Published in
Oxford University Press, Inflammatory Bowel Diseases, 11(27), p. 1853-1857, 2021
DOI: 10.1093/ibd/izab139
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Mevalonate Kinase Deficiency: A Cause of Severe Very-Early-Onset Inflammatory Bowel Disease
Journal article published in 2021 by
Brigitte Bader-Meunier,
Andreia Luís Martins,
Fabienne Charbit-Henrion,
Ulrich Meinzer,
Alexandre Belot,
Laurence Cuisset,
Albert Faye,
Sophie Georgin-Lavialle,
Pierre Quartier 
,
Vanessa Remy-Piccolo,
Frank Ruemmele,
Florence Uettwiller,
Jérôme Viala,
Nadine Cerf Bensussan,
Dominique Berrebi
and other authors.
,
Vanessa Remy-Piccolo,
Frank Ruemmele,
Florence Uettwiller,
Jérôme Viala,
Nadine Cerf Bensussan,
Dominique Berrebi
and other authors.
This paper is made freely available by the publisher.
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Abstract
Abstract Mevalonate kinase deficiency should be considered in patients with severe very-early-onset inflammatory bowel disease (IBD), especially in patients with a history of recurrent or chronic fever, peritoneal adhesions, and atypical IBD pathology. Anti-interleukin-1 therapy may be efficacious in these patients with monogenic very-early-onset IBD.