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  • sol-gel  (4)
  • D600  (3)
  • Electron microscopy  (3)
  • Key words Phenylketonuria  (3)
  • 1
    ISSN: 1432-1440
    Keywords: Whipple's disease ; Electron microscopy ; Disturbed cellular immunology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Whipple's disease is a curious disorder with the involvement of many organ systems, primarily gut, synovium and the central nervous system, characterized by the presence of numerous proliferating bacteria in tissue macrophages and other cell types. While clinically this disease entity has previously been defined by the classical triad of diarrhoea, malabsorption and weight loss, some patients do not show these features. In this report, a clinically unusual case of Whipple's disease is described presenting with high persistent fever, severe arthralgias and headaches, but without malabsorption, diarrhoea or weight loss. Nevertheless, the histological and electron microscopical pictures demonstrated the typical findings of intracellular micro-organisms along with the presence of bacteria in Schwann nerve cells, which has only once been described previously. Immunological findings before treatment demonstrated a decrease of T cells with the helper/inducer phenotype, and a concomitant rise in cells with the suppressor/cytotoxic phenotype, an elevation of “activated” Ia positive T cells and a significant reduction of T cell mitogenic responsiveness. Of special interest, after a successful treatment these immunological abnormalities shifted to normal with the exception of a still elevated number of Ia+ T cells. The discussion of this unusual case of Whipple's disease includes — besides possible cellular immunological abnormalities — genetic factors, especially since this patient was HLA-B-27-positive as was his son who is suffering from ankylosing spondylitis.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pflügers Archiv 410 (1987), S. 75-82 
    ISSN: 1432-2013
    Keywords: Twitch fibre ; K-contracture ; Inactivation ; D600
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract (1) Single twitch fibres were dissected from tibialis muscles ofRana temporaria and used to study the effect of D600 (gallopamil) on potassium-induced contractures. (2) 95 mM K-Ringer's was applied for 8–15 s at intervals of generally 2.5–5 min; at temperatures of 6–8°C and in the absence of D600 the amplitude of the contractures remained fairly constant. After pretreatment with D600 (30 μM) a single (conditioning) K-contracture was sufficient to ‘paralyze’ the fibres (cf. Eisenberg et al. 1983). (3) Complete paralysis could also be achieved at 18–20°C. In three fibres a single conditioning K-application was sufficient; in two more fibres two or three conditioning K-applications were required. (4) D600-paralysis could not only be achieved with high K-concentrations but also by conditioning with subor suprathreshold K-concentrations (20–40 mM); the duration of the conditioning periods required to induce complete paralysis was approximately the same before and after D600-treatment. (5) Contractures were partially abolished by application of 20–40 mM K-Ringer's for short conditioning periods; after D600-treatment the degree of contracture loss was similar. (6) At low temperature the state of partial or complete paralysis induced by subthreshold K-concentrations and D600 was maintained for long periods of time. (7) The presence of 10 mM Ca2+ did not protect the fibres from being paralyzed by treatment with D600 and high K-Ringer's at low temperature; however, more than one conditioning K-application was required. (8) Resting and action potentials of paralyzed fibres were not significantly different from control values. However, endplate potentials were reduced in size, and failure of neuromuscular transmission was observed in some of these fibres. (9) It is concluded that D600 prolongs the state of inactivation of the contractile mechanism, while the development of inactivation does not seem to be markedly affected.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pflügers Archiv 412 (1988), S. 390-396 
    ISSN: 1432-2013
    Keywords: Slow fibre ; K-Contracture ; D600 ; Inactivation ; Repriming
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract (1) The effect of 30 μM D600 on the amplitude and time course of isometric contractures was studied in single slow fibres ofRana temporaria. (2) D600 only slightly reduced the amplitude of contractures evoked with 30 or 95 mM K-Ringer's. Maintenance of tension was strongly impaired by D600 only during exposure to 95 mM K. The caffeine contracture was not affected. (3) Addition of 10 mM Ca2+ or other divalent cations to the medium strongly counteracted the effect of D600 on maintained tension. The order of efficiency was Ca2+=Ni2+〉Co2+〉Mn2+〉Mg2+. (4) During 2 min exposure to 95 mM K-Ringer's the slow fibres inactivated to a variable degree; recovery from inactivation in normal Ringer's proceeded with a half time of the order of 1 min, while in the presence of D600 recovery was prolonged 3.3 to 27 times. (5) It is concluded that the effect of D600 on the contractile behaviour of slow fibres fromRana temporaria is predominantly due to a prolongation of the inactivated state. It is suggested that D600 binds to a site at the outer membrane surface which also binds divalent cations and determines the degree of contractile inactivation during exposure to potassium. Blocking of Ca2+ channels is unlikely to be the mechanism of this D600-effect.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Der Pathologe 16 (1995), S. 404-407 
