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  • 1
    Electronic Resource
    Electronic Resource
    s.l. : American Chemical Society
    Journal of the American Chemical Society 100 (1978), S. 2366-2369 
    ISSN: 1520-5126
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 59 (1981), S. 343-346 
    ISSN: 1432-1440
    Keywords: Serum lipoproteins ; Diuretic treatment ; Tienylic acid ; Indapamide ; Serum-Lipoproteine ; Diuretikabehandlung ; Ticrynafen bzw. Tienilsäure ; Indapamid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In einer früheren Studie fanden wir, daß eine Kurzzeitbehandlung mit dem Diuretikum Chlorthalidon eine Erhöhung der Serum Low-Density-Lipoprotein-Cholesterin Fraktion (LDL-C) bewirkt. In der vorliegenden Arbeit wurde der Einfluß von zwei neueren Substanzen auf die Serum Lipidund Lipoprotein-Spiegel untersucht. Dabei handelte es sich um Tienilsäure, das gleichzeitig diuretische und uricosurische Wirksamkeit besitzt, sowie um Indapamid. Eine 6-wöchige Behandlung mit Tienilsäure in einer Dosis von 250 mg/Tag bewirkte bei 16 Männern eine deutliche Abnahme der Serum-Harnsäure und eine signifikante Zunahme von LDL-C und Serum-Triglyzeriden. Dagegen hatte eine gleichlange Behandlung mit Indapamid in einer Dosis von 2,5 mg/Tag bei 18 Männern keinen erkennbaren Einfluß auf Serum-Lipide oder -Lipoproteine.
    Notes: Summary Treatment with the commonly used diuretic, chlorthalidone, has previously been found to increase the serum low-density-lipoprotein cholesterol (LDL-C) fraction. Therefore, the effects of two new agents, tienilic acid (a combined diuretic-uricosuric) and indapamide on serum lipid and lipoprotein levels were assessed. Six weeks of treatment with tienilic acid, 250 mg/day, markedly decreased serum uric acid and significantly increased LDL-C and triglycerides in 16 men. In contrast, indapamide 2.5 mg/day, had no apparent influence on serum lipids or lipoproteins in 18 men.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1440
    Keywords: Serum lipoproteins ; Diuretics ; Muzolimine ; Renal disease ; Hypertension
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Patients with renal functional impairment are prone to develop hypertension and hyperlipidemia, and both abnormalities tend to occur already at an early stage of kidney disease. In 18 patients with mild renal disease (glomerular filtration rate 65±5 ml/min) and hypertension (mean blood pressure 126±4 mm Hg), the effect of six weeks of treatment with the loop-diuretic muzolimine on serum lipoproteins was assessed. Compared to placebo values, the diuretic significantly increased serum low-density lipoprotein cholesterol (LDL-C) and apoprotein B (+18 and 11%, respectively,P〈0.005) in 13 men or postmenopausal women, but not in 5 premenopausal women. Serum high-density lipoprotein cholesterol (HDL-C), and total triglycerides or lipoprotein triglyceride fractions were not consistently changed in both subgroups. Thus, the ratio LDL-C/HDL-C was increased from 3.2±0.3 to 3.9±0.3 (P〈0.05) in the men or postmenopausal women, while no such tendency occurred in the premenopausal women (4.1±0.6 to 3.7±0.6). Changes in serum LDL-C were not associated with hemoconcentration or alterations in carbohydrate metabolism and were not related to variations in serum potassium or blood pressure. Increased serum levels of the atherogenic LDL-C fraction during diuretic treatement in men or postmenopausal women with renal disease may represent a potentially undesirable effect, particularly since such patients may tend to have hyperlipidemia in the untreated state.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1440
