Erdheim-Chester disease in childhood: a challenging diagnosis and treatment. Tran, T. A., Fabre, M., Pariente, D., Craiu, I., Haroche, J., Charlotte, F., Eid, P., Durrbach, A., Taoufik, Y., & Kone-Paut, I. J Pediatr Hematol Oncol, 31(10):782–6, October, 2009. ![Erdheim-Chester disease in childhood: a challenging diagnosis and treatment [link]](https://bibbase.org/img/filetypes/link.svg "link")
Paper abstract bibtex Erdheim-Chester disease is a rare, non-Langerhans systemic histiocytosis characterized by bilateral sclerosis of the metaphyseal regions of the long bones and infiltration in other organs. The histopathologic hallmark is defined by a mononuclear infiltrate of foamy histiocytes and rare pathognomonic Touton giant cells with extensive fibrosis. This condition is exceptional in children. We report here a case of Erdheim-Chester disease in a 10-year-old girl with retroperitoneal infiltration and bone involvement, for whom the diagnosis was only established after a 3-year course with multiple biopsies. It is also the first pediatric case successfully treated with interferon-alpha suggesting that interferon-alpha can be a safe and efficient first-line therapy for this disease in children.
@article{tran_erdheim-chester_2009,
title = {Erdheim-{Chester} disease in childhood: a challenging diagnosis and treatment},
volume = {31},
issn = {1536-3678 (ELECTRONIC) 1077-4114 (LINKING)},
shorttitle = {Erdheim-{Chester} disease in childhood: a challenging diagnosis and treatment},
url = {http://www.ncbi.nlm.nih.gov/pubmed/19755920},
abstract = {Erdheim-Chester disease is a rare, non-Langerhans systemic histiocytosis characterized by bilateral sclerosis of the metaphyseal regions of the long bones and infiltration in other organs. The histopathologic hallmark is defined by a mononuclear infiltrate of foamy histiocytes and rare pathognomonic Touton giant cells with extensive fibrosis. This condition is exceptional in children. We report here a case of Erdheim-Chester disease in a 10-year-old girl with retroperitoneal infiltration and bone involvement, for whom the diagnosis was only established after a 3-year course with multiple biopsies. It is also the first pediatric case successfully treated with interferon-alpha suggesting that interferon-alpha can be a safe and efficient first-line therapy for this disease in children.},
number = {10},
journal = {J Pediatr Hematol Oncol},
author = {Tran, T. A. and Fabre, M. and Pariente, D. and Craiu, I. and Haroche, J. and Charlotte, F. and Eid, P. and Durrbach, A. and Taoufik, Y. and Kone-Paut, I.},
month = oct,
year = {2009},
keywords = {Bone, Cell, Child, Disease/*diagnosis/*drug, Diseases, Erdheim-Chester, Female, Humans, Induction, Interferon-alpha/therapeutic, Movement, Remission, Therapy, use},
pages = {782--6},
}
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