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  • 11
    ISSN: 1365-2133
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Ichthyosis bullosa of Siemens (IBS, MIM 146800) is a unique congenital ichthyosis characterized by mild epidermal hyperkeratosis over flexural areas, blister formation and the development of superficially denuded areas of hyperkeratotic skin. It is clinically difficult to distinguish severe IBS from mild bullous congenital ichthyosiform erythroderma (BCIE, MIM 113800). In the current literature, 19 IBS families with keratin 2e (K2e) mutations have been reported, despite only five IBS families having been reported before the first identification of K2e mutation in 1994. We studied four patients from three Japanese IBS families. They had previously been misdiagnosed as having BCIE before the correct diagnosis was made after mutation detection. To detect the pathogenic mutations, we performed direct sequencing of the entire coding regions of KRT2E encoding K2e in the patients and healthy family members. K2e mutations, a 1469T→C transition (L490P) and a 1477G→A transition (E493K) within the conserved 2B helix termination motif of the rod domain were detected in the families and the definite diagnosis of IBS was made in the four cases. The present results indicate that IBS is not such a rare entity as was previously thought, and accurate diagnosis is now available by mutation analysis.
    Materialart: Digitale Medien
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  • 12
    ISSN: 1365-2133
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 13
    Digitale Medien
    Digitale Medien
    Springer
    Optical and quantum electronics 12 (1980), S. 159-167 
    ISSN: 1572-817X
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Elektrotechnik, Elektronik, Nachrichtentechnik , Physik
    Notizen: Abstract A high repetition, mobile laser radar system with a computerized real-time data acquisition and display system is described. Signal-to-noise levels of the system are compared with theoretical values, and it is found that they are affected by the shot noise in the system. An example of a stack plume observation is also presented to show the performance of the system.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 14
    Digitale Medien
    Digitale Medien
    Springer
    Journal of statistical physics 26 (1981), S. 719-743 
    ISSN: 1572-9613
    Schlagwort(e): Self-synchronization ; Van der Pol oscillator ; fluctuations ; phase transition ; rhythm
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Physik
    Notizen: Abstract A statistical mechanical theory is presented for the self-organization of a macroscopic oscillation with the presence of external fluctuations in a system of Van der Pol oscillators coupled through dissipative interactions. Starting from Langevin equations for the Van der Pol oscillators, the static and dynamic characteristics are studied. The threshold condition is given by the relative size between the fluctuation and the interaction. The transitions between synchronous and asynchronous phases are well discussed by a Landau-type equation. The steady state value of the order parameter and the onset time are compared between the theory and the computer experiments and a good agreement is obtained.
    Materialart: Digitale Medien
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  • 15
    Digitale Medien
    Digitale Medien
    Springer
    Archives of dermatological research 270 (1981), S. 413-419 
    ISSN: 1432-069X
    Schlagwort(e): Epidermal vacuolation ; Local anesthetics ; Intradermal (i.d.) injection ; Subcutaneous (s.c.) injection ; Epidermale Vacuolisierung ; Lokalanaestheticum ; intradermale (i.d.) Injektion ; subcutane (s.c.) Injektion
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Zusammenfassung Um den Mechanismus der Vacuolisierung zu erklären, die man häufig in den normalen Epidermiszellen in der Umgebung der Hauttumoren beobachtet, wurden 0.5 und 1.0% Procainhydrochlorid mit und ohne Epinephrine injiziert — entweder i.d. oder s.c. — und zwar bei reifen weißen männlichen Kaninchen. Die Injektionsstellen entweder sofort oder nach 15 min excidiert. Die Epidermis zeigte an der Stelle der i.d. Injektion eine signifikantere Zunahme der Zahl der vacuolisierteten Zellen als die der s.c. Injektionen (P〈0.001) ohne Rücksicht auf die Art der Lokalanaesthetica und die Zeitdauer zwischen Injektion und Excision. Die Elektronenmikroskopie stellte die intracytoplasmischen Vacuolen ohne abgrenzende Membran im Cytoplasma der Keratinocyten dar. Das im oberem Corium lokalisierte Ödem infolge der Injektion des Betäubungsmittels verursacht das Ödem und die Vacuolisierung in den bedeckenden Epidermiszellen. Die i.d. Injektion des Lokalanaestheticums möchten wir als eine Ursache der epidermalen Vacuolisierung auffassen.
