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  • Maple syrup urine disease  (2)
  • Plasma  (2)
  • Whole body counter  (2)
  • 1
    Electronic Resource
    Electronic Resource
    Plasma glutathione peroxidase after selenium supplementation in patients with reduced selenium state (1982)
    Springer
    European journal of pediatrics 138 (1982), S. 138-140 
    Details
    ISSN: 1432-1076
    Keywords: Selenium ; Supplementation ; Plasma ; Glutathione peroxidase ; Glutathione S-transferase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The plasma glutathione peroxidase (GSHPx) activity was measured in normal adults and children and in patients with reduced selenium state because of dietary treatment of metabolic diseases (phenylketonuria or maple-syrup-urine disease) before and after selenium supplementation. Besides GSHPx (measured with t-butyl hydroperoxide, cumene hydroperoxide and hydrogen peroxide as acceptor substrates) the activity of glutathione S-transferase was estimated in plasma. Plasma GSHPx activity in healthy children was significantly lower than in healthy adults. In 11 dietetically treated patients with phenylketonuria or maple-syrup-urine disease the plasma GSHPx was reduced to about 17% of the values of healthy children of the same age. No glutathione S-transferase activity could be found in plasma of children in normal or reduced Se state. During administration of yeast rich in Se (200μg Se/d) for 90 days 2 healthy adults showed no significant change of plasma GSHPx activity. During Se supplementation (75–100μg Se/d) for 120–163 days 5 dietetically treated patients with PKU or MSUD exhibited a significant increase of plasma GSHPx activity within 2 days. The values reached a plateau after 1 to 3 weeks of supplementation and remained at this level within the following 4 to 5 months. Therefore, the activity of plasma glutathione peroxidase can be used as an indicator of short-term changes of selenium intake in selenium deficient individuals.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441140
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    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
    Keywords: Maple syrup urine disease ; Acute phase of MSUD ; Insulin treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01377350
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  • 3
    Electronic Resource
    Electronic Resource
    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
    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
    URL: http://dx.doi.org/10.1007/BF00445928
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    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
    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
    URL: http://dx.doi.org/10.1007/BF00442499
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  • 5
    Electronic Resource
    Electronic Resource
    Primary hypomagnesemia (1975)
    Springer
    European journal of pediatrics 118 (1975), S. 249-258 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00492330
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  • 6
    Electronic Resource
    Electronic Resource
    Akrodermatitis enteropathica — eine Zinkstoffwechselstörung mit Zinkmalabsorption (1975)
    Springer
    European journal of pediatrics 120 (1975), S. 181-189 
    Details
    ISSN: 1432-1076
    Keywords: Acrodermatitis enteropathica ; Zinc ; Malabsorption ; Therapy ; Zinc retention ; Zinc elimination ; Whole body counter
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 3 Patienten mit Akrodermatitis enteropathica wurde mit Hilfe der Ganzkörpermessung nach oraler Applikation von 65Zn eine verminderte intestinale Zinkresorption gedunden. Dagegen war die Zinkelimination aus dem Körper normal. Die Zinkkonzentration im Serum war bei den Patienten stark erniedrigt. Alle klinischen Symptome verschwanden nach sehr hohen oralen Zinkdosen. Diese Befunde sprechen um so mehr für eine ursächliche Rolle des Zinks in der Pathogenese der Akrodermatitis enteropathica, als bei dieser Krankheit ultrastrukturelle Veränderungen in den Panethschen Zellen nachzuweisen sind [12], die auch beim Zinkmangel der Ratten gefunden wurden [Beitr. Path. 145, 336 (1972)].
    Notes: Abstract The intestinal resorption of zinc using 65ZnCl2 was estimated in 3 patients with acrodermatitis enteropathica, 2 healthy controls, and 3 heterozygotes. After oral application of 65Zn the whole body activity was measured by a whole body counter for 34 days. The 65Zn resorption of the patients amounted to 16, 42 and 30% of the applied dose, whereas the resorption values of the heterozygotes and the controls were in the range of 58 and 77%. The elimination of 65Zn from the body amounted to about 0.7% of the applied dose with no difference between controls and patients with acrodermatitis enteropathica. Before therapy the serum-zinc levels of patients were markedly decreased. After oral application of high doses of zinc aspartate (2×400 mg/day) all clinical symptoms disappeared within a week. The results point at a causal connection between zinc and the pathogenesis of acrodermatitis enteropathica. Ultrastructural alterations of the Paneth cells of the intestine are also shown in this disease [12] as have also been seen in Paneth cells of zinc deficient rats [Beitr. Path. 145, 336 (1972)].
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00439007
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    Paper (German National Licenses)
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