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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 23 (1971), S. 69-83 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The results of the WBC of 11 298 patients of the Medizinische Poliklinik Würzburg were compiled. By statistic calculation we searched for age and sex differences in the groupings. Sex differences in the values are small, but were nevertheless significant in the statistic evaluation (male mean value 6470, female mean value 6300 leuc./mm3 blood). The WBC of the male, not of the female population rises slightly with age. The values of the blood smear showed no differences with respect to age and sex.
    Notes: Zusammenfassung Die Ergebnisse von 11 298 Untersuchungen der weißen Blutzellen bei einem unausgelesenenen poliklinischen Krankengut wurden statistisch auf Altersabhängigkeit und Geschlechtsverteilung untersucht. Die geschlechtsabhängige Differenz des durchschnittlichen Leukozytenwertes ist nach unsererer Arbeit wenig bedeutsam, war aber infolge der großen Probandenzahl statistisch hochsignifikant (Männer 6470, Frauen 6300 Leukozyten/mm3 Blut). Bei den Männern stiegen die durchschnittlichen Leukozytenzahlen mit zunehmendem Alter etwas an, jedoch nicht bei den Frauen. Beim Differenzialblutbild fanden wir keine sicheren geschlechts- und altersabhängige Differenzen.
    Type of Medium: Electronic Resource
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  • 2
    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.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Biopterin ; Dihydrobiopterin ; Inborn errors of metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An assay for the phosphate-eliminating enzyme (PEE) activity in liver was developed which required only 5–10 mg tissue. PEE catalyses the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin (BH4). In the presence of substrate, magnesium, NADPH, and a sepiapterin reductase fraction from human liver, PEE catalysed the formation of BH4 which was measured by HPLC and electrochemical detection. In adult human liver, a PEE activity of 1.02±0.134 μU/mg protein (mean ±1 SD; n=5) was observed. In liver needle biopsy material from five patients with defective biopterin biosynthesis, no PEE activity was found (less than 2% and 6% of the control values, respectively). The presence of an endogenous inhibitor was excluded. In a patient who died without definite diagnosis and in a patient with β-thalassaemia liver PEE activity was increased. Sepiapterin reductase activity was present in all cases. Results indicate that in “dihydrobiopterin synthetase” deficiency, the most frequent of the rare BH4-deficient variants of hyperphenylalaninaemia, the molecular defect consists in a defect of PEE.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 152 (1993), S. 1021-1023 
    ISSN: 1432-1076
    Keywords: Propionic acidaemia ; Methylmalonic acidaemia ; Odd-numbered fatty acids ; Propionate metabolism ; Neonates
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Newborns with disturbed propionate metabolism (propionic and methylmalonic acidaemias) accumulate during fetal life large amounts of odd-numbered longchain fatty acids in adipose tissue (8% to 10% of total fatty acids vs 1% in normal controls) and other body lipids. During periods of acute catabolism, such as in the early days of life, mobilization and oxidation of odd-chain fatty acids from adipose tissue yield extensive amounts of toxic propionyl-CoA in the mitochondria and thus might contribute to the severe illness of the patients. Our data suggest that not only catabolism of protein but also that of adipose tissue has to be avoided in order to alleviate the neonatal illness of patients with propionic and methylmalonic acidaemias. It seems prudent to avoid prolonged fasting during the 1st year of life.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Type I tyrosinaemia ; Neonatal hepatitis ; Cytomegalovirus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A severely ill 2-month-old female infant was admitted with meningitis and septicaemia caused byStreptococcus pneumoniae. The patient, who also had an acute cytomegalovirus (CMV) infection, revealed the typical clinical and biochemical characteristics of type I tyrosinaemia (TIT). Clinical evidence of severe hepatocellular damage was shown, but urinary succinylacetone was not detected. The diagnosis of TIT was finally confirmed by decreased activity of fumarylacetoacetase (FAA) in skin fibroblasts from the patient and both parents. Following dietary treatment and after overcoming the bacterial and viral infection, the patient's liver function improved.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 73-76 
    ISSN: 1432-1076
    Keywords: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency ; macrocephaly
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A girl, now three years old, is reported, in whom at the age of 5 months the diagnosis of 3-HMG-CoA lyase deficiency was established. The characteristic excretion pattern consisted of 3-HMG, 3-CH3-glutaconic acid, 3-CH3-glutaric acid and 3-HIVA. Activity of 3-HMG-CoA lyase in leucocytes was very low. She had compensated metabolic acidosis and mild hypoglycemia. Therapy with a leucine restricted diet decreased excretion of metabolites moderaterely but did not influence the tendency to metabolic acidosis. Clinically the infant presented with macrocephaly. At the age of 3 years she is severely retarded. CAT scan revealed the picture of progressive demyelination of the white matter.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Atypical phenylketonuria ; Dihydropteridine reductase deficiency ; Hyperphenylalaninemia ; Screening ; Tetrahydrobiopterin ; Therapy ; Biopterin ; Neopterin ; Sepiapterin ; Phenylalanine ; Serotonin ; Metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Administration of a single dose of tetrahydrobiopterin dihydrochloride, 10–20 mg/kg orally, to a patient with dihydrobiopterin deficiency led to disappearance of clinical symptoms for 4 days, normalization of urinary phenylalanine and serotonin and decrease of elevated neopterin for 2–3 days. A dose-dependent stimulation of serotonin production was observed. A similar effect was note with even lower doses of L-sepiapterin. The patient is now under monotherapy with tetrahydrobiopterin·2 HCl, 2.5 mg/kg daily. Other patients with this disease may not respond as well. Results of screening for tetrahydrobiopterin deficiency in 228 cases with hyperphenylalaninemia, including 140 newborns, are reported. There is evidence that biopterin biosynthesis in human kidney and liver proceeds via a dioxo compound and L-sepiapterin.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Photochemistry and Photobiology B: Biology 12 (1992), S. 311-313 
    ISSN: 1011-1344
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Photochemistry and Photobiology A: Chemistry 80 (1994), S. 161-167 
    ISSN: 1010-6030
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Photochemistry and Photobiology B: Biology 17 (1993), S. 84-86 
    ISSN: 1011-1344
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine
    Type of Medium: Electronic Resource
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