Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis
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 fe...
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Veröffentlicht in: | Clinical nephrology 2012-07, Vol.78 (1), p.81-84 |
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creator | CHEN, Meng-Tsai WANG, Shu-Ming LIN, Shih-Yi TING, I-Wen LIU, Jiung-Hsiun KUO, Huey-Liang HUANG, Chiu-Ching |
description | 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. |
doi_str_mv | 10.5414/CN107041 |
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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.</description><identifier>ISSN: 0301-0430</identifier><identifier>DOI: 10.5414/CN107041</identifier><identifier>PMID: 22732342</identifier><identifier>CODEN: CLNHBI</identifier><language>eng</language><publisher>München-Deisenhofen: Dustri</publisher><subject>Administration, Oral ; Biological and medical sciences ; Biopsy ; Cardiology. Vascular system ; Diabetic Nephropathies - complications ; Diabetic Nephropathies - therapy ; Diseases of the pericardium ; Echocardiography ; Erdheim-Chester Disease - complications ; Erdheim-Chester Disease - diagnosis ; Erdheim-Chester Disease - drug therapy ; Fever - etiology ; Heart ; Humans ; Injections, Intravenous ; Male ; Medical sciences ; Methylprednisolone - administration & dosage ; Middle Aged ; Nephrology. Urinary tract diseases ; Pericardial Effusion - diagnosis ; Pericardial Effusion - etiology ; Pericardial Effusion - therapy ; Pericardiocentesis ; Prednisolone - administration & dosage ; Pulse Therapy, Drug ; Renal Dialysis ; Steroids - administration & dosage ; Treatment Outcome ; Whole Body Imaging</subject><ispartof>Clinical nephrology, 2012-07, Vol.78 (1), p.81-84</ispartof><rights>2015 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c313t-cb8f611b3f1b8f5afb1d0e21eba934259b36cd2afeeaa28a49274892aaf94d213</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=26043437$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22732342$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>CHEN, Meng-Tsai</creatorcontrib><creatorcontrib>WANG, Shu-Ming</creatorcontrib><creatorcontrib>LIN, Shih-Yi</creatorcontrib><creatorcontrib>TING, I-Wen</creatorcontrib><creatorcontrib>LIU, Jiung-Hsiun</creatorcontrib><creatorcontrib>KUO, Huey-Liang</creatorcontrib><creatorcontrib>HUANG, Chiu-Ching</creatorcontrib><title>Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis</title><title>Clinical nephrology</title><addtitle>Clin Nephrol</addtitle><description>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.</description><subject>Administration, Oral</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Cardiology. Vascular system</subject><subject>Diabetic Nephropathies - complications</subject><subject>Diabetic Nephropathies - therapy</subject><subject>Diseases of the pericardium</subject><subject>Echocardiography</subject><subject>Erdheim-Chester Disease - complications</subject><subject>Erdheim-Chester Disease - diagnosis</subject><subject>Erdheim-Chester Disease - drug therapy</subject><subject>Fever - etiology</subject><subject>Heart</subject><subject>Humans</subject><subject>Injections, Intravenous</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Methylprednisolone - administration & dosage</subject><subject>Middle Aged</subject><subject>Nephrology. Urinary tract diseases</subject><subject>Pericardial Effusion - diagnosis</subject><subject>Pericardial Effusion - etiology</subject><subject>Pericardial Effusion - therapy</subject><subject>Pericardiocentesis</subject><subject>Prednisolone - administration & dosage</subject><subject>Pulse Therapy, Drug</subject><subject>Renal Dialysis</subject><subject>Steroids - administration & dosage</subject><subject>Treatment Outcome</subject><subject>Whole Body