Source:http://linkedlifedata.com/resource/pubmed/id/18460866
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
3
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pubmed:dateCreated |
2009-4-6
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pubmed:abstractText |
Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytosis usually affecting bone, that may progress to multi-organ involvement, with pulmonary involvement as an indicator of poor prognosis. Herein, we present a 48-year-old man with a 2-year history of progressive exertional dyspnoea, dry cough, malaise and exophthalmos. High-resolution computed tomography showed peripheral interstitial thickening with a lymphangitic distribution throughout both lungs, suspected of representing lymphangitic spread of neoplasia. Transbronchial biopsy specimen and bronchoalveolar lavage were non-diagnostic; thus, a surgical lung biopsy was performed which showed features diagnostic of ECD. Subsequent systematic investigations showed widespread bone involvement, cardiac involvement manifested as left heart failure and renal/perirenal disease. Treatment with pulsed corticosteroids and cyclophosphamide elicited neither clinical nor functional response, with death at 6 months. This case highlights the aggressive nature of ECD when there is pulmonary involvement, as well as problems in diagnosis when there is pulmonary presentation and when systemic disease is asymptomatic.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:status |
MEDLINE
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pubmed:issn |
1423-0356
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pubmed:author | |
pubmed:copyrightInfo |
Copyright 2008 S. Karger AG, Basel.
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pubmed:issnType |
Electronic
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pubmed:volume |
77
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
337-40
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pubmed:dateRevised |
2009-11-11
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pubmed:meshHeading | |
pubmed:year |
2009
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pubmed:articleTitle |
Erdheim-Chester disease: pulmonary presentation in a case with advanced systemic involvement.
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pubmed:affiliation |
Departments of Thoracic Medicine and Radiology, Medical School, University of Crete, Heraklion, Greece Department of Pathology, Royal Brompton Hospital, London, UK.
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pubmed:publicationType |
Journal Article,
Case Reports
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