Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease).

Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease).

Bennett MJ; Hosking GP; Gayton R; Thompson G; Galloway JH; Cartwright IJ

American Journal Of Medical Genetics

1988

Humans; Follow-Up Studies; Longitudinal Studies; adolescent; Corn Oil/therapeutic use; Dietary Fats; Fish Oils/therapeutic use; Lipid Metabolism; Lipoproteins/blood; Neuronal Ceroid-Lipofuscinoses/diet therapy/drug therapy/therapy

Five patients with juvenile neuronal ceroid-lipofuscinosis, who were shown to have absent or reduced serum prebetalipoprotein and a deficiency of polyunsaturated fatty acids in erythrocyte membrane lipids, were treated for a period of one year with supplements of fish oil extract (rich in omega-3 fatty acids) and encouraged to increase dietary intake of corn oil lipid (rich in omega-6 fatty acids) in an attempt to promote incorporation of these fatty acids into membranes. After one year there was a significant increase in omega-3 fatty acids (P less than 0.05) and in the total polyunsaturated fatty acids (P less than 0.02) in erythrocyte membranes but no change in the incorporation of omega-6 fatty acids. The patients were assessed clinically, psychometrically and neurophysiologically before and after the supplementation. No significant changes were noted after one year of treatment.

1988

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Journal Article

Backlog