Imaging</subject><issn>0301-0430</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkctOwzAQRb0A0fKQ-ALkDRKbgF9tEnao4iUhYAHraOKMqSGJi6dBQuLjcUVLV7bsc67G14wdS3E-MdJczB6lyIWRO2wstJCZMFqM2D7RuxBKFLrYYyOlcq20UWP284zRW4iNh5ajcwP50HMgDtzGwa5OFxEJ-yUsVzfB8evYzNF32WyOtMTIG08IhNwnj8-xC6usb_LEF8lJ5iWHJH5hbEP4wCYJ8NaHBByyXQct4dF6PWCvN9cvs7vs4en2fnb1kFkt9TKzdeGmUtbaybSbgKtlI1BJrKFMj5iUtZ7aRoFDBFAFmFLlpigVgCtNo6Q-YGd_uYsYPoc0ddV5sti20GMYqJJCKZ2XMi-2qI2BKKKrFtF3EL8TVK36rTb9JvRknTrUHTb_4KbcBJyuASALrYvQW09bbpq-xuhc_wJC4IYj</recordid><startdate>20120701</startdate><enddate>20120701</enddate><creator>CHEN, Meng-Tsai</creator><creator>WANG, Shu-Ming</creator><creator>LIN, Shih-Yi</creator><creator>TING, I-Wen</creator><creator>LIU, Jiung-Hsiun</creator><creator>KUO, Huey-Liang</creator><creator>HUANG, Chiu-Ching</creator><general>Dustri</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20120701</creationdate><title>Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis</title><author>CHEN, Meng-Tsai ; WANG, Shu-Ming ; LIN, Shih-Yi ; TING, I-Wen ; LIU, Jiung-Hsiun ; KUO, Huey-Liang ; HUANG, Chiu-Ching</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c313t-cb8f611b3f1b8f5afb1d0e21eba934259b36cd2afeeaa28a49274892aaf94d213</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Administration, Oral</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>Cardiology. Vascular system</topic><topic>Diabetic Nephropathies - complications</topic><topic>Diabetic Nephropathies - therapy</topic><topic>Diseases of the pericardium</topic><topic>Echocardiography</topic><topic>Erdheim-Chester Disease - complications</topic><topic>Erdheim-Chester Disease - diagnosis</topic><topic>Erdheim-Chester Disease - drug therapy</topic><topic>Fever - etiology</topic><topic>Heart</topic><topic>Humans</topic><topic>Injections, Intravenous</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Methylprednisolone - administration & dosage</topic><topic>Middle Aged</topic><topic>Nephrology. Urinary tract diseases</topic><topic>Pericardial Effusion - diagnosis</topic><topic>Pericardial Effusion - etiology</topic><topic>Pericardial Effusion - therapy</topic><topic>Pericardiocentesis</topic><topic>Prednisolone - administration & dosage</topic><topic>Pulse Therapy, Drug</topic><topic>Renal Dialysis</topic><topic>Steroids - administration & dosage</topic><topic>Treatment Outcome</topic><topic>Whole Body Imaging</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>CHEN, Meng-Tsai</creatorcontrib><creatorcontrib>WANG, Shu-Ming</creatorcontrib><creatorcontrib>LIN, Shih-Yi</creatorcontrib><creatorcontrib>TING, I-Wen</creatorcontrib><creatorcontrib>LIU, Jiung-Hsiun</creatorcontrib><creatorcontrib>KUO, Huey-Liang</creatorcontrib><creatorcontrib>HUANG, Chiu-Ching</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical nephrology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>CHEN, Meng-Tsai</au><au>WANG, Shu-Ming</au><au>LIN, Shih-Yi</au><au>TING, I-Wen</au><au>LIU, Jiung-Hsiun</au><au>KUO, Huey-Liang</au><au>HUANG, Chiu-Ching</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis</atitle><jtitle>Clinical nephrology</jtitle><addtitle>Clin Nephrol</addtitle><date>2012-07-01</date><risdate>2012</risdate><volume>78</volume><issue>1</issue><spage>81</spage><epage>84</epage><pages>81-84</pages><issn>0301-0430</issn><coden>CLNHBI</coden><abstract>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.</abstract><cop>München-Deisenhofen</cop><pub>Dustri</pub><pmid>22732342</pmid><doi>10.5414/CN107041</doi><tpages>4</tpages></addata></record> |
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subjects | Administration, Oral Biological and medical sciences Biopsy Cardiology. Vascular system Diabetic Nephropathies - complications Diabetic Nephropathies - therapy Diseases of the pericardium Echocardiography Erdheim-Chester Disease - complications Erdheim-Chester Disease - diagnosis Erdheim-Chester Disease - drug therapy Fever - etiology Heart Humans Injections, Intravenous Male Medical sciences Methylprednisolone - administration & dosage Middle Aged Nephrology. Urinary tract diseases Pericardial Effusion - diagnosis Pericardial Effusion - etiology Pericardial Effusion - therapy Pericardiocentesis Prednisolone - administration & dosage Pulse Therapy, Drug Renal Dialysis Steroids - administration & dosage Treatment Outcome Whole Body Imaging |
title | Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis |
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