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  • 11
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 145 (1986), S. 109-115 
    ISSN: 1432-1076
    Keywords: Essential fatty acids ; Delta-6-desaturase ; Nigeria ; Protein energy malnutrition ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The fatty acid (FA) composition of the main plasma lipids was analysed in eight well-nourished, generally healthy Nigerian children aged 14.1±7.2 months and in 17 malnourished children (8 marasmus, 9 kwashiorkor) aged 14.6±3.8 months within the first 2 days of admission at the Dept. of Child Health, University of Benin. In comparison to the control group, the malnourished children showed a marked decrease of polyunsaturated FA with low linoleic acid, mainly in sterolesters (STE), and severely reduced linoleic acid metabolites, including arachidonic acid, in all lipid fractions. ω-3-FA were not altered except for a reduction of docosapentaenoic and docosahexaenoic acids in phospholipids. Clearly increased values were found for saturated FA in STE and for the non-essential monoenoic FA in all lipid classes. This pattern indicates the presence of essential fatty acid deficiency in the malnourished children. There was no significant difference between marasmus and kwashiorkor. Eight malnourished children were followed up in the early phase of recovery during hospital treatment 14.0±3.1 days after obtaining the first sample. Linoleic acid had increased again in STE, but its metabolites were as low or even lower than before. An impaired activity of delta-6-desaturase, the rate limiting enzyme of linoleic acid metabolism, in suggested by elevated substrate-product-ratios of this enzyme in untreated children with protein energy malnutrition and in the early phase of recovery, which may be due to low insulin levels, protein and zinc deficiency. The trientetraen-ratio (20∶3ω9/20∶4ω6) thus is not a reliable indicator of essential FA status in protein-energy malnutrition.
    Type of Medium: Electronic Resource
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  • 12
    ISSN: 1432-1076
    Keywords: Essential fatty acids ; Plasma ; Developing countries ; Africa ; Child nutrition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 13
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 146 (1987), S. 92-93 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 14
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 153 (1994), S. S1 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 15
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 118 (1975), S. 249-258 
    ISSN: 1432-1076
    Keywords: Primary hypomagnesemia ; Hypocalcemia ; Intestinal absorption ; Magnesium ; Whole body counter
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Der klinische Verlauf zweier Patienten mit primärer Hypomagnesämie wird beschrieben. Bei einem Patienten, einem 5 Monate alten Säugling, wurden Magnesiumretention,-resorption,-Stuhlausscheidung und-Clearance gemessen. Die Retention (2,8%) und Resorption (7,8%) von Magnesium28 war deutlich erniedrigt im Vergleich zu Kontrollpersonen, die durchschnittlich 25% retinierten und 28% absorbierten. Zwischen den Retentionswerten der Eltern und Verwandten und denen der gesunden Erwachsenen bestand kein Unterschied. Der untersuchte Patient wurde erfolgreich mit Trimagnesiumdicitrat, entsprechend einer Dosis von 1,75 g Magnesium, behandelt.
    Notes: Abstract The clinical course of 2 patients with primary hypomagnesemia is reported. In one male patien, 5 months old, measurements of magnesium retention, intestinal absorption, fecal excretion and renal clearance were performed. The retention (2.8%) and absorption (7.8%) of 28-Mg were markedly reduced in comparison to controls (average retention 25% and average absorption 28%). The retention values of the parents and other relatives did not differ from those of healthy adults. The examined patient was successfully treated with trimagnesium dicitrate containing 1.75 g magnesium per day.
