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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Sjogren-Larsson syndrome


Other Names for this Disease

  • FADH deficiency
  • FALDH deficiency
  • FAO deficiency
  • Fatty aldehyde dehydrogenase deficiency
  • Ichthyosis, spastic neurologic disorder, and oligophrenia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment

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How might Sjogren-Larsson syndrome (SLS) be treated?

Topical application of various agents have been used to treat the ichthyosis.[1] Some clinical studies have found that a drug called zileuton can be beneficial in the treatment of SLS. In these studies, the drug successfully reduced the severity of the pruritis (itching), and improved the behavior of the treated child. While this drug does not cure SLS, it has the potential to greatly improve the quality of life of children with SLS.[2] Seizures usually respond well to anti-convulsant medications and spasticity is improved with surgery. Diets supplemented with medium-chain fatty acids have been reported to improve the skin, but the results are inconsistent.[1]

More detailed information about treatment options for SLS can be accessed through the Treatment and Medication sections of Medscape Reference.
Last updated: 9/21/2012

References
  1. Rizzo WB. Sjogren-Larsson Syndrome: Molecular Genetics and Biochemical Pathogenesis of Fatty Aldehyde Dehydrogenase Deficiency. Mol Genet Metab. September 22, 2006; http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1933507/?tool=pubmed. Accessed 9/21/2012.
  2. Sjogren-Larsson Syndrome. United Leukodystrophy Foundation. http://ulf.org/sjogren-larsson-syndrome. Accessed 9/21/2012.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Sjogren-Larsson syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Other Names for this Disease
  • FADH deficiency
  • FALDH deficiency
  • FAO deficiency
  • Fatty aldehyde dehydrogenase deficiency
  • Ichthyosis, spastic neurologic disorder, and oligophrenia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.