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  • 21
    Electronic Resource
    Electronic Resource
    Fatty acid composition of plasma lipids in acrodermatitis enteropathica before and after zinc supplementation (1985)
    Springer
    European journal of pediatrics 143 (1985), S. 310-314 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00442310
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  • 22
    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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  • 23
    Electronic Resource
    Electronic Resource
    Ornithine transcarbamylase deficiency in a male: strict correlation between metabolic control and plasma arginine concentration (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 349-352 
    Details
    ISSN: 1432-1076
    Keywords: Ornithine transcarbamylase (OTC) deficiency ; Hyperammonaemia ; Plasma arginine ; Management of metabolic disorders
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a male with a partial defect of ornithine transcarbamylase (OTC) we observed that maintenance of arginine supply was crucial for adequate metabolic control in conjunction with a low protein diet. The arginine supplement had to be given such that the concentrations of arginine and ornithine in plasma were above 50μmol/l. It appears that arginine is needed not only as an essential amino acid for protein synthesis but also as a precursor of ornithine. In this patient the substitution thus aimed at increasing the intramitochondrial ornithine in order to reach a critical substrate concentration for the kinetically abnormal OTC.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00444132
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  • 24
    Electronic Resource
    Electronic Resource
    Effects of dietary long-chain polyunsaturated fatty acids on the essential fatty acid status of premature infants (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 669-675 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00441531
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  • 25
    Electronic Resource
    Electronic Resource
    3-Methylglutaconic aciduria in a patient with Pearson syndrome (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 378-379 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01956761
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  • 26
    Electronic Resource
    Electronic Resource
    Neurological manifestations of organic acid disorders (1994)
    Springer
    European journal of pediatrics 153 (1994), S. S94 
    Details
    ISSN: 1432-1076
    Keywords: Organic acid disorders ; Neurological symptoms ; Central nervous system ; Cerebrospinal fluid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neurological manifestations are very common and can be the leading and/or presenting feature in organic acid disorders, sometimes in the absence of metabolic derangement. Review of the time course and presentation of neurological disease in organic acid disorders reveals characteristic clinical findings of ataxia, myoclonus, extrapyramidal symptoms, metabolic stroke and megalencephaly. A group of organic acid disorders presents exclusively with neurological symptoms. These include glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I), succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria), mevalonic aciduria,n-acetylaspartic aciduria (Canavan disease) andl-2-hydroxyglutaric aciduria. As a group these “cerebral” orgamic acid disorders appear to remain often undiagnosed and their true incidence is much less wellknown than that of the “classical” organic acid disorders. Unfortunately, stringent guidelines for a clinical preselection of neuropaediatric patients to be investigated for organic acid disorders cannot be provided. Today, screening for neurometabolic disorders should be as comprehensive as possible and include determinations of amino acids, purines and pyrimidines and markers of peroxisomal function in addition to organic acid analysis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02138786
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  • 27
    Electronic Resource
    Electronic Resource
    Clinical symptoms, biochemical studies and therapeutic approaches in a sibship with a new congenital tubulopathy (1999)
    Springer
    European journal of pediatrics 158 (1999), S. 673-678 
    Details
    ISSN: 1432-1076
    Keywords: Key words Chloride ; Hypokalaemia ; Kinins ; Captopril ; Bartter syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on two siblings suffering from a new congenital tubulopathy. Following normal pregnancies not complicated by polyhydramnios, severe renal losses of potassium, chloride, sodium and magnesium occurred in the first weeks after birth. Calcium metabolism was not affected. The distal tubular chloride reabsorption was considerably decreased in the two siblings (0.25 and 0.28, respectively). Secondary hyperaldosteronism, activation of the kallikrein-kinin system and elevated urinary prostaglandin excretion were observed. The effects of indomethacin, spironolactone and captopril on symptoms, electrolyte wasting, activation of renin-angiotensin-aldosterone and kallikrein-kinin system and prostaglandin synthesis were studied. In spite of persisting elevation of prostaglandin synthesis, captopril decreased electrolyte wasting, polyuria and hyperaldosteronism most effectively. Conclusion We delineate an apparently new disorder characterized by a postnatal onset, an extremely decreased chloride reabsorption with extensive hyperchloriduria and hypermagnesiuria in the presence of normal calcium metabolism. The disorder can be distinguished from other tubulopathies with hypokalaemic alkalosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051174
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  • 28
    Electronic Resource
    Electronic Resource
    Sekundäre Cystathioninurie (1971)
    Springer
    European journal of pediatrics 110 (1971), S. 46-58 
    Details
    ISSN: 1432-1076
    Keywords: Secondary Cystathioninuria ; Cystathionine ; Identification ; Amino Acids ; Liver Diseases
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es werden Methoden dargestellt, mit denen eine quantitativ geringe Cystathioninausscheidung im Urin festgestellt und bewiesen werden kann. Es wurden 5 Kinder gefunden, die eine mäßige Cystathioninausscheidung aufwiesen (14,5–99,0 μMol/Tag). Die klinischen Befunde dieser Patienten werden dargestellt. Drei von ihnen hatten Leberaffektionen, eines eine nichtketotische Hyperglycinämie, und bei einem Kind handelte es sich um ein Neugeborenes mit multiplen Fehlbildungen. Bei allen Fällen lag eine sekundäre Cystathioninurie vor. Die Ursachen, die zu einer sekundären Cystathioninurie führen, werden besprochen.
    Notes: Abstract A secondary cystathionuria was found in two children with liver disease (cirrhosis of unknown etiology, congenital biliary atresia), in one infant with hypercalcemia due to high doses of vitamin D, in one case of non-ketotic hyperglycinemia, and in one infant with multiple malformations. Methods for the determination of cystathionine, when excreted in only small amounts, are outlined.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00446345
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  • 29
    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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  • 30
    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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