{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,12,8]],"date-time":"2024-12-08T21:40:09Z","timestamp":1733694009051,"version":"3.30.1"},"reference-count":15,"publisher":"BMJ","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arch Dis Child"],"published-print":{"date-parts":[[2001,8,1]]},"abstract":"<jats:sec><jats:title>BACKGROUND<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>AIM<\/jats:title><jats:p>To assess the involvement of cysteinyl leukotrienes in the pathogenesis of fever attacks as reflected by urinary leukotriene E<jats:sub>4<\/jats:sub>(LTE<jats:sub>4<\/jats:sub>) excretion.<\/jats:p><\/jats:sec><jats:sec sec-type=\"methods\"><jats:title>METHODS<\/jats:title><jats:p>Urinary LTE<jats:sub>4<\/jats:sub>was measured in seven patients while febrile and afebrile.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>LTE<jats:sub>4<\/jats:sub>was raised during fever in all subjects (46\u2013199 nmol\/mol creatinine, mean 92; normal &lt;40). Urinary LTE<jats:sub>4<\/jats:sub>was normal between attacks, as well as in normal children with fever as a result of miscellaneous causes.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSION<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.1136\/adc.85.2.158","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T10:32:32Z","timestamp":1027765952000},"page":"158-159","source":"Crossref","is-referenced-by-count":9,"title":["Increased urinary leukotriene E<sub>4<\/sub>during febrile attacks in the hyperimmunoglobulinaemia D and periodic fever syndrome"],"prefix":"10.1136","volume":"85","author":[{"given":"J","family":"Frenkel","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M A A P","family":"Willemsen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C M R","family":"Weemaes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L","family":"Dorland","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E","family":"Mayatepek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2001,8,1]]},"reference":[{"key":"2021071919352061000_85.2.158.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(84)92505-4"},{"key":"2021071919352061000_85.2.158.2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199405000-00002"},{"key":"2021071919352061000_85.2.158.3","doi-asserted-by":"crossref","first-page":"3586","DOI":"10.1182\/blood.V85.12.3586.bloodjournal85123586","article-title":"Cytokine activation during attacks of the hyperimmunoglobulinemia D and periodic fever 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