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  • Developing countries  (2)
  • Maple syrup urine disease  (2)
  • Nutrition  (2)
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  • 1
    Digitale Medien
    Digitale Medien
    Maple syrup urine disease-therapeutic use of insulin in catabolic states (1982)
    Springer
    European journal of pediatrics 139 (1982), S. 172-175 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Maple syrup urine disease ; Acute phase of MSUD ; Insulin treatment
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract High and neurotoxic blood levels of leucine and its ketoanalogue develop in catabolic patients with maple syrup urine disease. The use of relatively high doses of insulin and additional glucose had a more pronounced effect on lowering leucine (and α-ketoisocaproate) blood levels than dietary elimination of leucine alone. This is demonstrated in 2 neonates after blood exchange transfusion and in one 4-months old patient suffering from febrile diarrhea.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF01377350
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  • 2
    Digitale Medien
    Digitale Medien
    The selenium state of healthy children (1977)
    Springer
    European journal of pediatrics 125 (1977), S. 81-88 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Selenium ; Glutathione peroxidase ; Normal values ; Blood ; Infants ; Children ; Milk ; Nutrition
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The selenium concentration of serum is age-dependent. The median value at birth ( $$\tilde x$$ =50×10−9g/ml) amounts to half of the median value of adults ( $$\tilde x$$ =102×10−9g/ml). After a decrease in early infancy to $$\tilde x$$ =34×10−9g/ml it steadily increases to $$\tilde x$$ =58×10−9g/ml in the second half of the first year, to $$\tilde x$$ =82×10−9g/ml in 1–5 year old children, and to $$\tilde x$$ =92×10−9g/ml in school children. The activities of the selenium containing enzyme glutathione peroxidase of erythrocytes are also reduced in early infancy (x=7.2±0.36 U37/g Hb), whereas the enzyme activities of cord blood erythrocytes (x=8.72±0.76 U37/g Hb) are in the same range as those of older children or adults. The selenium content of some commercially available milk formulas for infants are lower than those of human and cow's milk.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00470608
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  • 3
    Digitale Medien
    Digitale Medien
    Investigation of the nutritional state of children in a Congolese village (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 159-163 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Essential fatty acids ; Plasma ; Developing countries ; Africa ; Child nutrition
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The fatty acid status of an unselected group of 84 Congolese children was determined by estimating the fatty acid composition of the plasma phospholipid (PL) and cholesterolester (CE) fractions using capillary gas chromatography. In comparison with North American and European children a wide range of values and low mean percentages (related to the fatty acids of a chain length of 14–24 carbon atoms) were found for the following fatty acids (mean±SD): linoleic acid (LA) (PL: 17.19±3.85; CE: 40.13±7.72); arachidonic acid (AA) (PL: 6.60±2.28; CE 4.32±1.71); dihomo-gammalinolenic acid (DHLA) (PL: 1.80±0.68; CE 0.41±0.22). On average, ω3 fatty acids were higher than in reference groups, while similar values were found for the monoenoic and ω9 fatty acids. No sample contained eicosatrienoic acid, 20:3ω9 (ETA) which, dependent on analytical methods, may be difficult to separate from behenic acid (22:0). Changes in the relation between the two lipid fractions occurred when the LA content in CE was less than 35%. Also the correlation between LA and AA in CE, which was significant below 35% LA (r=0.84), changed with higher values for LA (r=0.01). These findings may indicate that alterations in fatty acid metabolism occur when LA values in CE — which are supposed to be directly related to the dietary intake of polyunsaturated fatty acids — fall below 35% of all fatty acids in this lipid fraction.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00445928
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  • 4
    Digitale Medien
    Digitale Medien
    Selenium requirements in patients with inborn errors of amino acid metabolism and selenium deficiency (1980)
    Springer
    European journal of pediatrics 134 (1980), S. 65-68 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Selenium ; Glutathione peroxidase ; Selenium deficiency ; Selenium supplementation ; Nutrition
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The diets of 5 patients with phenylketonuria or maple-syrup-urine disease were supplemented with yeast which was rich in selenium. For 120 days the patients received 45 μg Se/day to increase the Se content of their diets to 10–12ng Se/Kjoule. Before supplementation the selenium content of serum (5–15 ng/ml) and whole blood (10–27 ng/ml), and the activity of the erythrocyte glutathione peroxidase (0.19–2.69 U37/g Hb), amounted to only 10–20% of normal. The serum selenium content reached normal values within 4 weeks of supplementation, followed by normalisation of the selenium content of whole blood within 4–8 weeks. Restoration of the activity of erythrocyte glutathione peroxidase took 9 to 15 weeks —the red cell life span. There was a significant positive correlation between the selenium content of the erythrocytes and the activity of erythrocyte glutathione peroxidase.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00442405
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  • 5
    Digitale Medien
    Digitale Medien
    Exchange transfusion in acute episodes of maple syrup urine disease (1982)
    Springer
    European journal of pediatrics 138 (1982), S. 293-296 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Maple syrup urine disease ; Blood exchange transfusion
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Two neonates with maple syrup urine disease were treated by exchange transfusion. Within 15 h blood leucine and KICA concentrations were lowered from 2.6 mM to 1.1 mM using 570 to 620 ml blood per kg body weight. The other branched-chain amino acid/keto acid pairs fell to normal. During exchange transfusion the patient's nitrogen balance seems to be negative. Further exchange transfusion was useless. More importantly the patient should be forced into an anabolic state by high caloric supply or insulin plus glucose treatment. More KICA than leucine was eliminated, however, KICA blood levels remained slightly higher than that of leucine indicating different leucine/KICA equilibria in extravascular compartments than in blood. In a given time interval exchange transfusion was more effective than peritoneal dialysis, probably due to a lack of an additional (peritoneal) membrane. Renal excretion of branched-chain amino and keto acids was very inefficient. The allegedly most toxic metabolite, KICA, had the lowest renal clearance of the branched-chain keto acids.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00442499
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  • 6
    Digitale Medien
    Digitale Medien
    Investigation of the nutritional state of children in a Congolese village (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 155-158 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Plasma proteins ; Immunoglobulins ; Circulating immune complexes ; Anthropometry ; Developing countries
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The nutritional status of an unselected group of 111 children from the village of Bouansa, People's Republic of the Congo, was studied. Comprehensive clinical examinations, anthropometrical measurements and analysis of albumin, prealbumin, ferritin, C-reactive protein (CRP), IgA, IgG, IgM, IgE, IgG- and IgM-circulating immune complexes (CIC) were carried out. The results show, by anthropometrical classification, a high prevalence of moderate malnutrition. Low levels of plasma proteins and high levels of immunoglobulins and CIC were found. No correlation between anthropometrical classification and plasma proteins was established. Children with increased levels of CRP showed low prealbumin values and increased levels of ferritin. Patterns of immunoglobulins and CIC were close to those found in other studies in tropical countries. To evaluate the anthropometrical and biochemical findings it is necessary to take into consideration the apparently healthy appearance of the children, which shows the degree of adaptation to the limited availability of food and the high rate of acute and chronic infections.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00445927
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