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Hierbei besteht das H\u00e4moglobin zu \u00fcber 50\u202f% aus HbS. Die Sichelzellkrankheit tritt \u00fcberwiegend im Mittelmeerraum, dem mittleren Osten, in Zentralafrika, Indien und Amerika auf. Im desoxygenierten Zustand pr\u00e4zipitiert HbS, die Erythrozyten nehmen die typische Sichelzellform an und es kann zur Okklusion von kleinen Gef\u00e4\u00dfen kommen. Akut k\u00f6nnen Gewebeisch\u00e4mien mit Knochen- oder Organinfarkten auftreten, langfristig Organsch\u00e4den. Bez\u00fcglich des Krankheitsverlaufs und der Komplikationen gibt es deutliche Unterschiede zwischen den verschiedenen Genotypen. Daher ist eine genaue Kenntnis der Erkrankung besonders wichtig. Hydroxycarbamid senkt die Mortalit\u00e4t und Morbidit\u00e4t bei Patienten mit Sichelzellkrankheit. Derzeit sind neue Medikamente und Therapieans\u00e4tze wie die Gentherapie in Erprobung.<\/jats:p>","DOI":"10.1007\/s00108-020-00822-z","type":"journal-article","created":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T13:04:16Z","timestamp":1592312656000},"page":"754-758","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Sichelzellkrankheit","Sickle cell disease"],"prefix":"10.1007","volume":"61","author":[{"given":"L.","family":"Distelmaier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"U.","family":"D\u00fchrsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Dickerhoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,6,16]]},"reference":[{"key":"822_CR1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/727905","author":"M Agastinoiotis","year":"2013","unstructured":"Agastinoiotis\u00a0M, Corrons\u00a0JV, Soteriades\u00a0E et\u00a0al (2013) The impact of migrations on the health services for rares diseases in Europe: the example of haemoglobin disorders. 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