Increased urinary leukotriene E4 during febrile attacks in the hyperimmunoglobulinaemia D and periodic fever syndrome. Issue 2 (1st August 2001)

• Record Type: Journal Article
• Authors: Frenkel, J; Willemsen, M A A P; Weemaes, C M R; Dorland, L; Mayatepek, E
• Is Part Of: Archives of disease in childhood. Volume 85:Issue 2(2001)
• Page Start: 158
• Page End: 159
• Publication Date: 2001-08-01
• Subjects: cysteinyl leukotrienes -- leukotriene E4 -- mevalonate kinase -- periodic fever -- immunoglobulin D; Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/adc.85.2.158
• Languages: English
• ISSNs: 0003-9888

Abstract

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 E4 (LTE4 ) excretion. METHODS: Urinary LTE4 was measured in seven patients while febrile and afebrile. RESULTS: LTE4 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. 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.