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  • 1
    ISSN: 1432-0533
    Keywords: Mitochondrial disease ; Myoclonus epilepsy and ragged red fibers (MERRF) syndrome ; Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome ; Polarographic studies ; Immunoblot studies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Mitochondrial respiratory chain function was investigated with polarographic and enzymatic studies, and correlated with immunoblot studies using a battery of probes against respiratory chain holocomplexes in a series of patients with myoclonus epilepsy and ragged red fibers (MERRF) syndrome. State III respiration rates in intact skeletal muscle mitochondria were normal in two cases, suggested site I deficiency in one case and a midrespiratory defect in another. Immunological studies of complex I showed reduced levels of several subunits with the apparent absence of two bands (which at 45 and 42 kDa, coincide with the predicted electrophoretic mobility of the ND5 gene product) in one case. Complex I, III and IV composition was normal in the other three cases indicating no major disruption of complex assembly. A differing severity of skeletal muscle respiratory chain impairment in a group of unrelated patients with severe cerebral clinical involvement is best explained by uneven tissue distribution between brain and muscle of a heteroplasmic mtDNA mutation. The relationship between MERRF and mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) encephalopathies is reappraised by extension of this hypothesis.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    International journal of immunogenetics 21 (1994), S. 0 
    ISSN: 1744-313X
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology , Medicine
    Notes: Selective IgM deficiency is found commonly in patients with Bloom's syndrome (BS). Serum IgM concentrations were low though serum IgG and IgA concentrations were normal in both patients with BS included in the study. In a previous study the authors showed that selective IgM deficiency in BS is due to an abnormality in the maturation of surface IgM-bearing cells into IgM-secreting cells and a failure of secreted μ (μ s) mRNA synthesis. The membrane-bound μ (μ m) and μ s mRNA are produced from transcripts of a single immunoglobulin μ gene by alternative RNA processing pathways. The control of μ s mRN A synthesis depends on the addition of poly(A) to μ s C-terminal segment. The study described here demonstrated that there was no mutation or deletion in the sequence including μ s C-terminal coding sequence, the RNA splice site (GG/TAAAC) at the 5’ end of μ s C-terminal segment, and the AATAAA poly(A) signal sequence, and second GT-rich element immediately down-stream of the cleavage site in both patients.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Clinical & experimental allergy 24 (1994), S. 0 
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: This study was designed to analyse prostaglandins (PGs) in human nasal lavage- fluid using the combination of microcolumn high performance liquid chromatography and a He/Cd laser induced fluorescence detection system. Forty-seven patients with allergic rhinitis and 12 healthy volunteers were investigated. Four species of PG. i.e. PGD2, PGE2, PGF2x and 6-keto-PGF1, were detected in the nasal lavage fluid. Concentrations of PGD2; (1.33 ± 0.17 nmol/ml) and PGE2 (0.87 ± 0.11 nmol/ml) in nasal lavage fluid from patients with allergic rhinitis (the allergy group) were significantly increased compared with those of volunteers (the control group, 0.23 ± 0.16 nmol/ml, 0.29 ±0.19 nmol/ml, respectively). On the other hand, no significant differences were observed in concentrations of cither PGF2x, or 6-keto-PGF1x between the control group and the allergy group. Histamine concentration in nasal lavage fluid was significantly increased in the allergy group (53 ± 7.6 nmol/l) compared with the control group (3.4 ± 1.0 nmol/1). No significant correlation was observed between PGD2 and histamine concentration (r= 0.24), or between PGE2 and histamine concentration (r= 0.08) in nasal lavage fluid from patients with allergic rhinitis. Treatment with oxatomide, an anti-histamine and anti-allergic drug, significantly improved symptom scores, but did not alleviate them completely. Concentrations of each PG detected in nasal lavage fluid did not change significantly after oxatomide treatment. It is concluded that, not only histamine but also PGs, particularly PGD2 and PGE2, might be involved in the genesis of allergic rhinitis.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Five patients with atopic dermatitis (AD) who were sensitive to hen's egg were observed before and after natural measles virus infection. Within 4 weeks of natural measles virus infection, the eczematous lesions clearly improved in four of the five patients in whom neither offending foods were eliminated, nor anti-allergic drugs, systemic steroids and steroid ointment administered. This was accompanied by reduced proliferative responses of peripheral blood mononuclear cells (PBMCs) to ovalbumin (OA). Another patient showed a transient improvement of AD symptoms, from severe to mild, and thereafter returned to severe accompanied by increased proliferative responses of PBMCs to OA. Radioallergosorbent test (RAST) scores for hen's egg in all five patients did not change in each level in each patient, except the transiently decreased RAST scores for hen's egg in one patient, after the infection. Thus, in patients with AD who are sensitive to food, the improvement of AD symptoms that appeared within 4 weeks of natural measles virus infection was related to reduced proliferative responses of PBMCs to the food antigen following the infection.