title: Defective inactivation of leukotriene B4 in patients with Sjögren-Larsson syndrome.
abstract: In 6 patients with Sjögren-Larsson syndrome (SLS), the urinary excretion of leukotriene B(4) (LTB(4)) and omega-hydroxy-LTB(4) was found to be highly elevated, whereas omega-carboxy-LTB(4) was absent. This abnormal pattern of urinary excretion of LTB(4) and its metabolites appears to be unique to patients with SLS and offers a new approach to the diagnosis of this disorder. Moreover, defective inactivation of LTB(4) might be of pathophysiologic significance in the disease.
authors.affiliation: Department of Pediatric Neurology and the Laboratory of Pediatrics and Neurology, University Hospital Nijmegen, The Netherlands.
authors.forename: M A
authors.initials: MA
authors.lastname: Willemsen
authors.forename: J G
authors.initials: JG
authors.lastname: de Jong
authors.forename: P H
authors.initials: PH
authors.lastname: van Domburg
authors.forename: J J
authors.initials: JJ
authors.lastname: Rotteveel
authors.forename: R J
authors.initials: RJ
authors.lastname: Wanders
authors.forename: E
authors.initials: E
authors.lastname: Mayatepek
mesh_terms: D016022:Case-Control Studies; D002648:Child; D005260:Female; D006801:Humans; D007975:Leukotriene B4 / Q000378:metabolism / Q000652:urine*; D008297:Male; D016111:Sjogren-Larsson Syndrome / Q000175:diagnosis / Q000276:immunology / Q000652:urine*
chemical_list: D007975:Leukotriene B4