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  • 1
    ISSN: 1432-0533
    Keywords: Pyroglutamic aciduria ; 5-Oxoprolinuria ; Glutathione deficiency ; Encephalopathy ; Cerebellar atrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical and pathologic features of a male patient with generalized glutathione deficiency and pyroglutamic aciduria are presented. The patient died at the age of 28 years. He was mentally retarded from infancy and developed progressive tremor, retardation of movement, and ataxia as from the age of 16. Neuropathologic examination of the brain disclosed a selective atrophy of the granule cell layer of the cerebellum and focal lesions in the right frontoparietal cortex and bilaterally in the visual cortex and the thalamus. The type and distribution of the lesions resembled those seen after mercury intoxication. However, in our patient the damage was probably caused by the lack of protection by glutathione against oxidative damage in the brain. Possible treatment of this rare metabolic disorder might include external supply of an antioxidant, e.g., a thiol capable of penetrating the blood brain barrier.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1618-2650
    Keywords: Elementaranalyse, Datenverarbeitung ; Organisation eines Labors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Zusammenfassung Es wird das Konzept eines Echtzeit-Datenverarbeitungssystems beschrieben, das in Kombination mit mechanisierten und automatisierten Analysenmethoden eine Verbesserung der analytischen Serviceleistung ermöglicht. Die Hardware setzt sich im wesentlichen aus einem 28 K-Worte Kleincomputer mit 2 Plattenspeichereinheiten, Bedienungskonsolen und Beleglesern zusammen. Die hauptsächlichen Funktionen dieses Teils der Anlage sind: Registrieren der Aufträge, Erstellen von Arbeitslisten für die Laboratorien, Überwachen der termingerechten Auftragserledigung, Sammeln, Speichern und Auswerten der anfallenden Resultate, Treffen einfacher Entscheidungen und schließlich Erstellen und Ausdrucken der Analysenbefunde. Darüber hinaus verfertigt der Computer täglich Berichte über den Stand der Auftragserledigung und speichert Daten für die Statistiken und Kostenabrechnungen. Zur Datenübertragung aus den Laboratorien stehen 36 Linien zur Verfügung. Dabei ist wesentlich, daß elektronisch vorverarbeitete Daten, also fertig berechnete Resultate übertragen werden. Dazu dienen on-line angeschlossene Tischrechner, Datenstationen und Belegleser sowie Datenverarbeitungseinheiten (Prozent-Box) eigener Konstruktion. Die Arbeitsplätze in den Laboratorien sind somit weitgehend autonom. Dieses hierarchische Datenverarbeitungssystem bringt arbeitstechnische Vorteile, gewährleistet aber vor allem die ständige Servicebereitschaft unserer Laboratorien, da auch bei Ausfall des Computers an den Analysengeräten unbeeinflußt weitergearbeitet werden kann.
    Notes: Abstract To increase the efficiency of the analytical services the analytical methods have been improved by mechanisation and automation, and combined with a real-time data processing system. The hardware consists essentially of a 28 K-words minicomputer with 2 disc storage units, teletypes and optical mark readers. The main functions of this part of the system are as follows: registering the incoming requests, supplying work schedules for the various laboratories, controlling the completion of the work within the required time, collecting, storing, and processing the results, making simple decisions, and finally, editing and printing the analysis reports. Moreover, the computer produces daily reports on the state of progress of requests, and stores the data for usage in statistics and cost accounting. There are 36 lines available for the transfer of data from the laboratories. An important feature of the system design is the transfer of electronically preprocessed data, i.e. already calculated final results. For this purpose, on-line desk-top calculators, alphanumeric display data terminals, and data preprocessing units (percentage-box) designed and developed in our own laboratories are being used. The individual places of work in the laboratories are thus to a great extent autonomous. This hierarchical data processing system yields technical advantages, and most of all, guarantees a permanent service ability of our laboratories since even in the case of a breakdown of the computer, the analytical work can be continued without any interference.
    Type of Medium: Electronic Resource
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