title: Unsuccessful dietary treatment of Sjögren-Larsson syndrome.
abstract: We treated five children with Sjögren-Larsson syndrome. The patients, 5 months to 8 years of age, were given a low fat diet supplemented with medium-chain fatty acids. Plasma octadecanol levels remained unchanged, and skin lesions and neurologic symptoms did not abate. Two patients also failed to respond to dietary supplementation with polyunsaturated fatty acids. We conclude that dietary therapy is usually unsuccessful in patients with Sjögren-Larsson syndrome even when started in early infancy.
authors.affiliation: Department of Pediatrics, University Hospital Leiden, The Netherlands.
authors.forename: P D
authors.initials: PD
authors.lastname: Maaswinkel-Mooij
authors.forename: O F
authors.initials: OF
authors.lastname: Brouwer
authors.forename: W B
authors.initials: WB
authors.lastname: Rizzo
mesh_terms: D002675:Child, Preschool; D004041:Dietary Fats / Q000008:administration & dosage; D005228:Fatty Acids, Essential / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008041:Linoleic Acids; D008297:Male; D031665:Oenothera biennis; D010938:Plant Oils; D016111:Sjogren-Larsson Syndrome / Q000178:diet therapy*; D017211:Treatment Failure; D017965:gamma-Linolenic Acid
chemical_list: D004041:Dietary Fats; D005228:Fatty Acids, Essential; D008041:Linoleic Acids; D010938:Plant Oils; C028498:evening primrose oil; D017965:gamma-Linolenic Acid