Linked Life Data

19755920

Source:http://linkedlifedata.com/resource/pubmed/id/19755920

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
2010-1-6
pubmed:abstractText
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
1536-3678
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
31
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
782-6
pubmed:dateRevised
2011-10-6
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Erdheim-Chester disease in childhood: a challenging diagnosis and treatment.
pubmed:affiliation
Department of Pediatrics, Pediatric Rheumatology, Bicêtre University Hospital, Le Kremlin Bicêtre, France. tu-anh.tran@bct.aphp.fr
pubmed:publicationType
Journal Article, Case Reports