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  • 1
    ISSN: 1432-1289
    Keywords: Schlüsselwörter Familiäre Amyloid Polyneuropathie ; Transthyretin ; Amyloidose ; Genetische Lebererkrankung ; Lebertransplantation ; Key words Familial Amyloid Polyneuropathy ; Transthyretin ; Amyloidosis ; Genetic liver disease ; Liver Transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A 59-year old male of German origin noticed exercise-independent cardiac arrhythmia two years before admission. An alanine 47 transthyretin variant of Familial Amyloid Polyneuropathy with hypertrophic cardiomyopathy, peripheral sensory-motor polyneuropathy, I. degree AV heart block was diagnosed. To diminish production and deposition of mutant transthyretin and to prevent disease progression orthotopic liver transplantation was performed. Prior to transplant the patient complained of inappetence. Postoperatively, he received a chemically defined enteral nutrition regime that was discontinued after 30 months until return of appetite and weight gain indicated marked improvement. However, a duodenal biopsy still demonstrated amyloid deposits 24 months after transplantation. Echocardiographic findings remained unchanged. Neurologic examination showed an improvement of sensory-motor polyneuropathy with regression of electromyographic changes. Only traces of variant transthyretin were detectable in plasma samples taken 12 months after the operation. During the 3 year follow-up, no additional symptoms have occurred and progression of amyloidosis was prevented. Currently, orthotopic liver transplantation is the only specific treatment to prevent progression of familial amyloid polyneuropathy.
    Notes: Zusammenfassung Anläßlich der Abklärung von Herzrhythmusstörungen wurde bei einem 59-jährigen Patienten deutscher Herkunft eine Familiäre Amyloid Polyneuropathie (FAP) mit hypertropher Kardiomyopathie, beginnender peripherer Polyneuropathie, AV-Block I. Grades, intermittierend II. Grades Typ MOBITZ, und einer Aminosäuresubstitution von Glycin durch Alanin an Position 47 des Transthyretins (TTR Ala47) diagnostiziert. Um die weitere Bildung und Ablagerung des mutierten Transthyretins zu reduzieren und den Progreß der Erkrankung zumindest aufzuhalten, wurde die Indikation zur Lebertransplantation gestellt. Bei sonstiger Symptomfreiheit entwickelte der Patient bereits vor Transplantation eine progrediente Inappetenz. Postoperativ erhielt der Patient eine enteral-supportive Ernährung über Duodenalsonde für einen Zeitraum von 30 Monaten, bis sich der Appetit verbesserte und das Körpergewicht stabilisierte. In Biopsien der Duodenalschleimhaut ließ sich 24 Monate nach Transplantation weiterhin Amyloid nachweisen. Wie bereits vorTransplantation bestanden eine Verminderung von Magenperistaltik und Duodenalmotilität sowie eine hypertrophe Kardiomyopathie ohne wesentliche funktionelle Einschränkungen. Eine diskrete Verbesserung der sensomotorischen Polyneuropathie konnte anhand der Normalisierung elektromyographischer Veränderungen belegt werden. 12 Monate nach Transplantation waren nur noch Spuren der Transthyretinvariante (TTR Ala47) im Plasma zu finden. Während des bisherigen Beobachtungszeitraumes von mehr als 3 Jahren nach Transplantation sind keine neuen Symptome aufgetreten. Eine Progression der Erkrankung konnte eindeutig verhindert werden. Die orthotope Lebertransplantation ist gegenwärtig die einzige erfolgversprechende Therapieform, um eine Progression der Familiären Amyloid Polyneuropathie zu verhindern.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1573-4854
    Keywords: porous silicon ; patterning ; photolythography dielectric filters ; reactive ion etching (RIE) ; microoptics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: Abstract Porous silicon (PS) layer systems have a broad range of possible applications. An advantage is the good control of the refractive index and the etch rate of the layers by the applied current density and the time respectively. For micro-optical devices you need patterned PS. For some optical devices it is not sufficient to have only one filter but it is necessary to form filters with different properties on a small area. We applied a method (M. Frank, U.B. Schallenberg, N. Kaiser, and W. Buß, in Conference on Miniaturized Systems with Microoptics and Micromechanics, edited by M.E. Moamedi, L.J. Hornbeck, and K.S.J. Pister (SPIE, San Jose, 1997), SPIE Proceedings Series 3008, p. 265) to PS which fits this goal by the following steps: fabrication of the desired reflectors below each other and partial removal of upper reflectors with reactive ion etching (RIE). The technological aspects of patterning PS after the fabrication are an important topic of this work. Problems are discussed in detail and solutions are given.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1573-4854
    Keywords: porous silicon ; oxidation ; liquid crystal
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: Abstract In this paper we demonstrate the filling of porous silicon (PS) layers with liquid crystals (LC's) in order to control the reflectance electrically. The preparation of PS and the choice of the right group of LC's will be presented. Especially an oxidation of PS is necessary so that the methods and parameters of oxidation will also be discussed. As a first result the increasing and decreasing of the thickness oscillations in the reflectance as a function of the applied voltage can be observed.
    Type of Medium: Electronic Resource
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