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Weltweit wurden bislang 50\u00a0F\u00e4lle genetisch gesichert. Autosomal-dominante und rezessive Mutationen des <jats:italic>COL6A1<\/jats:italic>\/<jats:italic>COL6A2<\/jats:italic> im Chromosom 21q22.3 oder des <jats:italic>COL6A3<\/jats:italic> im Chromosom 2q37.3 f\u00fchren zu einem Mangel an Kollagen\u00a0VI. Typische Merkmale der UCMD sind Muskelschw\u00e4che von K\u00f6rperstamm und Extremit\u00e4ten, Hyperflexibilit\u00e4t der distalen und Kontrakturen der proximalen Gelenke, Rollstuhlpflichtigkeit im Alter von 9 bis 11\u00a0Jahren, Versteifung und Skoliose der Wirbels\u00e4ule und eine progrediente restriktive Ventilationsst\u00f6rung. Etwa 50\u202f% der Kinder ben\u00f6tigen im Alter von 11 bis 12\u00a0Jahren eine nichtinvasive Ventilation (NIV), wozu auch eine gest\u00f6rte Funktion des Diaphragmas beitr\u00e4gt. Es wird \u00fcber die Narkose bei einer 21-j\u00e4hrigen Patientin mit einer UCMD berichtet, die seit dem 6.\u00a0Lebensjahr rollstuhlpflichtig war und bei der seit 2018 eine lebenserhaltene NIV erfolgte. Wegen einer subpleuralen Einblutung in den linken Lungenunterlappen nach Entlastung eines Pneumothorax wurde eine videoassistierte thorakoskopische Chirurgie (VATS) vorgenommen. Die spezifischen Anforderungen durch die UCMD, das Atemwegsmanagement f\u00fcr die Einlungenventilation sowie Aspekte zur Auswahl der An\u00e4sthetika werden diskutiert. Nach erfolgreicher VATS konnte die Patientin am 7.\u00a0postoperativen Tag in die H\u00e4uslichkeit entlassen werden.<\/jats:p>","DOI":"10.1007\/s00101-022-01124-9","type":"journal-article","created":{"date-parts":[[2022,5,30]],"date-time":"2022-05-30T19:02:37Z","timestamp":1653937357000},"page":"784-788","update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["An\u00e4sthesie bei einer thoraxchirurgischen Patientin mit kongenitaler Muskeldystrophie Typ Ullrich"],"prefix":"10.1007","volume":"71","author":[{"given":"Thomas","family":"Hachenberg","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thorsten","family":"Walles","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eva","family":"L\u00fccke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Schilling","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,5,30]]},"reference":[{"issue":"10","key":"1124_CR1","doi-asserted-by":"publisher","first-page":"641","DOI":"10.1097\/EJA.0000000000000672","volume":"34","author":"M Schieren","year":"2017","unstructured":"Schieren\u00a0M, Defosse\u00a0J, B\u00f6hmer\u00a0A, Wappler\u00a0F, Gerbershagen\u00a0MU (2017) Anaesthetic management of patients with myopathies. 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F\u00fcr die aufgef\u00fchrten Studien gelten die jeweils dort angegebenen ethischen Richtlinien. F\u00fcr Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts, \u00fcber die Patienten zu identifizieren sind, liegt von ihnen und\/oder ihren gesetzlichen Vertretern eine schriftliche Einwilligung vor.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}}]}}