Yung-Sheng Huang, Andrew Sinclair's Lipids in infant nutrition PDF

By Yung-Sheng Huang, Andrew Sinclair

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2. DHA concentration in the developing human retina (nmol/g of wet tissue) against the logarithm of age. One of the two malnourished children, who received a milk formula with a very low n-3/n-6 ratio, had significantly decreased levels of retinal DHA. In comparison, however, the patient with Zellweger’s syndrome, had practically no DHA in his retina. ing peroxisomal patients with a high purity DHA derivative (DHA ethyl ester, 90–95% pure), in an attempt to alleviate the brain DHA deficiency of these patients.

One patient had classical Zellweger’s syndrome, and died a few weeks after starting the treatment from a pulmonary disease. The other 11 patients were children with either neonatal adrenoleukodystrophy (NALD) or infantile Refsum’s disease (IRD). In one of those having a severe form of NALD, treatment was initiated when the child was in a vegetative state, complicated with frequent episodes of bronchopneumonia, and could only be continued for 6 weeks (18). The other 10 patients have been treated for periods varying from 19 months to 6 years.

Inherit. Metab. Dis. 9, 169–174. 6. , and Borst, P. Severe Plasmalogen Deficiency in Tissues of Infants Without Peroxisomes (Zellweger Syndrome). (1983) Nature 306, 69–70. 7. L. Defects of Bile Acid Synthesis in Zellweger’s Syndrome. (1979) Science 203, 1107–1108. 8. J. 3-Hydroxy-3-Methylglutaryl Coenzyme A Reductase Is Present in Peroxisomes in Normal Rat Liver Cells. (1985) Proc. Natl. Acad. Sci. USA 82, 770–774. Copyright © 1998 by AOCS Press 38 M. Martinez 9. W. A New Dietary Therapy for Adrenoleukodystrophy: Biochemical and Preliminary Clinical Results in 36 Patients.

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