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        "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&#44; classified as localized and systemic &#40;participation of one or more internal organs&#41;&#46; 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&#46;</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 M&#233;xico Federico G&#243;mez&#44; between January 2000 and December 2007&#46;</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Sixty-two patients were included in the group&#46; All of them completed the classification criteria for juvenile sclerodema&#44; both systemic and localized&#46; The mean age at diagnosis was 7&#46;8 &#40;1&#8211;14&#41; years&#46; The mean time from disease onset to diagnosis&#44; based on clinical manifestations&#44; was 23 months&#46; The lesions found were&#58; linear scleroderma &#40;42&#37;&#41;&#44; mixed morphea &#40;22&#37;&#41;&#44; circumscribed morphea &#40;19&#37;&#41;&#44; generalized morphea &#40;13&#37;&#41;&#44; and panclerotic morphea &#40;4&#37;&#41;&#46; Involvement associated with systemic scleroderma was gastrointestinal 100&#37; &#40;18 patients&#41;&#44; pulmonary 100&#37; &#40;18&#47;18&#41;&#44; Raynaud&#39;s phenomenon 89&#37; &#40;16&#47;18&#41;&#44; proximal sclerosis 89&#37; &#40;16&#47;18&#41;&#44; sclerodactilia 67&#37; &#40;12&#47;18&#41;&#44; joint pain 28&#37; &#40;5&#47;18&#41;&#44; calcinosis 56&#37; &#40;10&#47;18&#41;&#46; Positive antinuclear antibodies &#40;ANA&#41; were present in 14&#47;62 &#40;23&#37;&#41; patients &#40;10 with systemic range and 4 localized&#41;&#44; antiSCL 70 in 2&#47;62 &#40;4&#37;&#41; cases&#46; The most common drug used was methotrexate&#46;</p> <span class="elsevierStyleSectionTitle">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">The most common skin lesions found were linear morphea&#44; followed by the mixed and circumscribed types&#46; In systemic scleroderma the most involved systems are the gastrointestinal&#44; respiratory&#44; and vascular &#40;associated with Raynaud&#39;s phenomenon&#41;&#46; There is a special need for knowledge of this disease in first contact physicians for a faster and better diagnosis and treatment&#44; in order to avoid complications&#46; It is also necessary to improve resources in developing countries for complimentary studies&#44; classification&#44; treatment&#44; and follow-up&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Introducci&#243;n</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">La esclerodermia es una enfermedad del tejido conectivo&#44; autoinmunitaria y caracterizada por fibrosis de la piel&#59; adem&#225;s&#44; puede ser localizada o sist&#233;mica &#40;participaci&#243;n de uno o de m&#225;s &#243;rganos internos&#41;&#46; El objetivo del presente estudio es describir y analizar las caracter&#237;sticas cl&#237;nicas y de laboratorio observadas en un grupo de ni&#241;os con esclerodermia en un hospital de referencia&#46;</p> <span class="elsevierStyleSectionTitle">Material y m&#233;todos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Recopilaci&#243;n de datos de los expedientes cl&#237;nicos de ni&#241;os con diagn&#243;stico de esclerodermia del Departamento de Reumatolog&#237;a del Hospital Infantil de M&#233;xico Federico G&#243;mez durante el per&#237;odo comprendido entre los a&#241;os 2000 y 2007&#46;</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Se incluy&#243; a 62 pacientes que cumplieron con los criterios de clasificaci&#243;n preliminar para esclerodermia juvenil&#44; localizada y sist&#233;mica&#46; La edad media al diagn&#243;stico fue de 7&#44;8 a&#241;os &#40;1&#8211;14&#41;&#46; La duraci&#243;n media de la enfermedad al diagn&#243;stico fue de 23 meses&#46; Las lesiones encontradas fueron esclerodermia lineal &#40;42&#37;&#41;&#44; morfea mixta &#40;22&#37;&#41;&#44; morfea circunscripta &#40;19&#37;&#41;&#44; morfea generalizada &#40;13&#37;&#41; y morfea panescler&#243;tica &#40;4&#37;&#41;&#46; Los hallazgos asociados a esclerodermia sist&#233;mica &#40;ES&#41; fueron afecci&#243;n gastrointestinal en 18 de 18 pacientes &#40;100&#37;&#41;&#44; afecci&#243;n pulmonar en 18 de 18 pacientes &#40;100&#37;&#41;&#44; fen&#243;meno de Raynaud en 16 de 18 pacientes &#40;89&#37;&#41;&#44; esclerosis proximal en 16 de 18 pacientes &#40;89&#37;&#41;&#44; esclerodactilia en 12 de 18 pacientes &#40;67&#37;&#41;&#44; calcinosis en 10 de 18 pacientes &#40;56&#37;&#41; y afecci&#243;n articular en 5 de 18 pacientes &#40;28&#37;&#41;&#46; Se hallaron anticuerpos antinucleares positivos en 14 de 62 pacientes &#40;23&#37;&#41; &#40;10 con ES y 4 con esclerodermia localizada&#41; y anticuerpos anti-Scl70 en 2 de 62 pacientes &#40;4&#37;&#41;&#46; El medicamento m&#225;s utilizado fue metotrexato&#46;</p> <span class="elsevierStyleSectionTitle">Conclusi&#243;n</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Las lesiones en piel m&#225;s frecuentes corresponden a morfea lineal seguida de morfea mixta y morfea circunscripta&#46; En la ES los sistemas m&#225;s frecuentemente afectados son el gastrointestinal&#44; el respiratorio y el vascular &#40;este &#250;ltimo relacionado con el fen&#243;meno de Raynaud&#41;&#46; Se necesita de un mayor conocimiento de esta enfermedad en medicina de primer contacto para el diagn&#243;stico r&#225;pido y tratamiento oportuno&#46; Es importante superar la carencia de recursos en pa&#237;ses en desarrollo para estudios auxiliares necesarios&#44; tanto para la clasificaci&#243;n como para el seguimiento&#46;</p>"
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Vol. 5. Issue 4.
Pages 158-162 (July - August 2009)
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Vol. 5. Issue 4.
Pages 158-162 (July - August 2009)
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Clinical characteristics of children with scleroderma in a referral hospital
Características clínicas de niños con esclerodesmia en un hospital de referencia
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Zoilo Morel
Corresponding author
zoiloma@hotmail.com

Corresponding author.
, Eduardo Benadón, Enrique Faugier, María del Rocío Maldonado
Departamento de Reumatología Pediátrica, Hospital Infantil de México Federico Gómez, México DF, Mexico
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Article information
Abstract
Introduction

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.

Material and methods

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.

Results

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.

Conclusion

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.

Keywords:
Systemic scleroderma
Localized scleroderma
Morphea
Raynaud's phenomenon
Methotrexate
Resumen
Introducción

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.

Material y métodos

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.

Resultados

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.

Conclusión

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.

Palabras clave:
Esclerodermia sistémica
Esclerodermia localizada
Morfea
Fenómeno de Raynaud
Metotrexato
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En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients.
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Copyright © 2009. Sociedad Española de Reumatología and Colegio Mexicano de Reumatología
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