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Lipid status and fatty acid metabolism in phenylketonuria
No full textOwing to dietary treatment, patients with phenylketonuria (PKU) are
supplied with only small amounts of animal fats and show low blood cholesterol
levels and a poor long-chain polyunsaturated fatty acid (LC-PUFA) status.
Endogenous synthesis should compensate for the physiological need for cholesterol
for growth, but supplementary exogenous LC-PUFA seems advisable. PKU infants
could develop depletion of docosahexaenoic acid, fundamental for the functional
development of the central nervous system. The availability of arachidonic acid
from plasma in PKU has been found to be related to dietary compliance, and seems
to influence the synthesis of arachidonate-derived eicosanoids. Trials of dietary
supplementation of PKU patients with oils containing LC-PUFA are in progress, but the functional consequences of these supplementations need further investigation
Lipid status and fatty acid metabolism in phenylketonuria
No full textOwing to dietary treatment, patients with phenylketonuria (PKU) are supplied with only small amounts of animal fats and show low blood cholesterol levels and a poor long-chain polyunsaturated fatty acid (LC-PUFA) status. Endogenous synthesis should compensate for the physiological need for cholesterol for growth, but supplementary exogenous LC-PUFA seems advisable. PKU infants could develop depletion of docosahexaenoic acid, fundamental for the functional development of the central nervous system. The availability of arachidonic acid from plasma in PKU has been found to be related to dietary compliance, and seems to influence the synthesis of arachidonate-derived eicosanoids. Trials of dietary supplementation of PKU patients with oils containing LC-PUFA are in progress, but the functional consequences of these supplementations need further investigation
Fatty acid status in treated galactosaemia
No full textNeurodevelopmental problems in patients who have been treated early for galactose-
1-phosphate uridyltransferase deficiency (classical galactosaemia, McKusick 230400)
are quite common, even when the dietary therapy is not delayed and is apparently
well followed (Komrower 1982). Removing dairy products from the diet does not
seem to reduce the incidence of the deleterious outcomes. Thus, new studies in
biochemical, endocrinological and genetic parameters are needed.
We checked in treated galactosaemics the status of the major n-3, n-6 and n-9
unsaturated fatty acids (FA) critical for the development of growing tissues, particularly
the central nervous system (Martinez 1992). Cases included 5 subjects (3 male
and 2 female, aged 3-14 years) treated from the first month of life for classical
galactosaemia, whose anthropometric indices averaged the 50th centile both for
weight and height. Controls consisted of 8 subjects (6 male and 2 female, aged
3-14.5 years), clinically healthy and on a free diet. For each subject, weight was
appropriate for height. In both groups the macronutrient supply was investigated by
a 24-h recall. A blood sample was drawn after an overnight fast to determine
circulating lipid levels and the FA composition of plasma and erythrocyte phospholipids.
Circulating plasma lipids (total and high-density lipoprotein cholesterol,
triglycerides, phospholipids) were determined through enzymatic methods. For
measurement of FA levels, lipids were extracted with chloroform-methanol (Folch) and phospholipids were separated by thin-layer chromatography. FA methyl esters
were analysed with a model 4160 Carlo Erba gas chromatograph equipped with
Supelcowax 10 capillary column and were expressed as weight percentages.
The analysis of the nutrient supply showed lower protein and saturated fat and
higher monounsaturated fat intake in galactosaemics; the calorie supply was similar
in both groups. No differences were detected between groups for any of the lipidic
classes. As regards the FA status, galactosaemics exhibited higher oleic acid (18:1 n-9,
median 12.5%, range 10.9-13.2%, vs median 10.3%, range 8.49-12.0% in controls,
p < 0.05 (Mann-Whitney U-test)) and lower eicosapentaenoic acid (20 : 5 n-3, median
0.25%, range 0.18-0.41%, vs median 0.43%, range 0.12-0.79% in controls, p < 0.05)
levels in plasma phospholipids. No significant differences were present in the FA
composition of erythrocyte phospholipids.
