Document Detail


Muckle-Wells syndrome/neonatal-onset multisystem inflammatory disease overlap associated with myelodysplasia and cerebrovascular accident.
MedLine Citation:
PMID:  20456407     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a patient with overlapping clinical features of Muckle-Wells syndrome and neonatal-onset multisystem inflammatory disease with an absence of mutation in exon 3 of the CIAS1 ⁄ PYPAF1 ⁄ NALP3 gene. Myelodysplasia and cerebrovascular accident were additional features in this patient, which to our knowledge have not been previously described in association with these disorders. The urticarial rash, myelodysplasia and raised inflammatory markers responded to treatment with the interleukin-1 receptor antagonist, anakinra.
Authors:
V Madan; H Kingston; L A Jamieson; N Goyal; R Ead
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical and experimental dermatology     Volume:  35     ISSN:  1365-2230     ISO Abbreviation:  Clin. Exp. Dermatol.     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-09-21     Completed Date:  2010-10-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7606847     Medline TA:  Clin Exp Dermatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  752-5     Citation Subset:  IM    
Affiliation:
Dermatology Centre, Salford Royal NHS Foundation Trust, Manchester, UK. vishalmadan@doctors.org.uk
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MeSH Terms
Descriptor/Qualifier:
Adult
Cryopyrin-associated Periodic Syndromes / diagnosis*,  drug therapy
Female
Humans
Interleukin 1 Receptor Antagonist Protein / therapeutic use
Myelodysplastic Syndromes / diagnosis*,  drug therapy
Stroke / diagnosis*,  drug therapy
Chemical
Reg. No./Substance:
0/Interleukin 1 Receptor Antagonist Protein

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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