Linked Life Data

17618076

Source:http://linkedlifedata.com/resource/pubmed/id/17618076

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
11 Pt 2
pubmed:dateCreated
2007-10-23
pubmed:abstractText
Erdheim-Chester disease is a non-Langerhans cell histiocytosis, classically thought to be rare, but diagnosed more frequently nowadays (250 published cases). Histiocytes of Erdheim-Chester disease are positive for CD68 but not for CD1a, contrary to Langerhans cell histiocytosis. Two signs highly evocative of this diagnosis are nearly constant tracer uptake by the long bones on (99)Tc bone scintigraphy and a "hairy kidney" appearance on abdominal CT scan. A more "elegant" diagnostic method is ultrasound-guided biopsy of the perirenal infiltration. Cardiovascular involvement, which affects the aorta ("coated aorta") as well as all the cardiac layers, leads to one third of the deaths related to this disease. Central nervous system infiltration (especially cerebellar) is severe and difficult to treat. The prognosis is extremely variable and is often worse when there is a cardiovascular and/or central nervous system involvement. The treatment, decided upon on a case-by-case basis at a specialist center, often begins with interferon alpha.
pubmed:language
fre
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Nov
pubmed:issn
0755-4982
pubmed:author
pubmed:issnType
Print
pubmed:volume
36
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1663-8
pubmed:meshHeading
pubmed:year
2007
pubmed:articleTitle
[Erdheim-Chester disease].
pubmed:affiliation
Service de médecine interne, Hôpital Pitié-Salpêtrière, Paris. julien.haroche@psl.aphp.fr
pubmed:publicationType
Journal Article, English Abstract, Review