Defective inactivation of leukotriene B-4 in patients with Sjogren-Larssonsyndrome

Authors
Willemsen, MAAP de Jong, JGN van Domburg, PHMF Rotteveel, JJ Wanders, RJA Mayatepek, E
Citation
Maap. Willemsen et al., Defective inactivation of leukotriene B-4 in patients with Sjogren-Larssonsyndrome, J PEDIAT, 136(2), 2000, pp. 258-260
Citations number
13
Categorie Soggetti
Pediatrics,"Medical Research General Topics
Journal title
JOURNAL OF PEDIATRICS
ISSN journal
00223476 → ACNP
Volume
136
Issue
2
Year of publication
2000
Pages
258 - 260
Database
ISI
SICI code
0022-3476(200002)136:2<258:DIOLBI>2.0.ZU;2-8
Abstract
In 6 patients with Sjogren-Larsson syndrome (SL), the urinary excretion of leukotriene B-4 (LTB4) and omega-hydroxy-LTB4 was found to be highly elevat ed, whereas omega-carboxy-LTB4 was absent. This abnormal pattern of urinary excretion of LTB4 and its metabolites appears to be unique to patients wit h SLS and offers a new approach to the diagnosis of this disorder. Moreover , defective inactivation of LTB4 might be of pathophysiologic significance in the disease.