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  • 1
    Electronic Resource
    Electronic Resource
    Neonatal pyruvate dehydrogenase deficiency with lipoate responsive lactic acidaemia and hyperammonaemia (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 543-547 
    Details
    ISSN: 1432-1076
    Keywords: Pyruvate dehydrogenase deficiency ; Hyperammonaemia ; Peritoneal dialysis ; Lipoic acid ; Plasma amino acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 2-day-old girl developed a severe lactic acidosis with a normal lactate/pyruvate ratio and hyperammonaemia. Plasma arginine and citrulline levels were below the limit of detection. In muscle total pyruvate dehydrogenase complex (PDHC) and pyruvate decarboxylase (E1) activities were reduced to a fraction of lower control values. The acute neonatal period was bridged with peritoneal dialysis, dichloroacetate therapy, supplements of arginine and branched chain amino acids, a complete vitamin B complex and lipoic acid. Lactate homeostasis responded to pharmacological supplements of lipoic acid. At age 1 year the child was hypotonic, showed severe developmental retardation, optic atrophy and cranial dysmorphism. She died aged 1 year 8 months with signs of respiratory paralysis but with normal lactate levels under assisted breathing. Pathological findings at autopsy were suggestive of Leigh syndrome, interstitial pneumonia and extensive fatty infiltration of hepatocytes. Regression analysis of data from 187 plasma amino acid determinations from the patient over a period of 1 year 8 months revealed a persistent imbalance involving alanine, glutamic acid, glutamine, proline, citrulline and branched chain amino acids. Aspects of acute and long-term therapy in this patient and some implications of the imbalances in plasma amino acids are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441554
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    The value of determination of homovanillic and vanillylmandelic acids in plasma for the diagnosis and follow-up of neuroblastoma in children (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 489-493 
    Details
    ISSN: 1432-1076
    Keywords: Neuroblastoma ; Catecholamine metabolites ; Homovanillic acid ; Vanillylmandelic acid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The plasma levels of homovanillic and vanillylmandelic acids were determined in 84 children with neuroblastoma prior to treatment to evaluate the usefulness of these parameters for the diagnosis of this disease. Elevated levels of one or both of these metabolites were observed in 50% of children with Stage I or II disease, in 88% of those with Stage III, in 93% of those with Stage IV and in 100% of children with Stage IV-S for a total of 88% of all patients. The clinical course of the disease can be followed readily by determination of these metabolites, successful therapy or surgery being accompanied by a corresponding decrease in the plasma levels and relapse by a corresponding increase. Determination of homovanillic and vanillylmandelic acids in plasma can obviate many of the problems associated with the collection of urine, especially in young children or ambulant patients, and thus facilitates a more frequent control with consequent advantages for the management of this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441600
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Effect of age on homovanillic and 4-hydroxy-3-methoxymandelic acid levels in plasma (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 555-557 
    Details
    ISSN: 1432-1076
    Keywords: Homovanillic acid ; 4-hydroxy-3-methoxymandelic acid ; Catecholamine acidic metabolites ; Plasma ; Mass fragmentography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The plasma levels of homovanillic and 4-hydroxy-3-methoxymandelic acids have been determined in 524 normal subjects ranging from birth to 49 years of age using a mass fragmentographic method. The mean concentration of homovanillic acid in plasma declines exponentially from the age of 1 day (x=2342.0 nmol/l, 426.6 ng/ml) to adulthood (x=60 nmol/l, 10.9 ng/ml). 4-Hydroxy-3-methoxymandelic acid behaves in a similar although not so extreme manner (392 nmol/l, 77.6 ng/ml–50 nmol/l, 10 ng/ml).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02429064
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  • 4
    Electronic Resource
    Electronic Resource
    Effectiveness of nicorandil in the preservation of myocardium stressed by transient ischemia and its influence on cardiac metabolism during coronary artery occlusion with subsequent reperfusion: a comparison with isosorbide dinitrate (1985)
    Springer
    Naunyn-Schmiedeberg's archives of pharmacology 329 (1985), S. 440-446 
    Details
    ISSN: 1432-1912
    Keywords: Cardiac metabolism ; Ischemia ; Isosorbide dinitrate ; Nicorandil ; Oxygen-debt ; Oxygen-repayment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This study was designed to investigate the effects of nicorandil in comparison to isosorbide dinitrate (ISDN) on hemodynamics, on myocardial metabolism and on effectiveness in the preservation of ischemically stressed myocardium. Repeated ischemia (3 min) was produced in anaesthetized open-chest mongrel dogs by proximal, intermittent left anterior descending artery occlusion with subsequent reperfusion. In each experiment 2–3 control occlusions were compared to 2–3 occlusions under nicorandil or ISDN. Application of both nicorandil (0.64 μmol·kg−1 body weight, i.v.) and ISDN (1.27 μmol·kg−1 body weight, i.v.) led to a significant afterload reduction and to a decrease of the coronary vascular resistance. The efficiency of the compounds in the protection of ischemic myocardium was examined by quantification of oxygen-debt and oxygen-repayment in the occlusion and reperfusion periods. Compared to control, premedication with nicorandil led to a significant increase of oxygen-debt, whereas ISDN reduced it significantly. Oxygen-repayment remained unchanged. The influence of the drugs on the metabolism of glucose, lactate and free fatty acids (FFA) was examined under basic conditions, in ischemia and during reperfusion. For all substrates, extraction, extraction ratio and oxygen extraction ratio were calculated. Under basic conditions, glucose metabolism was significantly enhanced in both groups but FFA metabolism was inhibited only by ISDN. In ischemia, FFA metabolism was enhanced by nicorandil and depressed by ISDN. Data obtained in this study suggest that nicorandil may even aggravate the metabolic and energetic situation of ischemic myocardium and, on the other hand, clearly support the well documented protective effects of ISDN.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496379
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  • 5
    Electronic Resource
    Electronic Resource
    A new method for studying the incorporation of nonesterified fatty acids into cardiac lipids by using deuterium-labelled palmitate (1988)
    Springer
    Basic research in cardiology 83 (1988), S. 87-93 
    Details
    ISSN: 1435-1803
    Keywords: nonesterifiedfatty acids ; myocardiallipids ; stable-isotope-labelledfatty acids ; cholesterol esters ; mass fragmentography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A new method for measuring the incorporation of exogenous fatty acids into myocardial lipids of working rat hearts using deuterium-labelled palmitate has been developed. After perfusing isolated hearts, the fatty acid composition of triglycerides, diglyccrides, monoglycerides, cholesterol esters, and nonesterified fatty acids (NEFA) was measured by a mass fragmentographic method. Hearts perfused only with 5 mM glucose and 1 mM lactate perfusion as basic substrates showed a significant decrease of triglyceride content, while the other lipids were not found to be significantly reduced. The fatty acid composition of all lipids were not affccted. An addition of D31-palmitate complexed to albumin at a molar ratio of 5∶1, caused a dose-dependent incorporation into triglycerides and diglycerides which suggested saturation kinetics. The tissue content of nonesterified D31-palmitate was found to be linearly related to its concentration in the perfusate. It may be of note that the concentration of the other NEFA was significantly affected neither by a fatty-acid-free perfusion nor by a perfusion with D31-palmitate. A significant incorporation of the supplied fatty acid into cholesterol esters and monoglycerides could not be detected.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01907108
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    Paper (German National Licenses)
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