    Notizen: Summary To elucidate the mechanism of vacuolation frequently encountered in the normal epidermal cells surrounding skin tumors, 0.5 and 1.0% procaine hydrochloride, and 1.0% lidocaine hydrochloride with and without epinephrine were injected either i.d. or s.c. into mature white male rabbits, and the sites of injection were biopsied either immediately or after 15 min. The epidermis of the site of intradermal injection showed a significant increase in the number of vacuolated cells in contrast to the site of s.c. injection (P〈0.001) irrespective of the kinds of local anesthetics and the time lapse from injection to biopsy. Electron microscopy revealed vacuole-formations without limiting membrane in the cytoplasm of the kerationocytes. It is suggested that the local edema in the upper dermis caused by injection of anesthetics gives rise to edema and vacuolation of overlying epidermal cells, and that i.d. injection of local anesthetics may be regarded as a cause of epidermal vacuolation.
    Materialart: Digitale Medien
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  • 16
    Digitale Medien
    Digitale Medien
    [s.l.] : Nature Publishing Group
    Nature 307 (1984), S. 251-252 
    ISSN: 1476-4687
    Quelle: Nature Archives 1869 - 2009
    Thema: Biologie , Chemie und Pharmazie , Medizin , Allgemeine Naturwissenschaft , Physik
    Notizen: [Auszug] The experimental procedures used are almost identical to those of Tanaka and Masuda2. About 0.7 to 3 g of each sample was decomposed. Rare-earth elements (REE) were separated from the major elements by an AG50W-X8 resin column in HC1 media, while Ce and Nd were separated using an AG50W-X8 resin ...
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 17
    Digitale Medien
    Digitale Medien
    [s.l.] : Nature Publishing Group
    Nature 299 (1982), S. 557-559 
    ISSN: 1476-4687
    Quelle: Nature Archives 1869 - 2009
    Thema: Biologie , Chemie und Pharmazie , Medizin , Allgemeine Naturwissenschaft , Physik
    Notizen: [Auszug] The structure of the rotor is shown schematically in Fig. 1; it has an axis of rotation on which rests a float and three transverse bars to which blades (or vanes) are attached with wax. The axis and transverse bars are made of glass capillaries. The blades at each end of the transverse bars ...
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 18
    Digitale Medien
    Digitale Medien
    Springer
    Cellular and molecular life sciences 37 (1981), S. 861-862 
    ISSN: 1420-9071
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Biologie , Medizin
    Notizen: Summary Swimming speed of sea urchin spermatozoa, measured by a light scattering technique, did not change with 0-20 mM Ca2+ in the medium. The speed was maximum at the normal concentration of Mg2+ (49 mM) in sea water.
    Materialart: Digitale Medien
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  • 19
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 138 (1982), S. 265-270 
    ISSN: 1432-1076
    Schlagwort(e): Myelin sheath ; neuropathy
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract A 3 year, 11 month-old Japanese male with congenital hypomyelination is described. Clinical features are delay of motor development, generalized muscle hypotonia and weakness, absent tendon reflexes due to peripheral neuropathy, and normal mental development. Electrophysiologically, nerve conduction velocities could not be measured. Histological examination of the right sural nerve revealed total of absence myelin of most of the myelinated fibers. Electronmicroscopically, there was a concentric network of lamellae formed by double-layered sheets of basement membranes with fragments of Schwann cell cytoplasm around the myelinated fibers, so called “onion-bulbs”. These peculiar features were similar to those in the cases reported by Lyon, (1969); Kennedy et al., (1977); Karch et al., (1975); and Anderson et al. (1973).
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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