. The most common drug used was methotrexate.</p> <span class="elsevierStyleSectionTitle">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">The most common skin lesions found were linear morphea, followed by the mixed and circumscribed types. In systemic scleroderma the most involved systems are the gastrointestinal, respiratory, and vascular (associated with Raynaud's phenomenon). There is a special need for knowledge of this disease in first contact physicians for a faster and better diagnosis and treatment, in order to avoid complications. It is also necessary to improve resources in developing countries for complimentary studies, classification, treatment, and follow-up.</p>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span class="elsevierStyleSectionTitle">Introducción</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">La esclerodermia es una enfermedad del tejido conectivo, autoinmunitaria y caracterizada por fibrosis de la piel; además, puede ser localizada o sistémica (participación de uno o de más órganos internos). El objetivo del presente estudio es describir y analizar las características clínicas y de laboratorio observadas en un grupo de niños con esclerodermia en un hospital de referencia.</p> <span class="elsevierStyleSectionTitle">Material y métodos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Recopilación de datos de los expedientes clínicos de niños con diagnóstico de esclerodermia del Departamento de Reumatología del Hospital Infantil de México Federico Gómez durante el período comprendido entre los años 2000 y 2007.</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Se incluyó a 62 pacientes que cumplieron con los criterios de clasificación preliminar para esclerodermia juvenil, localizada y sistémica. La edad media al diagnóstico fue de 7,8 años (1–14). La duración media de la enfermedad al diagnóstico fue de 23 meses. Las lesiones encontradas fueron esclerodermia lineal (42%), morfea mixta (22%), morfea circunscripta (19%), morfea generalizada (13%) y morfea panesclerótica (4%). Los hallazgos asociados a esclerodermia sistémica (ES) fueron afección gastrointestinal en 18 de 18 pacientes (100%), afección pulmonar en 18 de 18 pacientes (100%), fenómeno de Raynaud en 16 de 18 pacientes (89%), esclerosis proximal en 16 de 18 pacientes (89%), esclerodactilia en 12 de 18 pacientes (67%), calcinosis en 10 de 18 pacientes (56%) y afección articular en 5 de 18 pacientes (28%). Se hallaron anticuerpos antinucleares positivos en 14 de 62 pacientes (23%) (10 con ES y 4 con esclerodermia localizada) y anticuerpos anti-Scl70 en 2 de 62 pacientes (4%). El medicamento más utilizado fue metotrexato.</p> <span class="elsevierStyleSectionTitle">Conclusión</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Las lesiones en piel más frecuentes corresponden a morfea lineal seguida de morfea mixta y morfea circunscripta. En la ES los sistemas más frecuentemente afectados son el gastrointestinal, el respiratorio y el vascular (este último relacionado con el fenómeno de Raynaud). Se necesita de un mayor conocimiento de esta enfermedad en medicina de primer contacto para el diagnóstico rápido y tratamiento oportuno. Es importante superar la carencia de recursos en países en desarrollo para estudios auxiliares necesarios, tanto para la clasificación como para el seguimiento.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:12 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." 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| 2023 January | 24 | 20 | 44 |
| 2022 December | 20 | 28 | 48 |
| 2022 November | 23 | 29 | 52 |
| 2022 October | 27 | 27 | 54 |
| 2022 September | 18 | 28 | 46 |
| 2022 August | 25 | 48 | 73 |
| 2022 July | 20 | 45 | 65 |
| 2022 June | 24 | 33 | 57 |
