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Patienten mit dieser Erkrankung m\u00fcssen sich typischerweise h\u00e4ufig chirurgischen Interventionen unterziehen, die durch die Erkrankung und ihre Komplikationen notwendig werden. Die an\u00e4sthesiologische Betreuung der Betroffenen stellt naturgem\u00e4\u00df eine gro\u00dfe Herausforderung dar. 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