By Gustavo C Roman MD (Dr. Roman, Director of the Alzheimer Center of Excellence at the Methodist Neurological Institute in Houston, Texas, has no relevant financial relationships to disclose.) Pedro J Ruiz MD (Dr. Ruiz of the California Pacific Medical Center in San Francisco, California, has no relevant financial relationships to disclose.) Originally released June 28, 2006; last updated June...

Sjogren-Larsson syndrome (SLS) is an inherited disorder associated with impaired fatty alcohol oxidation due to deficient activity of fatty alcohol:NAD+ oxidoreductase (FAO). FAO is a complex enzyme which consists of two separate proteins that sequentially catalyze the oxidation of fatty alcohol to fatty aldehyde and fatty acid. To determine which enzymatic component of FAO was deficient in SLS...

Sjögren-Larsson syndrome is an inherited neurocutaneous disorder characterized by ichthyosis, intellectual disability, and spastic diplegia or tetraplegia. Patients have deficient activity of fatty aldehyde dehydrogenase due to mutations in the ALDH3A2 gene, which results in altered lipid composition of their tissues. In this article, the author discusses new information about the biochemical p...

sjogren syndrome (ss) can occur alone, primary sjogren syndrome, or in association with other rheumatic diseases, secondary sjogren syndrome (sss), such as rheumatoid arthritis (ra). the occurrence of sjogren syndrome with ra makes it course worse and increases high morbidity and mortality of ra. in this exploratory study we aim to determine the prevalence of sss (diagnosed based on the revised...

Sjögren’s syndrome is a slowly progressing autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, mainly the lacrimal and salivary glands, resulting in their impaired secretory function. Systemic involvement and symptoms of cutaneous, respiratory, renal, hepatic, neurologic, and vascular systems often occur.1 Renal involvement is a well recognized extra glandular m...