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  • 1
    Electronic Resource
    Electronic Resource
    High Pressure Liquid Chromatography Determination of Histamine in Fish (1987)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of food science 52 (1987), S. 0 
    Details
    ISSN: 1750-3841
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition , Process Engineering, Biotechnology, Nutrition Technology
    Notes: Histamine was determined by reversed-phase high-pressure liquid chromatography in trichloroacetic acid extracts after derivatization with o-phthaladehyde. Fluorescence was monitored at 350 nm excitation and 450 nm emission wavelength after elution with water containing 40% acetonitrile. The advantage of the described method is a rapid analysis in an automated system, where no selective extraction procedure is necessary and interfering substances are easily separated from the histamine fluorophore.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2621.1987.tb14244.x
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  • 2
    Electronic Resource
    Electronic Resource
    Evolution of right ventricular performance after CABG (1988)
    Springer
    Intensive care medicine 14 (1988), S. 499-502 
    Details
    ISSN: 1432-1238
    Keywords: Cardiac Monitoring ; RVEF ; Coronary Surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract 34 patients scheduled for coronary artery bypass graft (CABG) surgery were studied during postoperative period. Right ventricular performance was specially performed with use of cardiac output computer REF-1 Edwards Lab., before Anaesthesia (T1) and at 6 investigation times after surgery during and after mechanical ventilation. The sixth first postoperative hours were marked by a decrease of cardiac index (2.56±0.4 to 2.41±0.4 l·mn-1·m2) and right Ventricular Ejection Fraction (RVEF) (0.48±0.07 to 0.37±0.09). The second period was the weaning period with a further drop of RVEF (0.43±0.1 to 0.36±0.07) without change in cardiac index (2.80±0.5 l·mn-1·m2, suggesting a ventricular postoperative and weaning depression, as previously described for the left ventricle. In addition, postoperative tachycardia (Heart rate=59±9 at T1 to 95±14 at T7) may contribute to myocardial ischemia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00256970
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  • 3
    Electronic Resource
    Electronic Resource
    Heterogeneity of mRNA expression in Italian fucosidosis patients (1989)
    Springer
    Human genetics 〈Berlin〉 82 (1989), S. 63-66 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Genomic DNA and mRNA from six unrelated Italian patients affected with fucosidosis were analyzed using two probes (AF3 and AF11B) of partial length cDNA coding for the lysosomal enzyme alpha-l-fucosidase. DNA from patient 2, digested with EcoRI, showed a variant pattern of hybridization caused by the loss of an EcoRI site. The same patient showed a markedly decreased amount of mRNA on Northern blot hybridization. Among the remaining patients, who showed no variation at the DNA level, two apparently lacked mRNA for alpha-l-fucosidase whereas the other three showed a transcript similar in size and amount to that observed in controls. These data confirm the genetic heterogeneity of the molecular defects causing fucosidosis in Italy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00288274
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  • 4
    Electronic Resource
    Electronic Resource
    Sanfilippo type D disease: Clinical findings in two patients with a new variant of mucopolysaccharidosis III (1982)
    Springer
    European journal of pediatrics 138 (1982), S. 168-171 
    Details
    ISSN: 1432-1076
    Keywords: Mucopolysaccharidoses ; Sanfilippo D disease ; N-acetylglucosamine-6-sulfate sulfatase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A fourth genetic subtype of the Sanfilippo syndrome due to a deficiency of N-acetylglucosamine-6-sulfate sulfatase which is required for heparan sulfate degradation has recently been described. The clinical findings of two patients with a deficiency of this enzyme are reported here. Differential diagnosis from the other types of the Sanfilippo syndrome cannot be made by clinical criteria, but rests on specific enzyme assays. Since patients of either sex are known and consanguinity was present in one case, autosomal recessive inheritance is most probable.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441147
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  • 5
    Electronic Resource
    Electronic Resource
    Glycogen storage disease: recommendations for treatment (1988)
    Springer
    European journal of pediatrics 147 (1988), S. 226-228 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen storage disease ; Glucose-6-phosphatase ; Glucose-6-phosphate translocase ; Debranching enzyme ; Phosphorylase-6-kinase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A workshop was held on “Aspects of treatment of patients with glycogen storage disease” within the framework of the Concerted Action “Inborn errors of metabolism” of the European Communities. Consensus was reached on the main issues of treatment of patients with deficiency of glucose-6-phosphatase, glucose-6-phosphate translocase, debranching enzyme, liver phosphorylase and phosphorylase-b-kinase. The resulting recommendations are reported.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442683
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  • 6
    Electronic Resource
    Electronic Resource
    A mesoscale shear convective cell observed during the C.O.A.S.T. experiment: Acoustic sounder measurements (1988)
    Springer
    Boundary layer meteorology 44 (1988), S. 359-371 
    Details
    ISSN: 1573-1472
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Physics
