Strategien zur Verhinderung von Fehlbedienungen in verfahrenstechnischen Anlagen Materialband. Leitfaden zur Beruecksichtigung der Human-Factor-Aspekte in verfahrenstechnischen Anlagen

SIGLEAvailable from TIB Hannover: RN 8422(2001,12) / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekBundesministerium fuer Umwelt, Naturschutz und Reaktorsicherheit, Bonn (Germany)DEGerman

Strategien zur Verhinderung von Fehlbedienungen in verfahrenstechnischen Anlagen Abschlussbericht

SIGLEAvailable from TIB Hannover: RN 8422(2001,11) / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekBundesministerium fuer Umwelt, Naturschutz und Reaktorsicherheit, Bonn (Germany)DEGerman

Strategien zur Vermeidung von Fehlbedienungen in verfahrenstechnischen Anlagen Abschlussbericht und Materialband: Leitfaden zur Beruecksichtigung der Human Factor-Aspekte in verfahrenstechnischen Anlagen

As a consequence of the improved reliability of automatic plant regulating systems, the relative importance of operational errors as an incident cause is increasing, resulting in a growing relative importance of human reliability for the avoidance or control of unforeseen operating conditions. Technical reliability has been further improved in recent years. Therefore, systematic optimisation of human reliability remains a pending issue to improve the safety of plants. In the course of this research project, recommendations for an improved consideration of human factor were derived. In order to achieve this goal, new methods to represent the requirements to be met by operators (compared with the objective human limits) were developed and tested at existing plants. Existing regulations, rules, and instruments (e.g. safety management systems) aimed at taking into account the human factor were analysed and recommendations for their further development were derived. A 'Guide for the consideration of human factor-related aspects in chemical plants' was elaborated as an aid for the corporate practice. (orig.)Mit hoehrerer Zuverlaessigkeit automatischer Anlagensteuerungen steigt der Anteil menschlicher Fehlhandlungen als Stoerfallursache in verfahrenstechnischen Anlagen. Daraus resultiert eine zunehmende relative Bedeutung menschlicher Zuverlaessigkeit fuer die Vermeidung bzw. Beherrschung von nicht-bestimmungsgemaessen Betriebszustaenden. Der Bereich technischer Zuverlaessigkeit ist inzwischen weitgehend optimiert worden; als wichtiger Handlungsbereich zur Verbesserung der Anlagensicherheit verbleibt noch die systematische Optimierung der menschlichen Zuverlaessigkeit. Im Rahmen des Forschungsprojekts wurden Vorschlaege zur besseren Beruecksichtigung des 'Human Factors' erarbeitet. Hierzu wurden Methoden zur Darstellung der an einen Bediener gestellten Anforderungen (zum Vergleich mit den objektiven Leistungsgrenzen des Menschen) entwickelt und anhand konkreter Anlagen getestet. Es wurden bestehende Vorschriften, Regeln und Instrumente (z.B. Sicherheitsmanagementsysteme) zur Beruecksichtigung des Human Factor analysiert und Vorschlaege zu deren Weiterentwicklung erarbeitet. Als Hilfsmittel fuer die betriebliche Praxis wurde ein 'Leitfaden zur Beruecksichtigung der Human Factor-Aspekte in verfahrenstechnischen Anlagen' entwickelt. (orig.)Published in 2 volumesAvailable from TIB Hannover: RN 8908(2000,140,1): RN 8908(2000,140,2) / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekSIGLEDEGerman

Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families

A biochemical variant of argininosuccinate lyase deficiency, found in five individuals, is introduced. In comparison to classical patients, the variant cases of argininosuccinate lyase deficiency were characterized by residual enzyme activity as measured by the incorporation of [14C]citrulline into proteins. The five patients of different ethnic backgrounds presented with relatively mild clinical symptoms, variable age of onset, marked argininosuccinic aciduria and severe, but not complete, deficiency of argininosuccinate lyase. [14C]Citrulline incorporation into proteins, which is completely blocked in classical argininosuccinic aciduria, was only partially reduced in fibroblasts of these patients. Further investigation showed that previous standard conditions of the assay were not optimal. Higher concentrations of citrulline in the incubation medium strongly stimulated 14C incorporation in normal cells, but not in the patients; as a result, the relative incorporation level in the patients dropped to 6-28% compared to 18-75% of normal in the original procedure. Prenatal diagnosis was successfully performed in three of the families. Affected pregnancies were indicated by (partial) deficiency of [14C]citrulline incorporation in chorionic villi and/or increased levels of argininosuccinate in amniotic fluid. Analysis of the ASL gene in the five patients revealed a considerable allelic heterogeneity. Three novel mutations--R385C (2 patients), V178M and R379C--were detected in homozygous states, whereas one patient was compound heterozygous for the known mutations R193Q and Q286R. In conclusion, there are patients of different ethnic backgrounds who are characterized by residual activity of argininosuccinate lyase and who present with less severe clinical courses. In addition, we present an improved biochemical assay for accurate prenatal and postnatal diagnosi