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Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis.
MedLine Citation:
PMID:  22732342     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis whose hallmark is tissue infiltration by CD68-positive, CD1a-negative and usually S-100 protein-positive foamy non-Langerhans histiocytes and mononuclear cells. Here, we report a hemodialysis (HD) patient who presented with fever and pericardial effusion. We performed pericardiocentesis with pericardial biopsy and the histological findings indicated ECD. We administered intravenous methylprednisolone pulse therapy (250 mg/d) followed by oral prednisolone (50 mg/d). The patient's fever gradually subsided and there was no recurrence of pericardial effusion. This is the first report of an HD patient with ECD. We suggest that ECD be considered in the differential diagnosis of new HD patients who present with pericardial effusion, especially when this did not improve following increased dose of HD.
Authors:
Meng-Tsai Chen; Shu-Ming Wang; Shih-Yi Lin; I-Wen Ting; Jiung-Hsiun Liu; Huey-Liang Kuo; Chiu-Ching Huang
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical nephrology     Volume:  78     ISSN:  0301-0430     ISO Abbreviation:  Clin. Nephrol.     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-06-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0364441     Medline TA:  Clin Nephrol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  81-4     Citation Subset:  IM    
Affiliation:
Division of Nephrology and Kidney Institute, Department of Internal Medicine, and China Medical University, Taichung, Taiwan.
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