    Keywords: Orale Glukose-Belastung ; Insulin ; Kalium ; Renin ; Aldosteron ; Oral glucose-load ; Insulin ; Potassium ; Renin ; Aldosterone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The effects of a standard oral glucose load (100 g) on plasma glucose, insulin, potassium, renin and aldosterone levels were investigated in 10 supine normal subjects (Group A). Responses of plasma glucose, insulin, potassium and aldosterone to glucose ingestion were evaluated further in 16 seated normal or borderline hypertensive subjects (Group B), studied in the untreated state as well as following renin-aldosterone activation by diuretic pre-treatment. In both groups, the increase in plasma glucose and insulin following glucose ingestion was accompanied by an acute decrease (p〈0.01) in plasma potassium and aldosterone levels, which in Group A was associated with an increase (p〈0.02) in plasma renin activity. In all subjects analyzed together, significant (p〈0.005) correlations were noted between plasma aldosterone and potassium levels and between glucose-induced changes in these factors. In Group A, there were significant (p〈0.001) correlations between glucose-induced changes in plasma aldosterone and renin values and between absolute aldosterone and renin levels in the glucose-loaded state. Plasma aldosterone or renin levels following glucose-load were unrelated to glucose or insulin values. These findings indicate that an oral standard glucose load causes acutely marked aldosterone suppression and mild but distinct renin stimulation. The glucose-induced inhibition of aldosterone secretion appears to depend on insulin-mediated changes in potassium metabolism and may be partly counteracted by concomitant renin activation.
    Notes: Zusammenfassung Der Einfluß einer oralen Standard-Glukosebelastung (100 g) auf die Plasma-Konzentrationen von Glukose, Insulin, Kalium, Renin und Aldosteron wurde bei 10 liegenden Normalpersonen untersucht (Gruppe A). Das Verhalten der Plasma-Konzentrationen von Glukose, Insulin, Kalium und Aldosteron nach einer solchen Glukose-Einnahme wurde zudem bei 16 sitzenden, normalen oder grenzwertig-hypertensiven Personen studiert (Gruppe B), wobei die Untersuchungen im unbehandelten Zustand sowie nach Aktivierung des Renin-Aldosteron-Systems durch Vorbehandlung mit Diuretika erfolgten. In beiden Gruppen wurde der Anstieg der Plasma-Glukose und -Insulinwerte nach Glukose-einnahme von einer akuten Senkung (p〈0,01) der Plasma-Kalium und -Aldosteron-Konzentrationen begleitet. In Gruppe A fand sich gleichzeitig ein signifikanter Anstieg (p〈0,02) der Plasma-Renin-Aktivität. Die gemeinsame Analyse aller Personen ergab signifikante (p〈0,005) Korrelationen zwischen den basalen (prä-Glukose-Infusion) Absolutwerten von Plasma-Aldosteron und -Kalium einerseits und zwischen den Glukose-induzierten Änderungen dieser beiden Parameter andererseits. In Gruppe A fanden sich zudem nach der Glukose-Einnahme signifikante Beziehungen zwischen den absoluten Plasma-Aldosteron und-Reninwerten, sowie zwischen den Änderungen dieser beiden Parameter (p〈0,001). Dagegen korrelierten Plasma-Aldosteron- oder -Reninspiegel nicht signifikant mit den Glukose- oder Insulinwerten. Diese Befunde zeigen, daß eine orale Standard-Zufuhr von Glukose akut eine markante Aldosteron-Hemmung und eine leichte, aber signifikante Renin-Stimulation bewirkt. Die Glukose-induzierte Aldosteron-Suppression scheint mit den Insulin-vermittelten Änderungen des Kaliummetabolismus in Zusammenhang zu stehen und durch die Renin-Aktivierung teilweise antagonisiert zu werden.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1990), S. 634-636 
    ISSN: 1432-1076
    Keywords: Hyperammonaemia ; N-acetylglutamate synthetase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 13-month-old female presented with neurological deterioration of 1 month duration and hyperammonaemia. N-acetylglutamate synthetase activity in the liver was reduced to 33% of the control. A male cousin and a female sister had died following a similar clinical course. This is the first report of late-onset N-acetylglutamate synthetase deficiency. An autosomal-recessive mode of inheritance is suggested.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 153 (1994), S. S23 
    ISSN: 1432-1076
