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  • 1
    ISSN: 1432-1076
    Keywords: Propionic acidaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A retrospective study was performed on the clinical outcome and long-term treatment of 17 patients with propionic acidaemia diagnosed during the last 20 years in our hospital. The study group consisted of 12 patients with early onset type of disease and 5 patients with late onset. Seven (41%) patients died, five with early onset and two with late onset. The deceased early onset patients had a median survival of 0.4 years while the deceased late onset patients died at the age of 2.8 and 4 years respectively. Median age of the living early onset patients was 5.2 (1–9.25) years, the late onset patients were 4,7 and 23 years old. Patients were all treated with natural protein restriction and in most cases carnitine and metronidazole were added. The early onset patients were almost all treated with daily home tube feeding. The mean natural protein intake of early onset patients (6.3±1.5 g/day) was significantly lower than the natural protein intake of late onset patients (17.6±5.3 g/day). Supplemental protein intake was higher in early onset patients. The general neurological outcome of our study group was satisfactory with a better outcome for early onset patients. As to growth, many patients showed a failure to thrive, this was particularly for height. The strong protein restriction during the first years of life probably contributed to this. Conclusion The prognosis for patients with propionic acidaemia appeared to be satisfactory in terms of survival and outcome characteristics such as neurological and mental development. Despite these results the authors feel that the prognosis and quality of life of these patients might be improved with liver transplantation or possibly somatic gene therapy in the future.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1041
    Keywords: haemodynamics ; isosorbide dinitrate ; sustained release ; acute myocardial infarction ; left ventricular failure ; duration of effect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Summary The aim of the study was to assess the duration of the haemodynamic effects of a new sustained-release oral formulation of isosorbide dinitrate (ISDN). Twenty patients (17 men and 3 women; mean age 60 years) with acute myocardial infarction (10 anterior, 10 inferior) complicated by moderate left ventricular failure took part in a randomized controlled trial. Ten patients were randomly assigned to the placebo group and 10 to the ISDN group, who received 40 mg sustained release isosorbide dinitrate. Haemodynamic variables were measured before treatment, after 0.5 and 1 h and then every 2 hours up to the 8th hour after treatment. There was no significant change in any haemodynamic parameter in the placebo group, during the study period. In the ISDN group there was a significant fall in pulmonary artery diastolic pressure at 4 and 8 h, from 19.0±1.0 mmHg to 16.5±1.2 mmHg and 15.5±0.8 mmHg, respectively. The mean pulmonary capillary wedge pressure fell progressively from 17.9±1.0 to 12.5±1.2 mmHg at 2 h (p〈0.001 in comparison with the placebo group. The fall remained significant up to 8 h. There was no statistically significant change in heart rate, cardiac index, systemic blood pressure or systemic and pulmonary vascular resistances. On the whole the cardiac index remained unchanged. There were numerous individual variations of cardiac index in relation to the initial mean pulmonary capillary wedge pressure and the magnitude of its fall following administration of ISDN. The change in cardiac index was inversely correlated with the control cardiac index (r=−0.69, p〈0.02).
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochemical and Biophysical Research Communications 114 (1983), S. 175-182 
    ISSN: 0006-291X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Clinica Chimica Acta 176 (1988), S. 269-277 
    ISSN: 0009-8981
    Keywords: Aminoacidopathies ; Ion-exchange chromatography ; Screening
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Clinica Chimica Acta 188 (1990), S. 101-108 
    ISSN: 0009-8981
    Keywords: Branched chain amino acid ; Chronic renal failure ; Nutrition ; Plasma amino acid
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 346 (1990), S. 799-799 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] SIR-The Loma Prieta earthquake registered 7.1 on the Richter scale. It occurred at 17:04 hours on 17 October 1989, along the San Andreas fault in the Santa Cruz mountains. Surprisingly, it destroyed about 40 houses in the Marina district of San Francisco, 67 miles away from the epicentre. Here, I ...
