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

Willemsen, M A; de Jong, J G; van Domburg, P H; Rotteveel, J J; Wanders, R J; Mayatepek, E · J Pediatr · 2000

case_series · Level IV

Where this comes from

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.

Medical subject headings