Milk and dairy products are rich in saturated fats, so their avoidance could be at
the origin of the differences in the dietary habits and in the phospholipid level of
18:1 n-9 FA in galactosaemics. Nevertheless, in contrast to other metabolic disorders
(Holman and Johnson 1981; Galli et al 1991), the FA status of subjects treated for classical galactosaemia seems to be minimally affected by the dietary treatment
and/or the metabolic defect. The observed differences should not have a role in the clinical course of the disease
Fatty acid metabolism in phenylketonuria
No full textChildren treated for phenylketonuria (PKU) have a low intake of whole animal foods. Consequently, the dietary intake of long-chain polyunsaturated fatty acids (PUFA) is just a few milligrams per day, mostly represented by arachidonic acid (AA). In a consecutive series of studies, we assessed in treated PKU children their long-chain PUFA status, the AA-related eicosanoid synthesis and the effects of specific PUFA supplementations. We found that the good compliance with the dietary regimen negatively influences the long-chain PUFA status and serum eicosanoid release from platelets. Supplementation with either marine or blackcurrant oils modifies the long-chain PUFA status of PKU children without approaching the fatty acid pattern of a healthy control population. Good-compliant PKU patients have diet-related, low levels of circulating long-chain PUFA, whose clinical and functional consequences deserve further investigation. The effects of dietary supplementations with long-chain PUFA of both the n-6 and n-3 series should be carefully evaluated
Effect of diet on the lipid and fatty acid status of full-term infants at 4 months
No full textOBJECTIVE: To compare the effects of the exogenous supply of long-chain polyunsaturated fatty acids (LCP) and cholesterol on the lipid and fatty acid status in full-term, 4-month old infants.
METHODS: Twenty-three infants received a standard infant formula while twenty-one were given a formula enriched with LCP and cholesterol in a prospective, randomized study. The composition of the two formulas differed only in fat quality. A group of fifteen breastfed infants fed was used as reference. No one was complemented with solid foods before blood sampling at 4 months of life.
RESULTS: Differences in total-cholesterol and low-density lipoprotein-cholesterol (LDL-C) levels between feeding groups were mainly gender-related. Dietary cholesterol tended to increase LDL-C plasma levels. The breastfed and the enriched formula-fed groups had higher levels of circulating LCP than the group that received the standard formula. In the erythrocytes of infants fed the standard formula, C22:6 n-3 levels were less than 50% those of the breastfed and the enriched formula-fed ones. Higher C20:4 n-6 levels were found in the erythrocytes of the enriched formula-fed group.
CONCLUSIONS: Formula-fed, full-term infants maintain a lipid and fatty acid status close to that of breastfed infants when supplied with dietary LCP and cholesterol
The effects of n-3 and n-6 polyunsaturated fatty acids on plasma lipids and fatty acids of treated phenylketonuric children
No full textDietary-treated phenylketonuric patients (PKUs) display low levels of long-chain polyunsaturated fatty acids (PUFA) in plasma lipids. In a 6-month clinical trial we observed a decrease of triglycerides and an increase of n-3 long-chain PUFA in plasma of PKUs supplemented with fish oil, while no major differences in respect to the baseline values were found in a group supplemented with blackcurrant oil. A more complete source of long-chain PUFA of both the n-6 and n-3 series should be investigated for dietary supplementation of PKU patients
Preliminary results of a nutritional survey in a sample of 35,000 Italian schoolchildren
No full textAn investigation of the nutrient intake of a large-scale sample (n = 35,072), drawn from the Italian school-age population (7-10 years) was carried out in a nationwide survey of nutritional patterns. Friuli, the Piedmont, Latium and Sicily regions were selected as representative of the nation's north-south and east-west socio-economic divisions. A food frequency questionnaire was used to assess nutritional intake. Traditional methods of 24-h dietary recall and a weighted food diary were used in subsamples to assess the validity of the food frequency questionnaire. Our data suggest that the average diet of Italian schoolchildren is rich in protein (especially animal proteins) and lipids (prevalently saturated fatty acids), but that carbohydrate and fibre intakes remain generally low. The relatively low calcium intake among girls and a widespread, more than adequate iron intake are also noteworthy. The food and nutrient intakes assessed suggest a dietary pattern with several positive points, but also reveal potential hazards for a wider population spectrum. The type of large-scale nutritional monitoring with a multi-method approach can be used in Italy and elsewhere to describe the dietary trends of a school-age population
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