Liver transplantation in familial amyloid polyneuropathy. Case report and review of the literature

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.     

Si-containing copolymer of 2,3-epoxypropyl-1-methacrylate

Summary Poly-p-trimethylsilylphenylmethacrylate(75)-co-glycidylmethacrylate (25) containing aromatic bisazides exhibits uv sensitivity as a negative type photoresist. Sensitometric data have been evaluated. The pressure-dependant reactivity under the conditions of oxygen reactive ion etching was determined.     

Einfaches und spezifisches HPLC-Verfahren zur Bestimmung von Cyclamat in fruchtsafthaltigen Getr?nken nach Derivatisierung zu N,N-Dichlorcyclohexylamin

Zusammenfassung Es wird eine HPLC-Methode zur Bestimmung von Cyclamat in fruchtsafthaltigen Getränken vorgestellt, welche auf der Umsetzung von Cyclamat zuN,N-Dichlorcyclohexylamin beruht. Die HPLC-Analyse erfolgt an einer RP 18-Trennsäule mit Hilfe der Detektion bei 314 nm. Es lassen sich Gehalte von 10 bis 800 mg/1 mit einer relativen Standardabweichung von weniger als 2% analysieren. Bei verschiedenen fruchtsafthaltigen Getränken lag die Wiederfmdungsrate für Cyclamat bei 96 bis 99%. Die Einfachheit und Schnelligkeit (Gesamtanalysenzeit ca. 30 min) lassen die Methode insbesondere für die Routineanalytik geeignet erscheinen. Daneben wird eine Methode zur Isolierung von Cyclamat mittels Festphasenextraktion an Amino-Anionenaustauscher beschrieben.

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Simple and specific HPLC procedure for determination of cyclamate in fruit juice beverages after conversion to N,N-dichlorcyclohexylamine

Summary A high pressure liquid chromatography (HPLC) procedure for the determination of cyclamate in fruit juice beverages is described, which depends on the conversion of cyclamate toN,N-dichlorcyclohexylamine. For HPLC analysis an RP 18 separation system with detection at 314 nm is used. Contents of 10 to 800 mg/L can be detected in about 30 min with a relative standard deviation of less than 2%. Recovery of cyclamate from several fruit juice beverages amounts to 96–99%. This simple and rapid method seems to be very well suited for routine analysis. A method for solid-phase extraction of cyclamate with amino-anion-exchange columns is also presented.

     

Alkali Capacity and Physical Properties of Blast Furnace Type Slags

Alkali compounds, mainly those of Na and K, generally play a negative role in ironmaking blast furnaces. As easily circulating substances they consume energy, disturb furnace operation in the shaft and represent undesired impurities in the discharged slag and dust. An important task is to concentrate the alkalis in the slag as the best possible way. Control and optimisation of BF slag composition with respect to the alkalis requires reliable knowledge of basic data on the thermochemical and physical properties of the various slag compositions in the relevant iron‐slag system and in a wider range of temperatures according to the different reaction zones in the blast furnace. In the present paper, data are critically reviewed on free energies of reaction, the solubilities, bacicities and capacities of alkali oxides as well as on physical properties of alkali‐containing slags such as density, viscosity and surface tension. Furthermore alkali behaviour in primary and secondary BF slags and alkali circulation in the blast furnace are outlined. In a subsequent paper, new data on the chemical activities of alkali oxides in BF type slags and the kinetics and rates of alkali evaporation from these slags will be presented.     

Aluminum and the human body

Aluminium is one of the trace elements in the human organism the presence of which is described as contamination. The authors describe the toxic action in subjects working in an environment contaminated with powdered aluminium or its compounds. Non-professional exposure is observed in subjects with renal failure as aluminium is retained, in particular in case of concurrent antacid therapy on the basis of aluminium salts. Dialyzation encephalopathy may develop, aluminium osteopathy and microcytic anaemia. The most effective prevention is dialysis using a dialyzation solution with a low aluminium content and elimination of aluminium binding phosphates. The results of hitherto accomplished studies call for further research into the action of this element on the human organism.     

Gebrauchsgegenst?nde

Ohne Zusammenfassung     

Fleisch, Fleischwaren und di?tetische N?hrmittel

Ohne Zusammenfassung     

Die Bestimmung von St?rke in Pektin- und Apfels?ften auf sedimentrischem Wege

Ohne Zusammenfassung