Trace element status of PKU children ingesting an elemental diet

We assessed the trace metal status of 10 children (3.5 to 13.3 yr) with phenylketonuria (PKU) who were successively treated for at least 6 months with a semi-synthetic formula restricted in phenylalanine, then for 6 months with a reformulated formula. The reformulated product contained higher concentrations of magnesium (Mg), potassium (K), phosphorus (P), selenium (Se), and zinc (Zn) and lower concentrations of calcium (Ca), sodium (Na), and unsaturated fats. Diet records, blood samples, and urine specimens were collected from each subject. Mean intakes of Mg, K, and Zn increased significantly when subjects were switched to the reformulated product. The subject's mean intakes of Se (for all age groups) and Zn (children 11 to 14 yr old) were below 67% of recommended intakes while they were ingesting the original formula. The mean trace metal concentrations of urine, blood, and serum did not differ significantly during the two periods. Mean serum Zn, Se, and Cu concentrations of the subjects were less than those reported for normal children. Significant negative correlations were obtained between serum Zn concentrations and the molar ratio of dietary (Ca + P):Zn, and the molar ratio of dietary Fe:Zn. No significant differences in mean urinary Cu and Zn excretion (mg/g creatinine) were observed in subjects after they were changed to the reformulated product. Individuals whose primary source of energy and protein is derived from semi-synthetic elemental diets are especially at risk for multiple trace element deficiencies.     

Trace element status of children with PKU and normal children

The trace mineral (chromium, copper, iron, selenium, and zinc) status of 10 children (4 to 13.8 years) with phenylketonuria (PKU) and 9 normal children (6.5 to 15.9 years) was assessed. The children with PKU were treated with a phenylalanine-free L-amino acid mix that supplied the following percentages (mean +/- standard deviation) of total daily intake: energy, 45 +/- 13; protein, 75 +/- 11; copper (Cu), 62 +/- 10; iron (Fe), 82 +/- 7; selenium (Se), 40 +/- 20; and zinc (Zn) 87 +/- 8. Diet records and blood samples were collected from each subject. Children with PKU had significantly greater mean intakes of Cu, Fe, and Zn than normal children. Mean serum Cu, Fe, and Zn concentrations of the children with PKU and normal children were not different despite significantly greater intakes by the children with PKU. Normal children had a significantly greater mean serum Se concentration and a mean blood chromium concentration 1.6 times that of children with PKU. Individuals whose primary source of protein is an elemental diet are especially at risk for multiple trace mineral deficiencies. Manufacturers of chemically defined medical foods should evaluate composition, specifically molar ratios between minerals, as a basis for product formulation.     

Trace element nutrition status and dietary intake of children with phenylketonuria

The trace element status (copper, iron, zinc, manganese, chromium, and selenium) of 20 dietetically treated phenylketonuric (PKU) children was assessed. Significantly higher intakes of copper (p = 0.002) and iron (p = 0.005) were noted in PKU children compared with their siblings. No significant differences were found for zinc, manganese, or chromium. Intake of selenium was significantly lower (p = 0.0001) in PKU children (8.4 +/- 3.9 micrograms/d) than in siblings (41.6 +/- 9.4 micrograms/d). Plasma and urine selenium and erythrocyte glutathione peroxidase activity (GSHpx) were significantly lower (p = 0.001) in PKU children (0.38 +/- 0.11 mumol/L, 58.0 +/- 34.5 nmol/d, and 14.2 +/- 5.5 U/g Hb, respectively) than in siblings (0.82 +/- 0.15 mumol/L, 165.2 +/- 49.4 nmol/d, and 22.7 +/- 5.2 U/g Hb, respectively). No differences were found in plasma and urine concentrations of other elements. Intake of selenium was significantly correlated with erythrocyte GSHpx (r = 0.87, p = 0.0001) and plasma selenium (r = 0.71, p = 0.0001) for the combined groups. The need and possible procedures, including dietary manipulation, for increasing selenium intake in PKU subjects are discussed.     