| 2022 May | 28 | 44 | 72 |
| 2022 April | 33 | 53 | 86 |
| 2022 March | 42 | 41 | 83 |
| 2022 February | 33 | 28 | 61 |
| 2022 January | 20 | 37 | 57 |
| 2021 December | 15 | 43 | 58 |
| 2021 November | 30 | 45 | 75 |
| 2021 October | 29 | 62 | 91 |
| 2021 September | 19 | 48 | 67 |
| 2021 August | 35 | 49 | 84 |
| 2021 July | 26 | 32 | 58 |
| 2021 June | 40 | 53 | 93 |
| 2021 May | 46 | 37 | 83 |
| 2021 April | 104 | 77 | 181 |
| 2021 March | 86 | 31 | 117 |
| 2021 February | 29 | 25 | 54 |
| 2021 January | 35 | 14 | 49 |
| 2020 December | 25 | 16 | 41 |
| 2020 November | 29 | 30 | 59 |
| 2020 October | 30 | 19 | 49 |
| 2020 September | 54 | 26 | 80 |
| 2020 August | 42 | 14 | 56 |
| 2020 July | 50 | 14 | 64 |
| 2020 June | 48 | 19 | 67 |
| 2020 May | 37 | 18 | 55 |
| 2020 April | 38 | 19 | 57 |
| 2020 March | 12 | 10 | 22 |
| 2020 February | 1 | 0 | 1 |
| 2019 April | 1 | 0 | 1 |
| 2018 December | 2 | 0 | 2 |
| 2018 May | 3 | 1 | 4 |
| 2018 April | 29 | 10 | 39 |
| 2018 March | 23 | 10 | 33 |
| 2018 February | 14 | 4 | 18 |
| 2018 January | 9 | 5 | 14 |
| 2017 December | 15 | 8 | 23 |
| 2017 November | 11 | 6 | 17 |
| 2017 October | 15 | 5 | 20 |
| 2017 September | 10 | 6 | 16 |
| 2017 August | 10 | 10 | 20 |
| 2017 July | 11 | 9 | 20 |
| 2017 June | 25 | 12 | 37 |
| 2017 May | 31 | 10 | 41 |
| 2017 April | 14 | 19 | 33 |
| 2017 March | 20 | 6 | 26 |
| 2017 February | 8 | 3 | 11 |
| 2017 January | 14 | 7 | 21 |
| 2016 December | 49 | 13 | 62 |
| 2016 November | 42 | 2 | 44 |
| 2016 October | 62 | 9 | 71 |
| 2016 September | 40 | 10 | 50 |
| 2016 August | 32 | 5 | 37 |
| 2016 July | 23 | 6 | 29 |
| 2016 May | 4 | 9 | 13 |
| 2016 March | 6 | 0 | 6 |
| 2016 February | 2 | 0 | 2 |
| 2015 December | 3 | 0 | 3 |
| 2015 October | 1 | 0 | 1 |
| 2015 September | 2 | 0 | 2 |
| 2015 July | 9 | 4 | 13 |
| 2015 June | 20 | 10 | 30 |
| 2015 May | 37 | 9 | 46 |
| 2015 April | 26 | 19 | 45 |
| 2015 March | 19 | 6 | 25 |
| 2015 February | 11 | 9 | 20 |
| 2015 January | 32 | 17 | 49 |
| 2014 December | 24 | 13 | 37 |
| 2014 November | 19 | 18 | 37 |
| 2014 October | 16 | 16 | 32 |
| 2014 September | 17 | 18 | 35 |
| 2014 August | 25 | 13 | 38 |
| 2014 July | 27 | 16 | 43 |
| 2014 June | 37 | 23 | 60 |
| 2014 May | 40 | 18 | 58 |
| 2014 April | 23 | 15 | 38 |
| 2014 March | 43 | 15 | 58 |
| 2014 February | 32 | 17 | 49 |
| 2014 January | 20 | 20 | 40 |
| 2013 December | 31 | 16 | 47 |
| 2013 November | 19 | 16 | 35 |
| 2013 October | 33 | 21 | 54 |
| 2013 September | 31 | 24 | 55 |
| 2013 August | 22 | 14 | 36 |
| 2013 July | 26 | 16 | 42 |
| 2013 June | 20 | 18 | 38 |
| 2013 May | 18 | 20 | 38 |
| 2013 April | 14 | 19 | 33 |
| 2013 March | 11 | 19 | 30 |
| 2013 February | 20 | 12 | 32 |
| 2013 January | 11 | 10 | 21 |
| 2012 December | 12 | 15 | 27 |
| 2012 November | 6 | 9 | 15 |
| 2012 October | 5 | 6 | 11 |
| 2012 September | 8 | 3 | 11 |
| 2012 June | 1 | 0 | 1 |
| 2012 April | 3 | 0 | 3 |
| 2012 March | 2 | 0 | 2 |
| 2012 February | 1 | 0 | 1 |
| 2012 January | 5 | 0 | 5 |
| 2011 December | 4 | 0 | 4 |
| 2011 November | 3 | 0 | 3 |
| 2011 October | 6 | 0 | 6 |
| 2011 September | 1 | 0 | 1 |
| 2011 August | 2 | 0 | 2 |
| 2011 July | 2 | 0 | 2 |
| 2011 June | 3 | 0 | 3 |
| 2011 May | 2 | 0 | 2 |
| 2011 April | 1 | 0 | 1 |
| 2011 March | 6 | 0 | 6 |
| 2011 February | 9 | 0 | 9 |
| 2011 January | 16 | 0 | 16 |
| 2010 December | 6 | 0 | 6 |
| 2010 November | 6 | 0 | 6 |
| 2010 October | 11 | 0 | 11 |
| 2010 September | 8 | 0 | 8 |
| 2010 August | 4 | 0 | 4 |
| 2010 July | 10 | 0 | 10 |
| 2010 June | 3 | 0 | 3 |
| 2010 May | 5 | 0 | 5 |
| 2010 April | 10 | 0 | 10 |
| 2010 March | 15 | 0 | 15 |
| 2010 February | 9 | 0 | 9 |
| 2010 January | 5 | 0 | 5 |
| 2009 December | 18 | 0 | 18 |
| 2009 November | 21 | 0 | 21 |
Show all
