J. Frenkel et al., Increased urinary leukotriene E-4 during febrile attacks in the hyperimmunoglobulinaemia D and periodic fever syndrome, ARCH DIS CH, 85(2), 2001, pp. 158-159
Background-The hyperimmunoglobulinaemia D and periodic fever syndrome is a
hereditary periodic fever, caused by deficiency of the enzyme mevalonate ki
nase. It is unclear how this defect leads to recurrent fever episodes.
Aim-To assess the involvement of cysteinyl leukotrienes in the pathogenesis
of fever attacks as reflected by urinary leukotriene E-4 (LTE4) excretion.
Methods-Urinary LTE4 was measured in seven patients while febrile and afebr
ile.
Results-LTE4 was raised during fever in all subjects (46-199 nmol/mol creat
mine, mean 92; normal <40). Urinary LTE4 was normal between attacks, as wel
l as in normal children with fever as a result of miscellaneous causes.
Conclusion-Our results suggest that cysteinyl leukotrienes play a role in t
he pathophysiology of this disorder. As no effective treatment is yet avail
able, leukotriene receptor antagonists might offer a new therapeutic approa
ch for patients with the hyperimmunoglobulinaemia D and periodic fever synd
rome.