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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 142 (1984), S. 271-275 
    ISSN: 1432-1076
    Keywords: Choanal atresia ; Multiple congenital anomalies ; CHARGE-association
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Six patients with both choanal atresia (ChA) and additional malformations are described and another 110 cases with this combination reviewed from the literature. Our study of these cases supports the existence of the CHARGE-association (Coloboma, Heart Disease, Atresia of choanae, Retarded mental development and growth, Genital hypoplasia, Ear anomalies and deafness). Our findings suggest the inclusion of orofacial clefts and oesophageal atresia among the main symptoms of this association. A certain degree of facial dysmorphism (low set, dysplastic ears, retrogenia, antimongoloid slant of palpebral fissures and anteverted nares) was observed in each of our cases. Infants with the bilateral type of ChA plus cardiac defects and those with ChA plus renal malformations have a high mortality rate. The aetiology of the association is not clear. The recurrence risk may be low.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Coeliac disease ; Diabetes mellitus type I ; Antigliadin antibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Screening for coeliac disease (CD) with serum antigliadin antibodies (AGA) was performed in 1032 diabetic children and adolescents. In 8 children CD had been diagnosed before study entry. Of the remaining 1024 children, 33 had an elevated AGA titre in the first serum sample. On follow-up an elevated AGA titre was confirmed in only 17 of 31 patients. Nine of the repeatedly positive patients underwent jejunal biopsy, and CD was diagnosed in two asymptomatic patients; both were positive for IgG- and IgA-AGA. Among 10 AGA-positive patients in whom biopsies could not be performed, only 1 showed IgA-AGA and thus carried a high risk for CD. From our results we estimate a prevalence of CD in Swiss and German diabetic children between 1.1% and 1.3%. Falsepositive AGA titres occurred significantly more often in patients with diabetes duration of less than 1 year. AGA testing teached a specificity of 99% if performed at least 1 year after the onset of diabetes. Children suffering from both diabetes and CD showed a diabetes manifestation at a significantly younger age than non-coeliac patients, whereas CD tended to be diagnosed at a remarkably late age.
    Type of Medium: Electronic Resource
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  • 3
    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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  • 4
    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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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 156 (1997), S. 142-147 
    ISSN: 1432-1076
    Keywords: Key words Lipids  ;  Infant nutrition  ;  Medium chain fatty acids  ;  Long-chain polyunsaturated fatty acids  ;  Trans fatty acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The fatty acid composition of human breast milk was determined longitudinally after term and preterm delivery by high resolution gas liquid chromatography. Milk samples were obtained at days 5, 10, 20 and 30 after term (n = 38) or preterm (n = 19) delivery. The saturated fatty acids C10:0 and C12:0 and the polyunsaturates linoleic acid (C18:2ω-6) and α-linolenic acid (C18:3ω-3) increased significantly from day 5 to day 10, whereas arachidonic acid (C20:4ω-6), total ω-6 long-chain polyunsaturates (LCP), docosahexaenoic acid (C22:6ω3) and total ω-3 LCP decreased significantly. Term and preterm milk did not differ in percentage content of linoleic acid, α-linolenic acid and LCP at any time point. Preterm milk contained significantly more medium and intermediate chain fatty acids (C10:0, C12:0 and C14:0) than term milk on days 5 (12.28 vs 9.78%; P 〉 0.05), 10 (16.25 vs 12.62%; P 〉 0.05) and 20 (17.29 vs 13.47%; P 〉 0.005). Conclusion The milk of mothers of preterm infants is not better suited to meet the high LCP requirements of their infants during the first weeks after birth. The slightly higher proportion of medium and intermediate chain fatty acids in preterm milk during the 1st month after birth might be advantageous for the fat and calcium absorption of preterm infants.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 157 (1998), S. 763-768 
    ISSN: 1432-1076