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Nerval-neuroendokriner Komplex ; Zollinger-Ellison-Syndrom ; Magenschleimhaut ; Elektronenmikroskopie ; Key words Nerve fibre-neuroendocrine cell complex ; Stomach ; Zollinger-Ellison-Syndrom ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Nerve fibre-neuroendocrine cell complexes (NF-NEC-C's) are neuroendocrine cells located in the lamina propria of the gastrointestinal tract directly connected with nerve fibres of Meissner's plexus. We report on a patient with sporadic Zollinger-Ellison syndrome (ZES) with electron microscopically demonstrated multiple NF-NEC-C's in non-antral gastric mucosa. It is suspected that in ZES the hypergastrinaemia may represent a trophic stimulus for the proliferation of NF-NEC-C's in the gastric mucosa.
    Notes: Zusammenfassung Unter nerval-neuroendokrinen Komplexen (N-NE-K) versteht man in der Lamina propria mucosae des Magen-Darm-Traktes gelegene Nester neuroendokriner Zellen, die in direktem Kontakt zu Nervenfasern des Meissner-Plexus stehen. Es wird über einen Patienten mit sporadischem Zollinger-Ellison-Syndrom (ZES) berichtet, bei dem sich elektronenmikroskopisch in nonantraler Magenschleimhaut multiple N-NE-K fanden. Möglicherweise stellt die Hypergastrinämie bei ZES einen trophischen Stimulus für die Proliferation von N-NE-K in der Magenschleimhaut dar.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Phenylalanine ; Magnetic resonance ; imaging ; Evoked potentials ; Myelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract White matter abnormalities on MRI have been observed in phenylketonuria (PKU) patients with late onset neurological symptoms as well as in neurologically inconspicious patients. We investigated 14 early treated adolescents at an age between 12 and 17 years (mean age 14.3 years) with classical PKU as well as one retarded patient with atypical PKU by cranial MRI with spinecho T1-, T2- and proton density sequences. Clinical examination was normal. Visual evoked potential (VEP) examination showed a prolonged latency of peak P100 (mean 122.6 ms; control mean 115.9) and IQ testing showed a mean IQ of 101.1. To investigate the influence of plasma phenylalanine (Phe) levels three approaches were used: Phe was determined for the day of MRI, for a period of 6 months prior to MRI and for lifetime up to 12 years. MRI scans revealed areas of abnormally increased signal intensity on T2-weighted and proton density images in 12 (86%) patients, preferably involving the parieto-occipital lobes. MRI of the patient with atypical PKU was normal. MRI findings correlated most strongly to long-term dietary control up to 12 years. We found no correlation with the other parameters of biochemical control, IQ or VEP latency. The nature and prognosis of MRI abnormalities in neurologically normal PKU patients remain unclear although abnormalities in VEPs which were not associated with the degree of MRI abnormalities in our sample indicate a disturbance in myelination along the visual pathways.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Genotype ; Treatment ; IQ
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The interdependence of the predicted in vitro residual enzyme activity (PRA), as deduced from the complete genotypes of 64 hyperphenylalaninaemic patients, and parameters for diagnosis of hyperphenylalaninaemic disorders, the fluctuation of the phyenlylalanine (Phe) values during treatment, long-term dietary control during treatment, and a parameter for the outcome of therapy (IQ) was investigated by correlation analysis. A highly significant correlation was found between the PRA and diagnostic parameters, as well as the fluctuation of the Phe values during treatment. Significant correlations were also observed between the parameter describing the fluctuation of the Phe values and the IQ, as well as between the quality of dietary control and IQ. The PRA is a valuable tool for the differential diagnosis of hyperphenylalaninaemic disorders and for the prediction of one aspect of the course of the disease which is related to the intellectual outcome of therapy. The quality of dietary control was independent of the genotype, indicating that the outcome of therapy can be successfully manipulated in spite of the genetic make-up.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. S97 
