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Increased urinary leukotriene E4 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.

AIM To assess the involvement of cysteinyl leukotrienes in the pathogenesis of fever attacks as reflected by urinary leukotriene E4 (LTE4) excretion.

METHODS Urinary LTE4was 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.

  • cysteinyl leukotrienes
  • leukotriene E4
  • mevalonate kinase
  • periodic fever
  • immunoglobulin D

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