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Mutations in the gene encoding mevalonate kinase cause hyper-IgD and periodic fever syndrome.
Hereditary Periodic Fever Syndromes: Childhood and Adolescent Hematology 2005; 45: 74-81.
Fever, relapsing fever, 5-day fever, periodic fever and 3-day fever were queried using 780.
Locus heterogeneity may be interpreted as non-MEFV genes responsible for periodic fevers other than FMF, e.
Jasmin, six, of Billingham, suffers from pulmonary hypertension, Ryan, six, of Hartlepool, suffers periodic fever syndrome, and Mohin, ten, of Acklam, has undergone 35 operations in his short life.
These cases are currently being investigated for other types of periodic fever by direct DNA sequencing of the MVK (HIDS) and TNFRSFIA (TRAPS) genes.
The hyperimmunoglobulinemia D and periodic fever syndrome is proposed to be caused by a defect in the activity of mevalonate kinase enzyme which is involved in cholesterol and non-sterol isoprenoid biosynthesis.

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