Defective inactivation of leukotriene B4 in patients with Sjögren-Larsson syndrome.
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.
Willemsen
M A
MA
Department of Pediatric Neurology and the Laboratory of Pediatrics and Neurology, University Hospital Nijmegen, The Netherlands.
de Jong
J G
JG
van Domburg
P H
PH
Rotteveel
J J
JJ
Wanders
R J
RJ
Mayatepek
E
E
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*
D007975:Leukotriene B4