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  • Selenium  (7)
  • Developing countries  (2)
  • GABA  (2)
  • Therapy  (2)
  • 1
    Electronic Resource
    Electronic Resource
    Selenium content of human milk, cow's milk and cow's milk infant formulas (1978)
    Springer
    European journal of pediatrics 129 (1978), S. 139-145 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00442157
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  • 2
    Electronic Resource
    Electronic Resource
    Beta cell nesidioblastosis (1978)
    Springer
    European journal of pediatrics 127 (1978), S. 75-89 
    Details
    ISSN: 1432-1076
    Keywords: Hyperinsulinism ; β-cell hyperplasia ; Nesidioblastosis ; Newborn ; Infants ; Somatostatin ; Electron microscopy ; Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two patients with severe hypoglycemia since birth are described. In both hyperinsulinism was demonstrated during spontaneous hypoglycemic attacks or could be provoked by various tolerance tests. In case I considerable obesity and psychomotor retardation was present at the age of one year whereas in case II weight gain was normal and development unaffected. Immunofluorescence microscopic and electron microscopic examination of the pancreas after subtotal pancreatectomy revealed diffuse islet cell hyperplasia with nesidioblastosis in case I and β-cell nesidioblastosis in case II. The hyperplastic and nesidioblastotic areas consisted mainly of β-cells. In addition, an accumulation of somatostatin producing cells was observed in case I, and some cells were found with ultrastructural signs of both endocrine and exocrine function. In both cases, pancreatic insulin release was inhibited by a prolonged somatostatin infusion. The results of tolerance tests did not allow a diagnosis of the underlying pancreatic lesion. In case II, leucine-sensitive hypoglycemia detected soon after birth, was present even after subtotal pancreatic resection. Therapeutic trials with diazoxide in case I and a leucine-restricted diet in case II were only of temporary benefit. After subtotal pancreatectomy there was clinical improvement in both cases, but case II still needs a leucine-restricted diet. The familial occurrence of persistent hypoglycemia in both cases suggests that β-cell nesidioblastosis may be a hereditary disorder.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445763
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  • 3
    Electronic Resource
    Electronic Resource
    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
    Keywords: Selenium ; Glutathione peroxidase ; Selenium deficiency ; Selenium supplementation ; Nutrition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442405
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  • 4
    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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  • 5
    Electronic Resource
    Electronic Resource
    The selenium state of healthy children (1977)
    Springer
    European journal of pediatrics 125 (1977), S. 81-88 
    Details
    ISSN: 1432-1076
    Keywords: Selenium ; Glutathione peroxidase ; Normal values ; Blood ; Infants ; Children ; Milk ; Nutrition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00470608
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  • 6
    Electronic Resource
    Electronic Resource
    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
    Keywords: Plasma proteins ; Immunoglobulins ; Circulating immune complexes ; Anthropometry ; Developing countries
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445927
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  • 7
    Electronic Resource
    Electronic Resource
    Atypical vitamin B12-unresponsive methylmalonic aciduria in a sibship with severe progressive encephalomyelopathy: a new genetic disease? (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 398-403 
    Details
    ISSN: 1432-1076
    Keywords: Key words Methylmalonic ; aciduria ; Vitamin B12 ; GABA ; Cerebrospinal fluid ; Encephalomyelopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on two siblings, a girl of 7 years and a boy of 2 years, who presented in infancy with hypotonia, athetoid movements, myopathy and severe developmental delay. The progressive clinical course was characterized by ophthalmoplegia, pyramidal tract signs, loss of visual contact and failure to thrive. The older sister died at the age of 7 years. The younger brother followed an almost identical clinical course. MRI of the brain revealed bilateral hypodensities and atrophy of the putamen. Neurophysiological investigations were consistent with peripheral neuropathy. Investigations for neurometabolic disorders in urine, plasma and CSF of both patients revealed a consistent increase of methylmalonic acid in urine, plasma and CSF as well as borderline low free GABA in CSF. Except for an inconstant elevation of lactate in the boy, metabolic acidosis, hypoglycaemia, episodic ketoacidosis, or hyperammonaemia, the usual concomitants of organoacidopathies, were absent in both children. Homocystinuria was excluded. Methylmalonic aciduria did not respond to antibiotic treatment, vitamin B12 therapy nor dietary protein restriction. Incorporation of [14C]propionate into protein in cultured fibroblasts was pathologically but inconsistently decreased. Both patients’ cell lines showed only minimal response to hydroxocobalamin and normal methylmalonyl-CoA mutase activity. Conclusion Even though the definitive underlying enzymatic defect in this sibship remains obscure our results suggest a new genetic disorder. This report illustrates that hitherto undescribed metabolic disorders remain to be elucidated even in long investigated areas of intermediary metabolism such as methylmalonic aciduria.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01955272
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  • 8
    Electronic Resource
    Electronic Resource
    Selenium intake of infants and young children, healthy children and dietetically treated patients with phenylketonuria (1984)
    Springer
    European journal of pediatrics 143 (1984), S. 99-102 
    Details
    ISSN: 1432-1076
    Keywords: Selenium ; Intake ; Children ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 20 healthy infants and children, 5–20 months old, the Se intake was estimated by analysing food samples by instrumental neutron activation analysis. The intake was calculated by weighing the portions offered and actually consumed. The median Se content of the food amounted to 27 ng/g wet weight (gww) and median daily Se intake to 33.5 μg. The Se intake was not equally distributed over the day. About 50% of the daily Se intake was derived from the supper. The main Se sources (41%) for young children were cereal paps. Commercially, available meals (30 ng/g) contained less Se than home-made ones (50 ng/g). In nine dietetically treated patients with phenylketonuria the median Se intake amounted only to 6.9 μg/day corresponding to a mean Se content of the diet of 7.9 ng/g. The main Se source in the diet was vegetables (36.3%) and 20% derived from their protein supplements. The Se intake of young children, healthy or dietetically treated, cannot be calculated accurately from tables but must be estimated by measuring the Se content of the local food because cereals and vegetables-the main Se sources-exhibit great regional variations.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445794
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  • 9
    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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  • 10
    Electronic Resource
    Electronic Resource
    The selenium state of children (1978)
    Springer
    European journal of pediatrics 128 (1978), S. 213-223 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00445606
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