Defective inactivation of leukotriene B4 in patients with Sjögren-Larsson syndrome

M A Willemsen; J G de Jong; P H van Domburg; J J Rotteveel; R J Wanders; E Mayatepek

doi:10.1016/s0022-3476(00)70113-2

Defective inactivation of leukotriene B4 in patients with Sjögren-Larsson syndrome

J Pediatr. 2000 Feb;136(2):258-60. doi: 10.1016/s0022-3476(00)70113-2.

Authors

M A Willemsen¹, J G de Jong, P H van Domburg, J J Rotteveel, R J Wanders, E Mayatepek

Affiliation

¹ Department of Pediatric Neurology and the Laboratory of Pediatrics and Neurology, University Hospital Nijmegen, The Netherlands.

PMID: 10657837
DOI: 10.1016/s0022-3476(00)70113-2

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.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Case-Control Studies
Child
Female
Humans
Leukotriene B4 / metabolism
Leukotriene B4 / urine*
Male
Sjogren-Larsson Syndrome / diagnosis
Sjogren-Larsson Syndrome / immunology
Sjogren-Larsson Syndrome / urine*

Substances

Leukotriene B4