Increased urinary leukotriene E(4) during febrile attacks in the hyperimmunoglobulinaemia D and periodic fever syndrome.
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.


OBJECTIVE
To assess the involvement of cysteinyl leukotrienes in the pathogenesis of fever attacks as reflected by urinary leukotriene E(4) (LTE(4)) excretion.


METHODS
Urinary LTE(4) was measured in seven patients while febrile and afebrile.


RESULTS
LTE(4) was raised during fever in all subjects (46-199 nmol/mol creatinine, mean 92; normal <40). Urinary LTE(4) was normal between attacks, as well as in normal children with fever as a result of miscellaneous causes.


CONCLUSIONS
Our 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.
Frenkel
J
J
Department of General Pediatrics, Wilhelmina Children's Hospital, KE.04.133.1, University Medical Center Utrecht, PO Box 85090, 3580AB Utrecht, Netherlands. j.frenkel@wkz.azu.nl
Willemsen
M A
MA
Weemaes
C M
CM
Dorland
L
L
Mayatepek
E
E
D000293:Adolescent; D002648:Child; D002849:Chromatography, Gas; D002851:Chromatography, High Pressure Liquid; D010505:Familial Mediterranean Fever / Q000652:urine*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000652:urine*; D007072:Immunoglobulin D; D017999:Leukotriene E4 / Q000652:urine*; D013058:Mass Spectrometry; D008798:Mevalonic Acid / Q000652:urine; D011865:Radioisotope Dilution Technique
D007072:Immunoglobulin D; D017999:Leukotriene E4; D008798:Mevalonic Acid