Iron and zinc status in multiple sclerosis patients with pressure sores

Measurements of weighted dietary intakes and plasma determinations of albumin, iron, zinc, ascorbic acid and TIBC were carried out on twenty female multiple sclerosis patients in a long-stay hospital for disabled people. The group included ten patients with a recent history of pressure sores, closely matched with ten patients without pressure sores. Mean daily intake of carbohydrate was found to be higher in the non-pressure sore group whilst intake of zinc was lower in this group. Intakes of all other nutrients were comparable between the two groups. For both groups, intakes of energy, folate, vitamin D, iron and zinc were less than recommended values. Mean plasma levels of albumin and iron were towards the lower limit of the normal range, whilst that for zinc was considerably less than the normal range. Plasma TIBC was slightly above the normal range. Levels of plasma iron and zinc were significantly lower in the pressure sore group. The data indicate that severely disabled hospitalized patients with multiple sclerosis may be at risk of poor nutritional status. The results suggest that in the presence of pressure sores, there are increased requirements for specific nutrients, notably zinc and iron. Consideration is given to the possible value of supplementation of these individuals.     

Trace element intake and status of Italian subjects living in the Gubbio area

Daily intakes of certain trace elements (Pb, Cd, Ni, Hg, and Cr) were assessed using dietary history and weighed record methods and concurrent chemical analysis (CA) of duplicate portions, along with blood levels, in a group (21 M, 23 F) of inhabitants of the Gubbio area (Belvedere, Biscina, Scritto). The evaluation of only intake of trace elements was accomplished in 40 subjects (20 M, 20 F) 1 year later. In both surveys, trace element intakes were generally lower than the potential tolerable weekly intake. However, daily intakes of Pb, Cd, Ni, Hg, and Cr were higher in men compared to women in both surveys (P<0.05). In fact, intakes at the 50th percentile were greater in men by approximately 75% for Pb, 91% for Cd, 20% for Ni, 45% for Hg, and 29% for Cr in the first survey. In the second survey, percentage differences for Pb, Cd, Ni, and Cr were about 12%, 28%, 26%, and 26%, respectively. Blood trace element levels were slightly higher in men in the first survey. Interestingly, no correlation was observed between the intake of trace elements and corresponding blood values. Food basket and total diet, which were computed from the weighed record method for 2 days, showed relevant disagreements with CA of duplicate portion. Moreover, the comparison in content of Pb, Cd, Ni, and Cr obtained by CA of cooked dishes or by calculation using values of raw foods showed significant differences (P<0.05). It was concluded that trace element intake should be assessed by CA of duplicate portion.     

Evidence for genetic regulation of vitamin D status in twins with multiple sclerosis

BACKGROUND: Multiple sclerosis (MS) risk is determined by both genes and environment. One of the most striking features of MS is its geographic distribution, particularly the pattern of high MS frequency in areas with low sunlight exposure, the main inducer of vitamin D synthesis. Recent epidemiologic, experimental, and clinical evidence support an effect for low environmental supplies of vitamin D in mediating an increased susceptibility to MS. OBJECTIVES: We 1) examined the association of serum 25-hydroxy-vitaminD [25(OH)D] concentrations and MS status and 2) assessed the genetic contribution to serum 25(OH)D concentrations and tested for its association with genetic variants in 2 candidate genes [vitamin D receptor and 1-alpha-hydroxylase (CYP27B1)]. DESIGN: We used a twin study approach, comprising adult pairs identified from the longitudinal population-based Canadian Collaborative Project on Genetic Susceptibility to MS. Monozygotic (MZ; n = 40) and dizygotic (DZ; n = 59) pairs, both concordant and discordant for MS, were studied. End-of-winter serum 25(OH)D concentrations were measured by radioimmunoassay, and genotypes were assessed by single nucleotide polymorphism (SNP) assay. RESULTS: Serum concentrations of 25(OH)D were highly correlated in MS-concordant pairs (r = 0.83, P < 0.001), but they were not significantly associated with having the disease (P = 0.4) when analyzed by logistic regression. Intraclass correlation for 25(OH)D concentration was significantly greater in MZ pairs (MZ, r: 0.71 > DZ r: 0.32, P = 0.006). Significant associations of 2 CYP27B1 SNP variants and 25(OH)D concentrations were observed. CONCLUSION: The findings indicate important genetic influences on regulation of seasonal circulating 25(OH)D concentrations in MS twins.     

