American Thoracic Society/European Respiratory Society: Standards in Diagnostik und Therapie bei Patienten mit Alpha-1-Antitrypsin-Mangel

Inhalt Seite Zusammenfassung Einleitung 37 Ziele, Organisation des Projektes und Zeitplan 37 Zusammenfassung der wichtigsten Empfehlungen der Arbeitsgruppe zur Diagnose und Behandlung des Alpha-1-Antitrypsin-Mangel 38 Klinische Erkennung des Alpha-1-Antitrypsin-Mangels 38 Genetische Testung auf Alpha-1-Antitrypsin-Mangel 38 Lebererkrankungen 39 Andere Erkrankungen 40 Wirksamkeit der Substitutionstherapie 40 Allgemeines Management bei obstruktiver Atemwegserkrankung 40 Literatur 41 Lungenerkrankung Erstellung dieses Dokuments 41 Einleitung 41 Epidemiologie 42 Pathophysiologie des Alpha-1-Antitrypsin-Mangels 42 Labortests 44 Erkennung von Individuen mit Alpha-1-Antitrypsin-Mangel 45 Pathologie 46 Symptome 46 Klinische Befunde 47 Lungenfunktionsuntersuchungen 47 Bildgebende Verfahren (einschließlich Computertomographie und Ventilations-Perfusionstherapie) 48 Verlaufsparameter: FEV1, Desmosine Computertomographie 49 Risikofaktoren 50 Krankheitsverlauf 51 Risiken der MZ- und SZ-Phenotypen für die Entwicklung von Lungenemphysem und COPD 53 Prognose 55 Prävention von Lungenerkrankung 55 Allgemeine medizinische Behandlung 56 Substitutionstherapie 56 Operative Maßnahmen 58 Spezielle Situationen 59 Zukünftige Richtung in der Forschung bei Alpha-1-Antitrypsin-Mangel 59 Spezieller Forschungsbedarf bei Alpha-1-Antitrypsin-Mangel 61 Literatur 62 Zur Zusammensetzung der Arbeitsgruppe siehe Tabelle am Ende der Arbeit.Dieses Statement wurde gemeinsam von einer Arbeitsgruppe der ATS/ERS entwickelt. Die Veröffentlichung dieses Statements wurde zum Teil unterstützt durch die Alpha-1-Foundation und durch Aventis Behring, LLC. Weitere Unterstützung erfolgte durch die American Thoracic Society, die European Respiratory Socienty, das American College of Chest Physicians und die American Association for Respiratory Care.Mitglieder des ad hoc Statement Komitees haben jegliche direkte kommerziellen Verbindungen (finanzielle Verbindungen oder gesetzliche Verpflichtungen) offengelegt, die mit der Vorbereitung dieses Statements verbunden sind. Die Information kann am ATS Headquarter eingesehen werden.Darüber hinaus wurde ähnlich wie bei Lungenkrankheiten eine Arbeitsgruppe eingesetzt zum Thema „Leber und andere Erkrankungen” und eine Arbeitsgruppe zum Thema „Genetische Testung auf Alpha-1-Antitrypsin-Mangel: ethische, legale, psychologische, soziale und ökonomische Aspekte”. Beide wurden nicht für die Übersetzung ins Deutsche vorgesehen. Der interessierte Leser sei aber auf die beiden profunden Publikationen hingewiesen.

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