Abstract | UNLABELLED: The Sjögren-Larsson syndrome (SLS) is an inborn error of lipid metabolism, characterised clinically by congenital ichthyosis, mental retardation and spasticity. Patients also suffer from severe pruritus. The degradation of leukotriene (LT) B4 is one of the defective metabolic routes in SLS. Zileuton inhibits the synthesis of LTB4 and the cysteinyl leukotrienes. Five SLS patients were treated with zileuton for 3 months. Favourable effects were found on pruritus score (P = 0.006), general well-being, and background activity of electroencephalographic studies. Neuropsychological test results did not change significantly. There was, however, a clinically important trend towards improvement in the speed of information processing. Results of cerebral MRI and proton magnetic resonance spectroscopy did not change during therapy. Urinary concentrations of LTB4 and omega-OH-LTB4 decreased significantly (P=0.02 and P=0.003 respectively), while their concentrations in CSF were normal at baseline and remained so after therapy. CONCLUSION:
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Authors | M A Willemsen, M A Lutt, P M Steijlen, J R Cruysberg, M van der Graaf, M W Nijhuis-van der Sanden, J W Pasman, E Mayatepek, J J Rotteveel |
Journal | European journal of pediatrics
(Eur J Pediatr)
Vol. 160
Issue 12
Pg. 711-7
(Dec 2001)
ISSN: 0340-6199 [Print] Germany |
PMID | 11795678
(Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Lipoxygenase Inhibitors
- zileuton
- Hydroxyurea
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Topics |
- Adolescent
- Adult
- Brain
(pathology)
- Electroencephalography
- Female
- Humans
- Hydroxyurea
(analogs & derivatives, metabolism, therapeutic use)
- Lipoxygenase Inhibitors
(metabolism, therapeutic use)
- Magnetic Resonance Imaging
- Magnetic Resonance Spectroscopy
- Male
- Neuropsychological Tests
- Sjogren-Larsson Syndrome
(diagnosis, drug therapy, metabolism)
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