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Intelligence ; Education ; Professional career ; Phenylalanine ; control
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The intellectual status and professional careers of 51 young adults with phenylketonuria whose treatment started before 3 months of age are described. Their mean IQ was 97 (SD = 16). Of the IQs, 4% were more than 2 SD below the norm. The distribution of types of schooling of the patients was comparable to that in the German population. The professional careers of nearly all the patients were according to their educational level. Within the sample the outcome was significantly correlated with phenylalanine (Phe) control, even when the patients’ social background was statistically taken into account. The main influence of Phe on intelligence seems to occur during the first decade of life since IQ data remain stable even after Phe levels increased during adolescence.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Journal of cancer research and clinical oncology 105 (1983), S. 285-291 
    ISSN: 1432-1335
    Keywords: Borderline lesion ; Severe dysplasia ; Gastric precancerosis ; Electron microscopy ; Morphometric analysis ; Bleb formation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The “borderline lesion” of the stomach (severe dysplasia of the protruded type) is nowadays considered to be a true precancerous lesion. Histologically, this lesion is characterized by a proliferation in the area of the glandular neck, combined with severe cellular atypia. Investigation by means of transmission electron microscopy reveals the structure of these cells to be very similar to the structure of cells of an intestinal-cell-type carcinoma of the stomach. In addition to signs for a loss of differentiation of the cells, such as the absence of rough-surfaced endoplasmatic reticulum, mucin granules, and of the polarity of the cells, remarkably frequently luminal bulges and “blebs” developed to various extents can be detected. Morphometric analysis is delineating the tendency of structural features of the borderline lesion to develop toward the gastric cancer cell. These irregularities might be expressions of the beginning of expansive growth and signs pointing to differentiation into malignant neoplasia.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of sol gel science and technology 8 (1997), S. 927-929 
    ISSN: 1573-4846
    Keywords: organic-inorganic hybrids ; sol-gel ; photochromic performance ; spirooxazine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract Organic-inorganic hybrid materials synthesized via sol-gel processing are excellent solid matrices for photochromic dyes like spirooxazine, and the photochromic performance can be further enhanced by introducing suitable additives. This work describes the effects of additives on the photochromic intensity (Δ A 0), decolouration rate (k) and photostability of spirooxazine in sol-gel derived organic-inorganic hybrid matrices. They include fluoro-alkylsilane (FAS), bisphenol A (BPA) and methyl-imidazole (MI). FAS enhances both Δ A 0 and photostability, but has little effect on k. The higher the content of BPA, the higher the Δ A 0 and the higher the photostability, but the lower the k. The effect of MI on Δ A 0 and k is not so considerable as that of BPA and is a little complicated, while significant improvement in photostability is achieved by the addition of MI at an optimum concentration with the presence of BPA at a higher content.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of sol gel science and technology 8 (1997), S. 927-929 
    ISSN: 1573-4846
    Keywords: organic-inorganic hybrids ; sol-gel ; photochromic performance ; spirooxazine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract Organic-inorganic hybrid materials synthesized via sol-gel processing are excellent solid matrices for photochromic dyes like spirooxazine, and the photochromic performance can be further enhanced by introducing suitable additives. This work describes the effects of additives on the photochromic intensity (ΔA 0), decolouration rate (k) and photostability of spirooxazine in sol-gel derived organic-inorganic hybrid matrices. They include fluoroalkylsilane (FAS), bisphenolA (BPA) and methyl-imidazole (MI). FAS enhances both ΔA 0 and photostability, but has little effect onk. The higher the content of BPA, the higher the ΔA 0 and the higher the photostability, but the lower thek. The effect of MI on ΔA 0 andk is not so considerable as that of BPA and is a little complicated, while significant improvement in photostability is achieved by the addition of MI at an optimum concentration with the presence of BPA at a higher content.
    Type of Medium: Electronic Resource
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