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- W4214706886 endingPage "1278" @default.
- W4214706886 startingPage "1278" @default.
- W4214706886 abstract "Behçet's disease (BD) is a polygenic condition with a complex immunopathogenetic background and challenging diagnostic and therapeutic concepts. Advances in genomic medicine have provided intriguing insights into disease pathogenesis over the last decade, especially into monogenic mimics of BD. Although a rare condition, paediatric BD should be considered an important differential diagnosis, especially in cases with similar phenotypes. Emerging reports of monogenic mimics have indicated the importance of genetic testing, particularly for those with early-onset, atypical features and familial aggregation. Treatment options ought to be evaluated in a multidisciplinary setting, given the complexity and diverse organ involvement. Owing to the rarity of the condition, there is a paucity of paediatric trials; thus, international collaboration is warranted to provide consensus recommendations for the management of children and young people. Herein, we summarise the current knowledge of the clinical presentation, immunopathogenetic associations and disease mechanisms in patients with paediatric BD and BD-related phenotypes, with particular emphasis on recently identified monogenic mimics." @default.
- W4214706886 created "2022-03-02" @default.
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- W4214706886 creator A5059983393 @default.
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- W4214706886 creator A5079042387 @default.
- W4214706886 date "2022-02-26" @default.
- W4214706886 modified "2023-10-14" @default.
- W4214706886 title "Paediatric Behçet’s Disease: A Comprehensive Review with an Emphasis on Monogenic Mimics" @default.
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