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Different clinical presentation of the hyperimmunoglobulin D syndrome (HIDS) (four cases from Turkey).
MedLine Citation:
PMID:  22246419     Owner:  NLM     Status:  Publisher    
Hyperimmunoglobulin D syndrome (HIDS) is one of the autoinflammatory syndromes which are characterized by febrile attacks. Duration and frequency of the febrile attacks, as well as typical organ involvements vary greatly. Recently, it is possible to reach more reliable data by the possibilities that are opened up by molecular genetics in order to highlight the aetiopathogenesis of this group of diseases. Typical patients with HIDS have an onset of disease in the first year of life. Here, we report four Turkish HIDS cases; three of whom, the symptoms started at a later age. The diagnoses were made by relevant clinical symptoms along with MVK mutations detected by DNA sequencing method. As summarised in this article, HIDS could be presented with a broad spectrum of symptoms. Although most of the HIDS patients are reported from Europe and especially Dutch ancestry, case reports are presented from all over the world. For this reason, HIDS should be kept in mind for the differential diagnosis of periodic fever syndromes or before accepting an FMF patient as colchichine resistant. We suppose that the phenomenon of "later-onset HIDS" should shed light into unresolved clinical problems of patients with periodic fever. Especially in countries that FMF is more frequent such as Turkey, even though the symptoms start later than classic cases, HIDS should be kept in mind for differential diagnosis of periodic fever syndromes.
Dıdem Arslan Tas; Suzan Dınkcı; Eren Erken
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-1-14
Journal Detail:
Title:  Clinical rheumatology     Volume:  -     ISSN:  1434-9949     ISO Abbreviation:  -     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2012-1-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8211469     Medline TA:  Clin Rheumatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Rheumatology-Immunology Department, Faculty Of Medıcıne, Cukurova Unıversıty, Balcalı Hastanesi, Sarıçam, Adana, Turkey, 01330,
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