| Persistent systemic inflammation and atypical enterocolitis in patients with NEMO syndrome. | |
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MedLine Citation:
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PMID: 19375390 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The NEMO syndrome is a primary immunodeficiency with immune and non-immune manifestations. The immune deficiency is heterogeneous showing defects in humoral, innate, and cell-mediated immunity. While the clinical aspects of the immunodeficiency are increasingly well understood, little is known about autoimmune manifestations in NEMO patients. We therefore sought to examine serologic markers of systemic inflammation and intestinal pathology in a kindred of patients with the NEMO syndrome. We observed persistent elevation of erythrocyte sedimentation rates in five patients, and two were symptomatic, with a chronic but atypical enterocolitis. Though pathologic lesions in these two patients were consistent with acute inflammation, sustained clinical improvement was only achieved with systemic and/or topical glucocorticoid therapy. Our data suggest that some patients with the NEMO syndrome exhibit persistent elevation of inflammatory markers similar to systemic autoimmune diseases and may subsequently develop an atypical enterocolitis. |
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Authors:
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Laurence E Cheng; Bittoo Kanwar; Haig Tcheurekdjian; James P Grenert; Mica Muskat; Melvin B Heyman; Joseph M McCune; Diane W Wara |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2009-04-16 |
Journal Detail:
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Title: Clinical immunology (Orlando, Fla.) Volume: 132 ISSN: 1521-7035 ISO Abbreviation: Clin. Immunol. Publication Date: 2009 Jul |
Date Detail:
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Created Date: 2009-06-08 Completed Date: 2009-07-08 Revised Date: 2011-06-14 |
Medline Journal Info:
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Nlm Unique ID: 100883537 Medline TA: Clin Immunol Country: United States |
Other Details:
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Languages: eng Pagination: 124-31 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, University of California, San Francisco School of Medicine, San Francisco, CA 94143, USA. ChengL@peds.ucsf.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Blood Sedimentation Child Child, Preschool Colonoscopy Enterocolitis / blood, etiology*, pathology Female Humans Immunologic Deficiency Syndromes / blood, complications*, pathology Infant Inflammation / blood, etiology*, pathology Male Pedigree |
| Grant Support | |
ID/Acronym/Agency:
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DP1 OD000329-06/OD/NIH HHS; K24 DK060617/DK/NIDDK NIH HHS; OD00329/OD/NIH HHS; T32 DK007762/DK/NIDDK NIH HHS; T32 HD044331/HD/NICHD NIH HHS; UL1 RR024131/RR/NCRR NIH HHS; UL1 RR024131-03S3/RR/NCRR NIH HHS |
| Comments/Corrections | |
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