Pediatric autoinflammatory bone disorders-a mini review with special focus on pathogenesis and inborn errors of immunity

Autoinflammatory bone disorders are a group of diseases characterized by sterile osteomyelitis. This includes chronic nonbacterial osteomyelitis and the monogenic forms, Majeed syndrome and deficiency of the interleukin-1 receptor antagonist. These disorders result from innate immune system dysregul...

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Bibliographic Details
Main Authors: Rebecca Hetrick (Author), Melissa Oliver (Author)
Format: Book
Published: Frontiers Media S.A., 2023-06-01T00:00:00Z.
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100 1 0 |a Rebecca Hetrick  |e author 
700 1 0 |a Melissa Oliver  |e author 
245 0 0 |a Pediatric autoinflammatory bone disorders-a mini review with special focus on pathogenesis and inborn errors of immunity 
260 |b Frontiers Media S.A.,   |c 2023-06-01T00:00:00Z. 
500 |a 2296-2360 
500 |a 10.3389/fped.2023.1169659 
520 |a Autoinflammatory bone disorders are a group of diseases characterized by sterile osteomyelitis. This includes chronic nonbacterial osteomyelitis and the monogenic forms, Majeed syndrome and deficiency of the interleukin-1 receptor antagonist. These disorders result from innate immune system dysregulation and cytokine imbalance that triggers inflammasome activation causing downstream osteoclastogenesis and excessive bone remodeling. In this review, we will summarize the immunopathogenesis of pediatric autoinflammatory bone diseases with a special focus on the genetics and inborn errors of immunity, while briefly touching on the clinical manifestations and management of each disease as well as areas for future research. 
546 |a EN 
690 |a autoinflammatory bone disorders 
690 |a chronic nonbacterial osteomyelitis 
690 |a chronic recurrent multifocal osteomyelitis 
690 |a synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome 
690 |a Majeed syndrome 
690 |a deficiency of the interleukin-1 receptor antagonist 
690 |a Pediatrics 
690 |a RJ1-570 
655 7 |a article  |2 local 
786 0 |n Frontiers in Pediatrics, Vol 11 (2023) 
787 0 |n https://www.frontiersin.org/articles/10.3389/fped.2023.1169659/full 
787 0 |n https://doaj.org/toc/2296-2360 
856 4 1 |u https://doaj.org/article/cfa3b7a5138d4dbab13a829e31df4ec8  |z Connect to this object online.