Article
Increased urinary leukotriene E4 during
febrile attacks in the hyperimmunoglobulinaemia D and
periodic fever syndrome
J Frenkela, M A A P Willemsenc, C M R Weemaesd, L Dorlandb, E Mayatepeke
a Department
of General Pediatrics, Wilhelmina Children's Hospital, KE.04.133.1,
University Medical Center Utrecht, PO Box 85090, 3580AB Utrecht,
Netherlands, b Department of
Metabolic Diseases, Wilhelmina Children's Hospital/University Medical
Center, c Department of Pediatric
Neurology, University Medical Center, St Radboud, Netherlands, d Department
of Pediatrics, University Medical Center, e Division of Metabolic and Endocrine Diseases,
University Children's Hospital, Heidelberg, Germany
Correspondence to: Dr Frenkel j.frenkel{at}wkz.azu.nl
Accepted 26 April 2001
BACKGROUND
The
hyperimmunoglobulinaemia D and periodic fever syndrome is a
hereditary periodic fever, caused by deficiency of the enzyme mevalonate kinase. It is unclear how this defect leads to recurrent fever episodes.
AIMTo assess the involvement of
cysteinyl leukotrienes in the pathogenesis of fever attacks as
reflected by urinary leukotriene E4 (LTE4) excretion.
METHODSUrinary LTE4
was measured in seven patients while febrile and afebrile.
RESULTSLTE4 was raised
during fever in all subjects (46-199 nmol/mol creatinine, mean 92;
normal <40). Urinary LTE4 was normal between attacks, as
well as in normal children with fever as a result of miscellaneous causes.
CONCLUSIONOur results suggest that
cysteinyl leukotrienes play a role in the pathophysiology of this
disorder. As no effective treatment is yet available, leukotriene
receptor antagonists might offer a new therapeutic approach for
patients with the hyperimmunoglobulinaemia D and periodic fever syndrome.
Keywords: cysteinyl leukotrienes; leukotriene E4; mevalonate kinase; periodic fever; immunoglobulin D
© 2001 by Archives of Disease in Childhood
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