    Notes: Abstract A mesoscale shear convective cell was observed with a Doppler sodar and a minisodar on May 22, 1983 near the Netherland Coast. Boundary-layer modifications associated with fair weather precipitating clouds were observed and a separation is undertaken between the different scales of the perturbations. There is difficulty in estimating the turbulent dissipation rate by the inertial method in the presence of mesoscale perturbations, which suggests the need for independent computation of spatial structure functions of velocity fluctuations and spectral density of velocity fluctuations as functions of frequency to observe the relationship between wave lengths and frequencies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00123022
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  • 7
    Electronic Resource
    Electronic Resource
    Mucolipidosis IV, a sialolipidosis due to ganglioside sialidase deficiency (1982)
    Springer
    Journal of inherited metabolic disease 5 (1982), S. 218-224 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A female patient of Italian, non-Jewish, descent, 22 years of age, with cloudy corneae, capsular lens opacities and severe and progressive mental and motor deterioration is described. Ultrastructural examination of a skin biopsy sample showed storage of membranous cytoplasmic bodies in Schwann cells, vessel walls, fibroblasts, smooth muscle fibres and sweat glands, and the presence of some electron-lucent vacuoles, filled with fibrillo-granular material, in sweat glands. Biochemical analysis of cultured fibroblasts (from skin explant) showed a complete deficiency of the sialidase acting on gangliosides, while the sialidase acting on sialyllactose and MU-NeuAc, and several lysomal hydrolases were normal. The urine sediment analysis showed accumulation of all phospholipid species, of several glycolipids and of gangliosides, especially of the polysialylated species. We conclude that the patient under examination is affected by Mucolipidosis IV and the term ‘sialolipidosis’ is suggested for this inborn disorder.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02179146
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  • 8
    Electronic Resource
    Electronic Resource
    Lymphoid cell lines as a model system for the study of Wolman's disease: Enzymatic, metabolic and ultrastructural investigations (1986)
    Springer
    Journal of inherited metabolic disease 9 (1986), S. 193-201 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Epstein-Barr virus (EBV) transformed lymphoid cell lines (LCL) were established from blood lymphocytes of a patient affected with Wolman's disease (WD) and from her parents. These LCL showed a severe deficiency in acid lipase activity using every substrate in comparison to LCL from normal subjects, in which acid lipase activity was similar to that in blood lymphocytes. In the LCL from Wolman's disease a major accumulation of neutral lipids was observed, mainly cholesteryl esters, CE (amount around 7 times higher than in normal cells and ratio of esterified/free cholesterol increased by 10 times) and to a lesser extent triglycerides, TG (amount increased by 1.5 times). Electron microscopy showed the storage vacuoles of neutral lipids quite characteristic of this lysosomal disease. The reported data demonstrated the validity of transformed LCL as a cellular model system in culture for experimental studies of Wolman's disease and for investigating the lysosomal metabolism of neutral lipids.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799458
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  • 9
    Electronic Resource
    Electronic Resource
    The ‘MESOGERS 84’ experiment: A report (1988)
    Springer
    Boundary layer meteorology 42 (1988), S. 251-264 
    Details
    ISSN: 1573-1472
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Physics
    Notes: Abstract During the MESOGERS 84 experiment in SW France from September 10 to October 5, 1984, different aspects of the Atmospheric Boundary Layer over complex terrain were investigated using a regional meteorological network, two instrumented aircraft and a Doppler Sodar network. The main objective has been to understand: flow properties over inhomogeneous terrain; fluxes and inversion height behaviour from a regional point of view. A description of the experiment and of the different studies undertaken is given.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00123815
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  • 10
    Electronic Resource
    Electronic Resource
    Etude de la couche limite convective sahelienne en presence de brumes seches (Expérience ECLATS) (1984)
    Springer
    Boundary layer meteorology 28 (1984), S. 51-77 
    Details
    ISSN: 1573-1472
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Physics
    Notes: Abstract The ECLATS experiment was conducted in order to investigate the influence of radiative processes on the dynamics of the atmospheric boundary layer during its diurnal evolution. This experiment was carried out over Niger, near Niamey, by measuring continuously the energy balance at ground level and by using an instrumented aircraft for turbulence, radiative fluxes and aerosol measurements in the boundary layer during dusty conditions (‘brumes sèches’). This paper is restricted to an analysis of the turbulent structure in the homogeneous and stationary convective boundary layer. The turbulence moments for kinetic energy and the spectral characteristics of the vertical velocity are discussed. These results are compared with a set of data obtained for clear convective boundary layers. The differences observed are quite important and seem, at least in part, due to radiative processes (infrared radiative divergence in the surface layer and absorption of solar radiation in the boundary layer).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00119456
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