title: Increased urinary leukotriene E(4) during febrile attacks in the hyperimmunoglobulinaemia D and periodic fever syndrome.
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.
  
  
  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.
authors.affiliation: 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
authors.forename: J
authors.initials: J
authors.lastname: Frenkel
authors.forename: M A
authors.initials: MA
authors.lastname: Willemsen
authors.forename: C M
authors.initials: CM
authors.lastname: Weemaes
authors.forename: L
authors.initials: L
authors.lastname: Dorland
authors.forename: E
authors.initials: E
authors.lastname: Mayatepek
mesh_terms: 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
chemical_list: D007072:Immunoglobulin D; D017999:Leukotriene E4; D008798:Mevalonic Acid