    Type of Medium: Electronic Resource
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  • 16
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 128 (1978), S. 213-223 
    ISSN: 1432-1076
    Keywords: Selenium ; Gluthathione peroxidase ; Dietotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The selenium content of serum, whole blood and hair was measured by neutron activation analysis in dietetically treated patients with phenylketonuria (PKU) and maple-syrup-urine disease (MSUD). Follow-up studies showed a decrease of the serum selenium content and the glutathione peroxidase activity of erythrocytes—a selenoenzyme—from normal values at the beginning of the diet to 20% (selenium) and 50% (gluthione peroxidase) of normal within 10–12 weeks of dietary treatment. In 36 patients the serum selenium content was lower at 6.7–28×10-9 g/ml, independent of the age of the patients (0.5 to 10 years). The selenium content of whole blood was reduced: median=98×10-9 g/g dry weight; range 75 to 165×10-9 g/g dry weight (healthy children: median=381×10-9 g/g dry weight; range 245 to 588×10-9 g/g dry weight). The selenium content of hair was markedly lower in the patients (median=62×10-9 g/g; range 13–140×10-9 g/g) than in healthy children (median=429; range 213 to 720×10-9 g/g). The mean glutathione peroxidase activity of erythrocytes was reduced to 4.6±0.64 U37/g Hb, in comparison to normal values (mean=8.8±0.88 U37/gHb).
    Type of Medium: Electronic Resource
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  • 17
    ISSN: 1432-1076
    Keywords: Neuronal ceroid-lipofuscinosis ; Serum lecithin pattern ; Arachidonic acid ; Linoleic acid ; Ultrastructural inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A patient with a progressive neurological disorder beginning at the age of three years is described. Mental and visual disturbances were the first signs, soon followed by ataxia and myoclonic jerks. Fundoscopy revealed a decreased pigmentation of the retina. Ultramicroscopic investigations of muscle and skin disclosed the typical changes seen in the late infantile and juvenile forms of neuronal ceroid-lipofuscinosis. In contrast to the clinical and ultrastructural findings, the fatty acid pattern of the serum lecithin showed a significant increase of arachidonic acid and a corresponding decrease of linoleic acid which is characteristic of the so-called infantile form of neuronal ceroid-lipofuscinosis (Hagberg-Santavuori variant; polyunsaturated fatty acid lipidosis). The obvious heterogeneity of the clinical, histological and laboratory findings within the subgroups of neuronal ceroidlipofuscinosis is briefly discussed.
    Type of Medium: Electronic Resource
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  • 18
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 129 (1978), S. 139-145 
    ISSN: 1432-1076
    Keywords: Selenium ; Human milk ; Cow's milk ; Cow's milk infant formula
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The selenium content of human milk, cow's milk and cow's milk infant formula were estimated by instrumental neutron activation analysis. The highest values were found in 3 samples of human colostrum (524–865×10-9 g/g dry weight). There was a significant decrease with increasing time post partum. Mature human milk exhibited a selenium content of 230±79×10-9 g/g dry weight. The selenium content of 45 samples of cow's milk from the north-western area of Germany was 200±39×10-9 g/g dry weight. While there was no significant difference between the values of mature human milk and of cow's milk, cow's milk infant formula exhibited significantly (P〈0.01) lower values than human milk. The average selenium content of 107 samples of 10 different commercially available fluid and powdered cow's milk infant formulas (range: 18–171×10-9 g/g dry weight) amounted to about only one third of that in mature human milk.
    Type of Medium: Electronic Resource
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  • 19
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 126 (1977), S. 199-202 
    ISSN: 1432-1076
    Keywords: Zinc ; Serum ; Normal values ; Children ; Infants ; Cord blood ; Adults
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Serum zinc concentrations in subjects of different ages were estimated by neutron activation analysis to obtain reliable reference values for the diagnosis of primary or secondary zinc deficiency during childhood. In these healthy individuals a small age-dependent variation of the mean values was found. Serum zinc concentrations were lower in cord blood (mean value: 880×10−9 g/ml) and in infants (mean value: 820×10−9 g/ml) than in adults (mean value 1178×10−9 g/ml). In comparison to these values, the serum zinc concentration in a patient with secondary zinc deficiency and 4 patients with acrodermatitis enteropathica was reduced to about 25% of normal.
    Type of Medium: Electronic Resource
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  • 20
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 293-296 
    ISSN: 1432-1076
    Keywords: Maple syrup urine disease ; Blood exchange transfusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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