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 38 (1993), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In most patients with Bloom's syndrome (BS), selective IgM deficiency is commonly found. We examined proliferative responses by incorporation of [3H]-thymidine and the production of immunoglobulin after ultraviolet B (UVB) irradiation in the cells of two patients with BS. With regard to the proliferative responses in peripheral blood mononuclear cells (PBMC) cultured with pokeweed mitogen (PWM), the patients' PBMC were more sensitive to UVB irradiation than controls. Although the effect of UVB irradiation in lymphoblastoid cell lines (LCL) after 0 days of culture showed no difference between one patient and controls, the patient's LCL were more sensitive to UVB than the controls after 3 and 7 days of culture. These results suggest that the proliferative responses of the patient's LCL recovered later than those of controls. IgM production was the most sensitive to UVB in the patients' PBMC and LCL. IgG and IgA production in the patients' PBMC and LCL showed the same sensitivity as controls. From our results, it is suspected that the preferential damage to IgM production by UVB is connected with the selective IgM deficiency of BS.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 36 (1992), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report a case of common variable immunodeficiency (CVI) that shows low levels of IgG and IgA, but a normal quantitative or qualitalive level of IgM. T-cell functions were not disturbed. Increased numbers of surface IgM (sIgM) and sIgD, sIgM and sIgA, sIgM and sIgA double bearing B cells were observed as compared with a control. No IgG and IgA induction upon stimulation with Staphylococcus aureus Cowan I (SAC)and recombinant interleukin-2(rIL-2), or pokeweed mitogen (PWM) and rIL-4 or rIL-6 was observed, although there was proliferation. Although; μ mRNA was expressed as much as in a healthy control, transcription of γ mRNA and α mRNA was very low. Furthermore, no enhanced effects of γ mRNA and α mRNA were recognized upon stimulation with rIL-4 and rIL-6. These results suggest that the patient's B cells might be detective at the switching process from μ, μ and δ, μ and γ to γ or μ and α to α.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 32 (1990), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Five heterogeneous IgA-immunodeficient patients were analysed for expression of the α-chain gene. The number of surface IgA-bearing B cells was low in four patients Southern blot analysis indicated no deletion of immunoglobulin structural genes coding for Cα or α switching-region genes. The number of surface IgM and IgA double-bearing B cells increased in some patients. Addition of recombinant tnterieukin 4 (rIL-4). rIL.-5. and rIL-6 to the normal B cells enhanced IgA production. However. B cells of the patients showed no or one-third lower IgA production in response to these lymphokines, even though there was proliferation ill 4, rIL -5, and rIL-6 induced low or no expression of α mRNA of the patients B cells These results suggested that the patients lacked B cells able to produce transcripts for the IgA heavy chain, and that some patients’B cells might be defective at the switch-recombination process from u to a or from μ to α or from μ and α to α.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1573-689X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A new simulation system of diagnostic and therapeutic processes is developed. The aim is to train medical students for the practical use of their knowledge, utilizing patient data in a total hospital information system. The knowledge in the system is presented by the specialists for every case. In medical school there are many specialists in various fields. With their cooperation the system can grow up to a comprehensive CAI system for clinical education. The system is designed to work on the mainframe for easiness of development, maintenance and extensions of the system. The present framework has been applied to the simulation of diagnostic process. The usefulness of the present system has been confirmed by specialists and students.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 71 (1993), S. S112 
    ISSN: 1432-1440
    Keywords: Cholesterol ; Lipoprotein ; Ubiquinone ; Antioxidant ; Coronary heart disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary It has been postulated that oxidatively modified low-density lipoprotein (LDL) contributes to the genesis of atherosclerosis. Ubiquinone has been suggested to be an important physiological lipid-soluble antioxidant and is found in LDL fractions in the blood. We measured plasma level of ubiquinone using high-performance liquid chromatography and plasma levels of total cholesterol, high-density lipoprotein (HDL) cholesterol, and triglycerides in 245 normal subjects (186 males, 59 females) and in 104 patients (55 males, 49 females) who had coronary artery disease not receiving pravastatin and 29 patients (12 males, 17 females) receiving pravastatin. In the normal subjects, the plasma ubiquinone levels did not vary with age. In the patient groups, the plasma total cholesterol and LDL levels were higher and the plasma ubiquinone level lower than in the normal subject group. The LDL/ubiquinone ratio was higher in the patient groups. We found that ubiquinone level, either alone or when expressed in relation to LDL levels, was significantly lower in the patient groups compared with the normal subject group. The 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase inhibitor is thought to prevent atherosclerosis, however, it also inhibits ubiquinone production. The present study revealed that HMG CoA reductase inhibitor decreased plasma cholesterol level, and that it did not improve either the ubiquinone level or the LDL/ubiquinone ratio. From these results, the LDL/ubiquinone ratio is likely to be a risk factor for atherogenesis, and administration of ubiquinone to patients at risk might be needed.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Communications in mathematical physics 151 (1993), S. 619-645 
    ISSN: 1432-0916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mathematics , Physics
    Notes: Abstract We consider the scattering problem for the non-linear Schrödinger (NLS) equation with a power interaction with critical powerp=1+2/n in space dimensionsn=2 and 3 and for the Hartree equation with potential |x|−1 in space dimensionn≥2. We prove the existence of modified wave operators in theL 2 sense on a dense set of small and sufficiently regular asymptotic states.
    Type of Medium: Electronic Resource
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