    Keywords: Organic acid analysis ; Aminoacid analysis ; Short chain fatty acids ; 3-Hydroxypropionate Propionic acidaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pitfalls in organic acid analysis can originate from inadequate methodology, analytical interferences, in vivo interactions and from pre-analytical conditions which often are unknown to the specialized analytical laboratory. Among the latter, ingested food and additives, metabolites of food processing or medications have to be considered. Bacterial metabolites from the gastrointestinal or urogenital system or formed after sample collection can lead to pitfalls as well. An example of such a patient whose urinary metabolites mimick at first glance inherited propionic aciduria is described.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Screening ; Neonatal mass screening ; Metabolic disorders ; Hypothyroidism ; Hyperphenylalaninaemia ; Galactosaemia ; Maple syrup urine disease ; Adrenogenital syndrome ; Cystic fibrosis ; Duchenne's muscular dystrophy ; Histidinaemia ; Hypermethioninaemia ; Tyrosinaemia ; Haemoglobinopathies ; Glucose-6-phosphate dehydrogenase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The present situation of neonatal mass screening for metabolic disorders in eleven European countries is presented. The only disease screened for on a population wide basis in almost all countries is phenylketonuria. Screening for congenital hypothyroidism has been started in most countries or is under active consideration. A priority list of disorders that should be screened for routinely in all newborns comprises congenital hypothyroidism, hyperphenylalaninaemia, galactosaemia and maple syrup urine disease. Other disorders, like adrenogenital syndrome, cystic fibrosis. Duchenne's muscular dystrophy, histidinaemia, or tyrosinaemia cannot be recommended for mass screening at present because of an unsatisfactory test procedure or lack of effective treatment.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 134 (1980), S. 109-113 
    ISSN: 1432-1076
    Keywords: Orotic acid ; Urea cycle ; Metabolism, inborn errors ; Hyperammonemia ; Organic aciduria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Orotic acid excretion in urine is increased in ornithine transcarbamylase deficiency, citrullinemia and argininemia; it is barely increased in argininosuccinic aciduria and normal in carbamylphosphate synthetase deficiency and in hyperammonemia due to organic aciduria. The determination of orotic acid excretion is useful in differentiating the causes of hyperammonemia and reduces the need for enzymatic assays on tissue biopsies for decisions on therapy. The data indicate that orotic acid does not merely reflect ammonia concentration in plasma, but depends on carbamylphosphate concentration. Arginine could play a key role in the regulation of ammonia detoxication.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 139 (1982), S. 72-75 
    ISSN: 1432-1076
    Keywords: Carbamyl phosphate synthetase ; Hyperammonaemia ; Newborn
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A neonate with pronounced hyperammonaemia died at the age of 6 days. Absence of liver carbamyl phosphate synthetase activity was demonstrated. This is the eighth reported patient with a severe variant of isolated mitochondrial carbamyl phosphate synthetase deficiency.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 144 (1985), S. 203-204 
    ISSN: 1432-1076
    Keywords: Fructose 1,6-diphosphatase deficiency ; Glycerol excretion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A Turkish boy had suffered since the age of 10 months from recurrent attacks of severe metabolic acidosis and hypoglycaemia precipitated by moderate respiratory tract infections. A liver biopsy showed lack of fructose 1,6-diphosphatase and absence of phosphorylase. The patient died in shock following fructose ingestion. Upon fasting, acidosis with increased lactate and glycerol excretion was found. Findings indicate that, in this inherited disorder of gluconeogenesis, lactic acidosis combined with increased glycerol excretion upon fasting are of diagnostic importance.
    Type of Medium: Electronic Resource
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