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  • 1
    Electronic Resource
    Electronic Resource
    Co-existence of lysosomal storage diseases in a consanguineous family (2001)
    Oxford, UK : Blackwell Science Ltd
    Child 27 (2001), S. 0 
    Details
    ISSN: 1365-2214
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine , Psychology
    Notes: Lysosomal storage diseases are rare and coexistence of more than one in a family can present a diagnostic challenge as illustrated by this study. The index case born to consanguineous Asian parents presented with developmental delay. Investigations led to an incidental finding of Fabry disease. After numerous additional investigations over a year, a second diagnosis of aspartylglucosaminuria (AGU) was made. A family history of renal disease and developmental delay was disclosed. The sister and first cousin of the index case were diagnosed as homozygous for AGU, but do not have Fabry disease. The younger brother has since been diagnosed with both Fabry disease and AGU. Another cousin has learning difficulties and fits, but is heterozygous for AGU, and possibly has another uncharacterised autosomal recessive disorder. In a family with consanguinity when the clinical picture in an individual is not fully explained by the presence of one rare metabolic disease, it is essential to investigate further for the presence of others.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2214.2001.00165.x
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Modeling of the epitaxial crystallization of poly(vinylidene fluoride): T2, TGTG' and T3GT3G' chain conformations on (111) calcium fluoride (1990)
    s.l. : American Chemical Society
    Macromolecules 23 (1990), S. 680-682 
    Details
    ISSN: 1520-5835
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1021/ma00204a051
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    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    The cost-effectiveness of maintenance therapy for duodenal ulceration with an H2-antagonist (1988)
    Oxford, UK : Blackwell Publishing Ltd
    Alimentary pharmacology & therapeutics 2 (1988), S. 0 
    Details
    ISSN: 1365-2036
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Data from several sources are used to quantify the expected direct medical costs of a recently healed duodenal ulcer patient prescribed an H2-antagonist (famotidine) for a 6-month period. These costs are compared to the expected direct medical costs associated with not using maintenance therapy.Our results indicate that the estimated direct cost of patients prescribed a 6-month regimen of an H2-antagonist (famotidine) is 30.3% lower than patients who receive no H2-antagonist therapy. Most of the savings result from a reduced risk of hospitalization and surgery. The results of the sensitivity analysis of four varying scenarios indicate that H2-antagonist maintenance therapy remains less costly even when the assumptions underlying the model are varied enormously. We conclude that the decision to withhold maintenance therapy with H2-antagonists should not be based on economics.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2036.1988.tb00701.x
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Plasma lipoproteins from patients with poorly controlled diabetes mellitus and “in vitro” glycation of lipoproteins enhance the transfer rate of cholesteryl ester from HDL to apo-B-containing lipoproteins (1997)
    Springer
    Diabetologia 40 (1997), S. 1085-1093 
    Details
    ISSN: 1432-0428
    Keywords: Keywords Cholesteryl ester transfer ; CETP ; lipoprotein glycation ; diabetes mellitus ; atherogenesis.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Alterations in the reverse cholesterol transport system have been described in diabetic mellitus patients in several but not all studies. Furthermore, recently published investigations suggest that a faster “in vitro” transfer rate of cholesteryl ester from high density lipoproteins to apoB-containing lipoproteins could be solely ascribed to variation of the plasma lipoprotein composition and concentration in the diabetic state. The present study analysed the influence of lipoprotein glycation on the cholesteryl ester transfer protein-mediated transfer of esterified cholesterol from high density lipoprotein and its subfractions to lighter density lipoproteins. For this purpose two sets of “in vitro” experiments were carried out utilizing:1) plasma lipoproteins drawn from diabetic and from normal subjects and; 2) normal lipoproteins or partially purified cholesteryl ester transfer protein submitted to “in vitro” glycation. The transfer rate of 14C-cholesteryl ester labelled HDL subfractions to low or very low density lipoproteins was measured in all experiments. After incubations with plasma d 〉 1.21 g/ml or with purified cholesteryl ester transfer protein, apoB-containing lipoproteins were precipitated with a dextran sulfate/MgCl2 solution. The “in vitro” glycation of the partially purified cholesteryl ester transfer protein markedly impaired its activity. However, greater transfer rates were observed when lipoproteins from diabetic individuals or the “in vitro” glycated lipoproteins were utilized. This effect was attributed to glycation of the protein component of HDL. In conclusion, lipoprotein glycation elicits an enrichment of the apoB-containing lipoproteins with cholesteryl ester that is likely related to the premature atherosclerosis in patients with poorly controlled diabetes. [Diabetologia (1997) 40: 1085–1093]
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001250050791
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Interaction of lipid transfer protein with plasma lipoproteins and cell membranes (1990)
    Springer
    Cellular and molecular life sciences 46 (1990), S. 552-560 
    Details
    ISSN: 1420-9071
    Keywords: Lipid transfer protein ; lipoprotein metabolism ; lipoprotein remodeling ; cholesteryl ester ; triglyceride
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The hydrophobic lipid components of lipoproteins, cholesteryl ester and triglyceride, are transferred between all lipoproteins by a specific plasma glycoprotein, termed lipid transfer protein (LTP). LTP facilitates lipid transfer by an exchange process in which cholesteryl ester and triglyceride compete for transfer. Thus, LTP promotes remodeling of the lipoprotein structure, and plays an important role in the intravascular metabolism of these particles and in the lipoprotein-dependent pathways of cholesterol clearance from cells. The properties of LTP, its mechanisms of action, its roles in lipoprotein metabolism, and its modes of regulation are reviewed along with recent data that suggest a possible role for this protein in directly modifying cellular lipid composition.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01939693
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    Paper (German National Licenses)
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