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1238
    Keywords: Haemodynamic ; Dobutamine ; Isoproterenol
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Dobutamine was infused at a rate of 8 mcg/kg/min in 17 patients with or without congestive heart failure. Cardiac output increased from an average 2.92 to 4.45 1/min/m2 (p〈0.001) with no change in mean aortic pressure (93.4 to 97.8 mmHg) and only a slight increase in heart rate (78 to 87 beats/min). Left ventricular end-diastolic pressure decreased from an average 19 to 13.7 mmHg (p〈0.01). Peak left ventricular dp/dt was doubled (1147 to 2370 mmHg/sec, p〈0.001) and Vmax increased from 1.08 to 2.18 circ/sec (p〈0.001). In 10 patients given equi-inotropic doses (100 per cent increase in peak dp/dt) Isoproterenol produced a greater increase in cardiac output (71 percent) than Dobutamine (51 percent). isoproterenol caused mean aortic pressure to fall significantly (8 percent) while no change was noted with Dobutamine. Accordingly, peripheral vascular resistances were reduced to a greater extent with Isoproterenol than with Dobutamine (p〈0.05). Mean pulmonary arterial pressure decreased significantly (25±5.9 to 22±5.7 mmHg, p〈0.05) with Isoproterenol infusion and remained unchanged with Dobutamine infusion. Dobutamine increased both stroke work (57 percent) and minute work (83 percent). With Isoproterenol however, only minute work was significantly increased (90 percent). Dobutamine therefore is a potent inotropic drug, with mild chronotropic and peripheral vascular effects and may be valuable in the management of severe heart failure not associated with hypotension.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Intensive care medicine 3 (1977), S. 77-80 
    ISSN: 1432-1238
    Keywords: Pulmonary embolism ; Hypoxaemia ; Circulatory failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two cases of massive pulmonary embolism, confirmed by angiographic or necropsy findings, were remarkable by the absence of arterial hypoxaemia. The various mechanisms responsible for arterial hypoxaemia in pulmonary embolism are discussed. It is suggested that in patients with massive pulmonary embolism a markedly decreased cardiac output might account for the absence of arterial hypoxaemia. In the light of these two cases the finding of a normal PaO2 does not rule out the diagnosis of pulmonary embolism.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Intensive care medicine 4 (1978), S. 29-33 
    ISSN: 1432-1238
    Keywords: Intra-aortic balloon ; Cardiogenic shock ; Myocardiac infarction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Although the shock state due to acute myocardial infarction may be reversed by IABP in 80 per cent of patients, 55 to 65 per cent remain balloon-dependent. Therefore some attempt to correct the underlyng anatomic abnormalities (reversible ischaemic areas and/or mechanical defects) appears necessary if these patients are to sruvive. With IABP catheterization studies performed in these critically-ill patients are well tolerated. Moycardial depression after cardiopulmonary by-pass is often related to subendocardial ischaemia. The combination of IABP and surgery has resulted in survival of approximately 50 per cent of patients in cardiogenic shock secondary either to a mechanical defect complicating myocardial infarction or to open-heart surgery.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Statistical inference for stochastic processes 3 (2000), S. 85-99 
    ISSN: 1572-9311
    Keywords: wavelet ; long-range dependence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mathematics
    Notes: Abstract In this contribution, the statistical properties of the wavelet estimator of the long-range dependence parameter introduced in Abry et al. (1995) are discussed for a stationary Gaussian process. This contribution complements the heuristical discussion presented in Abry et al. (1999), by taking into account the correlation between the wavelet coefficients (which is discarded in the mentioned reference) and the bias due to the short-memory component. We derive expressions for the estimator's asymptotic bias, variance and mean-squared error as functions of the scale used in the regression and some user-defined parameters. Consistency of the estimator is obtained as long as the scale index j T goes to infinity and 2j T /T→0, where T denotes the sample size. Under these and some additional conditions assumed in the paper, we also establish the asymptotic normality of this estimator.
    Type of Medium: Electronic Resource
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