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Erdheim-Chester disease in a child / La enfermedad de Erdheim-Chester en una niña
Wen, C; Liang, Q; Yi, Z; Wan, W.
Afiliação
  • Wen, C; Central South University. Xiangya Second Hospital. Departments of Paediatrics. Changsha City. CN
  • Liang, Q; Central South University. Xiangya Second Hospital. Departments of Paediatrics. Changsha City. CN
  • Yi, Z; Central South University. Xiangya Second Hospital. Departments of Paediatrics. Changsha City. CN
  • Wan, W; Central South University. Xiangya Second Hospital. Departments of Paediatrics. Changsha City. CN
West Indian med. j ; 61(8): 834-837, Nov. 2012. ilus
Artigo em Inglês | LILACS | ID: lil-694353
Biblioteca responsável: BR21.1
ABSTRACT
Erdheim-Chester disease (ECD) is a rare systemic non-Langerhans histiocytosis that affects multiple organ systems. It occurs more often in adults, and paediatric ECD is extremely rare. The diagnosis of ECD can be established based on clinical presentations and imaging but the final diagnosis should be based on biopsy. Treatment of ECD has involved the use of corticosteroids, radiotherapy, chemotherapy, surgery and haematopoietic stem cell transplantation, yet the efficacy of these treatments is difficult to determine. At present, it is thought that the treatment of interferon-α (IFN-α) is safe and effective for ECD. Herein, we report on an 11-year old girl who was admitted to hospital because of systemic bone pain and limping, and the final diagnosis of ECD was based on evidence provided by her clinical presentation, imaging and biopsy of a lesion of the right ilium. The patient was treated with subcutaneous IFN-α at a dosage of 3 Χ 10(6) units three times weekly for 19 months. We thought that the treatment of IFN-α was safe and effective for the girl's clinical manifestations, and IFN-α might be a valuable first-line therapy for paediatric ECD.
RESUMEN
La enfermedad Erdheim-Chester (ECD) es una enfermedad sistémica rara caracterizada por histocitosis de células no Langerhans, que afecta múltiples sistemas orgánicos. Se presenta más a menudo en adultos, siendo su ocurrencia pediátrica sumamente rara. El diagnóstico de ECD puede establecerse a partir de sus manifestaciones clínicas e imagen médica, pero su diagnóstico final debe basarse en la biopsia. El tratamiento de ECD incluye el uso de corticosteroides, radioterapia, quimioterapia, cirugía y trasplante de células madre hematopoyéticas. Sin embargo, es difícil determinar la eficacia de estos tratamientos. En la actualidad, se piensa que el tratamiento con interferon-α (IFN-α), es seguro y eficaz para ECD. Aquí reportamos el caso de una niña de 11 años, que fue ingresada al hospital debido a dolores óseos sistémicos y cojera. El diagnóstico final de ECD se basó en evidencias proporcionadas por sus manifestaciones clínicas, el uso de la imagen médica, y la biopsia de una lesión del ilion derecho.
Assuntos

Texto completo: Disponível Coleções: Bases de dados internacionais Base de dados: LILACS Assunto principal: Interferon-alfa / Doença de Erdheim-Chester / Fatores Imunológicos Limite: Criança / Feminino / Humanos Idioma: Inglês Revista: West Indian med. j Assunto da revista: Medicina Ano de publicação: 2012 Tipo de documento: Artigo País de afiliação: China Instituição/País de afiliação: Central South University/CN
Texto completo: Disponível Coleções: Bases de dados internacionais Base de dados: LILACS Assunto principal: Interferon-alfa / Doença de Erdheim-Chester / Fatores Imunológicos Limite: Criança / Feminino / Humanos Idioma: Inglês Revista: West Indian med. j Assunto da revista: Medicina Ano de publicação: 2012 Tipo de documento: Artigo País de afiliação: China Instituição/País de afiliação: Central South University/CN
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