1. Increased urinary leukotriene E(4) during febrile attacks in the hyperimmunoglobulinaemia D and periodic fever syndrome.
- Author
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Frenkel J, Willemsen MA, Weemaes CM, Dorland L, and Mayatepek E
- Subjects
- Adolescent, Child, Chromatography, Gas, Chromatography, High Pressure Liquid, Humans, Mass Spectrometry, Mevalonic Acid urine, Radioisotope Dilution Technique, Familial Mediterranean Fever urine, Hypergammaglobulinemia urine, Immunoglobulin D, Leukotriene E4 urine
- Abstract
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., Aim: 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., Conclusion: 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.
- Published
- 2001
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