Survival in multiple sclerosis

Survival from onset of symptoms was calculated for two series of MS patients, a population-based epidemiologic series from southern Lower Saxony of 224 cases, and a revised hospital series of 1429 cases from 11 neurologic centers throughout the Federal Republic of Germany. Median survival for the epidemiologic series was 35-42 years, with no finer estimate possible. Observed survival was 3/5 expected survival after 35 years of illness. Survival in this series was similar to that from two other population-based reports. No significant difference was found between the sexes, though patients with onset at age 35 + had a significantly shorter survival than those with onset at age under 35 years. The much larger hospital series, where median survival was 30 years, demonstrated significant differences separately favoring females and young onsets. However, the ratios of observed to expected survival from population life tables were strikingly similar among all four subgroups defined by sex and age at onset. While overall there is a substantial reduction in the expectation of life in MS, the differential survival by sex and age appears to be a reflection of the expected survival by sex and age per se, and not that of the disease.     

Trace element deficiency and toxicity

Trace elements are involved in enzymatic activities, immunological reactions, physiological mechanisms and carcinogenesis. Deficiency in some trace elements, such as iron and iodine, is still an important health problem, especially in developing countries. Some groups of individuals are more likely to develop trace element deficiency. The role of trace elements deficiency is suspected in various clinical situations and is now confirmed by well designed supplementation studies. Although toxicity of trace elements with clinical manifestations is rare, it has been observed that manganese toxicity may occur in patients receiving parenteral nutrition. Recent data about trace elements deficiency and toxicity are indicated in this review.     

Trace element intakes of women

1. Energy, protein, zinc, copper, manganese, selenium and dietary fibre intakes of 100 pre-menopausal women (mean age 30.0 +/- 6.1 years) from a university community, and consuming self-selected diets, were calculated using 3 d dietary records and food composition values. Subjects also collected a 24 h food composite during the 3 d record period for analysis of Zn, Cu and Mn by atomic absorption spectrophotometry. Daily analysed intakes were compared with those calculated from the corresponding record day. 2. Mean daily calculated intakes of energy, protein, Zn, Cu, Mn, Se and dietary fibre were 7.54 +/- 1.61 MJ, 74 +/- 18 g protein, 10.1 +/- 3.3 mg Zn, 1.9 +/- 0.6 mg Cu, 3.1 +/- 1.5 mg Mn, 131 +/- 53 micrograms Se, 19.4 +/- 6.6 g dietary fibre. 3. Major food sources for each of the trace elements were (%): Zn meat + substitutes 43, dairy products 23.7; Cu breads and cereals 22, vegetables 21; Mn breads and cereals 47, fruits 12, Se meat + substitutes 38, breads and cereals 30. 4. Highly significant correlations (P = 0.001) were noted for analysed intakes of Zn, Cu and Mn and those calculated from the corresponding record day. Mean calculated intakes were higher (%): Zn 138, Cu 142, Mn 121, than corresponding mean analysed intakes (P = 0.01). However, the mean nutrient densities (mg/MJ) were comparable: Zn analysed 1.2, calculated 1.4; Cu analysed 0.2, calculated 0.2; Mn analysed 0.4, calculated 0.4. 5. All subjects met the Canadian Dietary Standard (CDS) recommended level for Cu but 48% received less than the CDS for Zn, 6% obtaining less than two-thirds of this recommended level. Daily Mn and Se intakes were similar to recent values for North American diets.