title: Early dietary treatments with Lorenzo's oil and docosahexaenoic acid for neurological development in a case with Zellweger syndrome.
abstract: We treated a girl with Zellweger syndrome using a special infant formula supplemented with middle chain triglyceride (MCT) milk, docosahexaenoic acid (DHA), Lorenzo's oil, and Lunaria oil, which is rich in nervonic acid (C24:1). We examined the fatty acid contents of the plasma and red blood cell (RBC) membrane. Neurological development was evaluated using Denver developmental screening test and auditory brainstem response (ABR). Her delayed neurological development, liver dysfunction, and cholestasis were all improved 2 weeks after starting the dietary treatment. DHA level in RBC membranes was increased and very long chain fatty acid (VLCFA,C26:0) levels were decreased. Our findings suggest that the dietary treatment with combination of MCT milk, DHA, Lorenzo's oil, and Lunaria oil in the patients with Zellweger syndrome bring some benefits for neurological development.
authors.affiliation: Department of Pediatrics, Juntendo University School of Medicine, 2-1-1 Hongo, 113-8421 Tokyo, Japan. kyoko-nishi@mse.biglobe.ne.jp
authors.forename: Kyoko
authors.initials: K
authors.lastname: Tanaka
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Shimizu
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Ohtsuka
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Yamashiro
authors.forename: Kyoichi
authors.initials: K
authors.lastname: Oshida
mesh_terms: D002657:Child Development / Q000187:drug effects* / Q000502:physiology; D004281:Docosahexaenoic Acids / Q000627:therapeutic use*; D004338:Drug Combinations; D004359:Drug Therapy, Combination; D004883:Erucic Acids / Q000627:therapeutic use*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005227:Fatty Acids / Q000097:blood; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D016896:Treatment Outcome; D014280:Triglycerides / Q000627:therapeutic use; D014304:Triolein / Q000627:therapeutic use*; D015211:Zellweger Syndrome / Q000178:diet therapy* / Q000503:physiopathology*
chemical_list: D004338:Drug Combinations; D004883:Erucic Acids; D005227:Fatty Acids; C079420:Lorenzo's oil; D014280:Triglycerides; D014304:Triolein; D004281:Docosahexaenoic Acids