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  • Electronic Resource  (6)
  • Essential fatty acids  (4)
  • Therapy  (2)
  • 1
    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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  • 2
    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
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 127 (1978), S. 75-89 
    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
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  • 4
    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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  • 5
    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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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 54 (1976), S. 415-422 
    ISSN: 1432-1440
    Keywords: Hyperlipoproteinemia type I ; Familial hyperlipidemia ; Diet therapy in hyperlipoproteinemias ; Lipoprotein lipase ; Medium chain triglycerides ; Essential fatty acids ; Hyperlipoproteinämie Typ I ; Familiäre Hyperlipidämie ; Diättherapie bei Hyperlipoproteinämie ; Lipoproteinlipase ; Mittelkettige Fettsäuren ; Essentielle Fettsäuren
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Der therapeutische Effekt verschiedener Diäten mit unterschiedlichen Relationen von lang- und mittelkettigem Fett, Kohlenhydraten und Eiweiß wurde bei zwei Geschwistern mit Hyperlipoproteinämie Typ I untersucht. Eine Normalisierung der TG-Werte konnte trotz extrem fettarmer Kost (〈5 g täglich) nicht erreicht werden, da es infolge der relativ kohlenhydratreichen Diät und wahrscheinlich auch wegen der Gabe von MCT zu einer starken Vermehrung der prä-β-Lipoproteine kam. Da es sich um eine lebenslange Therapie handelt, müssen die Risiken einer normal fetthaltigen Kost — vor allem rezidivierende Pankreatitiden durch die exzessiven Chylomikronenspiegel — gegen die einer fettreduzierten und kohlenhydrat-und MCT-reichen Kost — erhöhtes Atheroskleroserisiko durch Hyperpräbetalipoproteinämie — abgewogen werden. Daher erscheinen uns folgende diätetische Richtlinien sinnvoll: 1. Reduktion des langkettigen Fetts auf weniger als 30 g pro Tag, wobei besonders auf eine genügende Linolsäurezufuhr (4–6 g täglich) zu achten ist. 2. Der Kohlenhydratanteil sollte 50 Kalorienprozent nicht überschreiten und vor allem aus Stärke bestehen. 3. Das so entstehende Kaloriendefizit sollte vor allem durch Protein gedeckt werden, was durch spezielle Eiweißanreicherung einzelner Nahrungsmittel möglich ist. 4. Nur bei Schwierigkeiten bei der Einhaltung der eiweißreichen Kost sollte der Einsatz mittelkettigen Fetts erwogen werden.
    Notes: Summary The therapeutic effect of different diets varying in long chain and medium chain triglycerides, carbohydrate, and protein was tested in two siblings with type I hyperlipoproteinemia. Despite administration of an extremely fat reduced diet (〈5 g daily), a normalization of plasma TG could not be obtained because—as a consequence of its high carbohydrate and/or its MCT content—it resulted in a considerable increase in pre-β-lipoproteins. As life long dietary therapy has to be maintained, the risks of a normal fat containing diet (mainly bouts of pancreatitis) and those of a carbohydrate and MCT rich diet (premature atherosclerosis) are to be carefully considered. On the basis of our data we therefore suggest the following dietary regimen: 1. Reduced intake of long chain triglycerides (less than 30 gms per day), but with sufficient amounts of essential fatty acids (4–6 gms linoleate daily). 2. The carbohydrates should not exceed 50% of total calories and ought to consist mainly of starch. 3. The caloric deficit thus generated should be balanced by a high protein intake. This is facilitated by applying a specially protein-enriched food. 4. Medium chain triglycerides may be necessary when adherence to the protein-rich diet turns out to be bad.
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