FATTY ACID STATUS OF PKU CHILDREN THREE YEARS AFTER
LCPUFA SUPPLEMENTATION
Giovannini M, Fiori L, Agostoni C, Colombo L,
Verduci E, Gianni’ ML, Riva E
Department of Pediatrics, San Paolo Hospital, I-20142 Milan, Italy
Background: Long-chain polyunsaturated
fatty acid (LCPUFA) have relevant roles in growth and neural development.
Phenylketonuric (PKU) children on dietary treatment are supplied with foods
lacking LCPUFA. In a previous trial a balanced supplementation with LCPUFA for one year restored physiological
LCPUFA levels in blood in 10-year old PKU.
Aim: We have determined the LCPUFA
status of PKU children who completed the previous trial three years after
completion of the supplementation.
Subjects and methods: 20 dietary
treated PKU (mean age 13.5 years), who had been randomized to receive a
supplementation with 0.5 g fat caps/4 kg body weight, were sampled after an
overnight fasting. Fat caps provided either 26% fatty acids (FA) as LCPUFA
(18:3n-6 = 4.6%, 20:4n-6 = 7.4%, 20:5n-3 = 5.5%, 22:6n-3 = 8%) or olive oil
(control group). The composition of FA methyl esters (wt%) of plasma total
lipids, TL and lipid fractions (phospholipids, PL, cholesterol esters, CE,
triglycerides, TG) has been measured with capillary gas-chromatography.
Statistics:
non-parametric tests.
Results: The two groups did not
differ for the major LCPUFA levels in plasma TL, CE and PL. A trend for
higher levels of n-3 LCPUFA (20:5n-3 and 22:6n-3) was present in plasma TG
of the supplemented group. In this group the 22:6n-3 levels in plasma PL
three years before were associated with the actual 22:6n-3 levels in plasma
TG (Rsq=0.41, P=0.03).
Conclusions: Since plasma PL
reflect current dietary habits and fasting plasma TG are a partial marker
of stored fats, a supplementation with LCPUFA may have a long-term effect
in treated PKU.