Willemsen M A, Rotteveel J J, Steijlen P M, Heerschap A, Mayatepek E
Department of Pediatric Neurology, University Hospital Nijmegen, The Netherlands.
Neuropediatrics. 2000 Feb;31(1):1-3. doi: 10.1055/s-2000-15288.
The Sjögren-Larsson syndrome (SLS) is a severe neurocutaneous disorder due to fatty aldehyde dehydrogenase (FALDH) deficiency. The recent discovery of the role of FALDH in the degradation of leukotriene B4 (LTB4) opened the way to the development of a new therapeutic strategy for SLS, i.e. 5-lipoxygenase inhibition. We treated one SLS patient with zileuton during five weeks. During the treatment period we found decreased values of LTB4 and omega-OH-LTB4. The severity of the pruritus diminished, and favorable changes in the child's behavior were observed. The height of the prominent "lipid peak" of cerebral white matter (that is characteristically found on proton magnetic resonance spectroscopy in SLS patients) decreased during treatment, and increased again when treatment was stopped. In conclusion, the beneficial effects of 5-lipoxygenase inhibition in SLS are very promising and encourage further research.
舍格伦-拉松综合征（SLS）是一种由于脂肪醛脱氢酶（FALDH）缺乏引起的严重神经皮肤疾病。最近发现FALDH在白三烯B4（LTB4）降解中的作用，为开发一种针对SLS的新治疗策略开辟了道路，即5-脂氧合酶抑制。我们用齐留通治疗了一名SLS患者五周。在治疗期间，我们发现LTB4和ω-OH-LTB4的值降低。瘙痒的严重程度减轻，并且观察到患儿行为有良好变化。治疗期间，大脑白质突出的“脂质峰”（这是SLS患者质子磁共振波谱的特征性表现）高度降低，停药后又再次升高。总之，5-脂氧合酶抑制对SLS的有益作用非常有前景，并鼓励进一步研究。
