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Mevalonate Kinase Deficiency

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Book cover Periodic and Non-Periodic Fevers

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Mevalonate kinase deficiency (MKD) is a rare autosomal recessive autoinflammatory disease caused by mutations in the mevalonate kinase (MVK) gene. It is characterized by recurrent attacks of fever mostly associated with cervical lymphadenopathy, cutaneous, digestive, and musculoskeletal manifestations. Disease activity may decrease with time or remain high. Anti-interleukin-1 agents are often effective in controlling and preventing flares in patients with severe forms of MKD.

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Authors and Affiliations

  1. Department of Paediatric Immunology-Haematology and Rheumatology, Necker University Hospital – AP-HP, Paris, France

    Brigitte Bader-Meunier

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  1. Brigitte Bader-Meunier
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Correspondence to Brigitte Bader-Meunier .

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Editors and Affiliations

  1. Department of Clinical Sciences and Community Health, University of Milano, Milano, Italy

    Rolando Cimaz

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Bader-Meunier, B. (2020). Mevalonate Kinase Deficiency. In: Cimaz, R. (eds) Periodic and Non-Periodic Fevers. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-19055-2_12

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  • DOI: https://doi.org/10.1007/978-3-030-19055-2_12

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-19054-5

  • Online ISBN: 978-3-030-19055-2

  • eBook Packages: MedicineMedicine (R0)

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