    Keywords: Key words Placental nutrient transfer ; Fetal nutrition ; Essential fatty acids ; Long-chain polyunsaturated fatty acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The fatty acid composition of plasma phospholipids, triglycerides, cholesterol esters and nonesterified fatty acids was determined by high-resolution capillary gas-liquid chromatography in 41 pairs of mothers and their term infants at time of birth. The total free fatty acid content in maternal and cord plasma was positively correlated, possibly reflecting a passive, gradient dependent transplacental passage of nonesterified fatty acids. Higher percentage values of several saturated and monounsaturated fatty acids in cord than in maternal plasma phospholipids, triglycerides and nonesterified fatty acids may have resulted from an active fetal fatty acid synthesis. Trans fatty acids were found in every lipid class at similar or slightly lower percentages in neonatal as in maternal plasma, thus confirming their placental passage. Long-chain n-6 and n-3 polyunsaturated fatty acids are preferentially incorporated into phospholipids and sterolesters of both maternal and cord plasma. Linoleic and α-linolenic acids were found in smaller portions in cord than in maternal fatty acids, in contrast to strikingly higher proportions of their long-chain polyunsaturated metabolites, which may indicate a discriminating placental transport for certain physiologically important long-chain polyunsaturated fatty acids. Conclusion The fetus appears to obtain fatty acids from a combination of de novo synthesis, a passive gradient dependent transplacental passage of nonesterified fatty acids and a selective materno-fetal placental transport for certain fatty acids, such as physiologically important long-chain polyunsaturated fatty acids.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 143 (1985), S. 310-314 
    ISSN: 1432-1076
    Keywords: Acrodermatitis enteropathica ; Zinc deficiency ; Essential fatty acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The fatty acid composition of different plasma lipid fractions has been estimated in a 6-month-old girl with acrodermatitis enteropathica before and after zinc supplementation. Linoleic acid and its metabolites were extremely reduced in triglycerides and sterol-esters. In contrast, n-3-fatty acids were increased in sterol-esters and phospholipids. Zinc supplementation led to quick clinical improvement, and linoleic and arachidonic acid increased rapidly in triglycerides and sterol-esters to the values of healthy infants. Fatty acids of phospholipids remained relatively stable. Our finding could be explained by impaired enteral absorption of linoleic acid. Further attention should be directed to the supply and metabolism of essential fatty acids in acrodermatitis enteropathica.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Infant feeding ; Lipid metabolism ; Essential dietary requirements ; Fatty acid desaturation ; Chain-elongation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The effect of different diets on the percentage content of long-chain polyunsaturated fatty acids (LCP; metabolites of linoleic and alpha-linolenic acids) in plasma lipids was studied in 29 premature infants on days 4 and 21 of life. Eleven infants were fed human milk which supplies LCP (1.7% of the fatty acids), 10 a commercially available milk formula without LCP, and 8 a new formula enriched with LCP of the omega-6 and the omega-3 series (0.5% LCP). LCP values in plasma lipids remained stable during the observation period in infants fed human milk. In contrast, LCP decreased markedly in plasma lipids of infants fed the conventional formula. Since the precursor fatty acids linoleic and alpha-linolenic acids were high in their diet and plasma, this finding indicates that premature infants have a limited capacity for LCP biosynthesis and may require their dietary supplementation. Infants fed the LCP enriched formula had significantly higher LCP proportions in plasma lipids than infants given the conventional formula, but less than infants fed human milk. Our results demonstrate that small concentrations of dietary LCP have marked effects on plasma lipid composition, particularly on phospholipids, suggesting that dietary LCP are preferentially channelled into structural lipids. We conclude that the essential fatty acid status of formula-fed premature infants can be improved by a supplementation of omega-6- and omega-3-LCP.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1989), S. 141-142 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 153 (1994), S. 520-525 
    ISSN: 1432-1076
    Keywords: Healthy children Essential fatty acids ; Trans fatty acids ; Plasma lipids ; Phospholipids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The fatty acid (FA) composition of plasma phospholipids, triglycerides and cholesterol esters (STE) was determined by high resolution capillary gas-chromatography in 115 apparently healthy subjects from birth to young adulthood. After birth, percentage contributions of linoleic and alpha-linolenic acid increased, whereas long-chain polyunsaturated fatty acids decreased in all FA fractions. No difference in plasma FA composition was seen between groups of children aged 1-〈5, 5-〈10 and 10-〈15 years, except for a decrease of total saturated FA in STE between 1-〈5 years (18.5%, wt/wt) and 10-〈15 years (14.5%,P〈0.005). No difference was observed for the values of boys and girls. The results on plasma lipid FA distribution in children aged 1–15 years may be used as reference values for metabolic and nutritional investigations.
    Type of Medium: Electronic Resource
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