| Melkersson-Rosenthal syndrome and acquired C1 inhibitor deficiency. | |
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MedLine Citation:
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PMID: 18477847 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disease whose full-blown form is characterized by orofacial swelling, facial palsy and lingua plicata. OBJECTIVE: To investigate the complement system as well as its role in patients with MRS. METHODS: Seven patients presenting at this hospital between November 2002 and May 2003 and meeting the diagnostic criteria according to Hornstein were evaluated retrospectively. The investigations included clinical signs, an analysis of the complement system including levels of CH50, C3, C4, C1 inhibitor (INH) functions and C1-INH antigen detection. RESULTS: Two female patients showed isolated low levels of functional C1-INH as determined by duplicate tests. Both patients took estrogen-progestin contraceptives. CONCLUSION: Since deficiency in plasma protease C1-INH is known to lead to recurrent angioedema, we hypothesize that low levels of functional C1-INH may have contributed to the orofacial swelling in the 2 patients. |
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Authors:
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F Masson; S Barete; V Frémeaux-Bacchi; H Szpirglas; S Agbo-Godeau; O Chosidow; J C Piette; C Francès |
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Publication Detail:
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Type: Journal Article Date: 2008-05-10 |
Journal Detail:
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Title: Dermatology (Basel, Switzerland) Volume: 217 ISSN: 1421-9832 ISO Abbreviation: Dermatology (Basel) Publication Date: 2008 |
Date Detail:
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Created Date: 2008-08-13 Completed Date: 2008-11-04 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9203244 Medline TA: Dermatology Country: Switzerland |
Other Details:
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Languages: eng Pagination: 114-20 Citation Subset: IM |
Copyright Information:
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(c) 2008 S. Karger AG, Basel. |
Affiliation:
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Department of Dermatology and Allergy, Tenon Hospital, Paris, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Biological Markers / analysis* Combined Modality Therapy Complement C1 Inactivator Proteins / deficiency*, immunology Complement C3 / analysis, immunology Complement C4 / analysis, immunology Complement Hemolytic Activity Assay Female Humans Male Melkersson-Rosenthal Syndrome / diagnosis*, immunology*, therapy Middle Aged Prognosis Rare Diseases Retrospective Studies Risk Assessment Sampling Studies Sensitivity and Specificity Severity of Illness Index |
| Chemical | |
Reg. No./Substance:
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0/Biological Markers; 0/Complement C1 Inactivator Proteins; 0/Complement C3; 0/Complement C4 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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