{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T05:47:32Z","timestamp":1751867252102,"version":"3.28.0"},"reference-count":8,"publisher":"Georg Thieme Verlag KG","issue":"03","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Z Geburtshilfe Neonatol"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Das Kasabach-Merrit-Syndrom ist charakterisiert als Assoziation eines\n                    Gef\u00e4\u00dftumors, typischerweise einem kaposiformem H\u00e4mangioendotheliom sowie einer\n                    schweren Verbrauchskoagulopathie mit potenziell lebensbedrohlichen\n                    Thrombozytopenien. Die schwere Gerinnungsst\u00f6rung mit erh\u00f6hter Blutungsneigung\n                    muss vor invasiven Eingriffen ber\u00fccksichtigt werden und erfordert nicht selten\n                    wiederholte Thrombozytenkonzentrat Transfusionen. Wir berichten hierbei \u00fcber ein\n                    reifes m\u00e4nnliches Neugeborenes mit Kasabach-Merrit-Syndrom und\n                    VACTERL-Assoziation. Die VACTERL-Assoziation beschreibt eine Gruppe von\n                    Fehlbildungen. Der Patient wies eine Analatresie (mit rektourethraler Fistel),\n                    zystischen Ver\u00e4nderungen im Spinalkanal (mit tethered cord) und einer\n                    Nierenagenesie links auf. Die VACTERL Assoziation sowie das\n                    Kasabach-Merritt-Syndrom fanden sich bei diesem Patienten als unabh\u00e4ngig\n                    voneinander gewertete Entit\u00e4ten. Ein gemeinsames Auftreten wurde bisher in der\n                    Literatur nicht beschrieben. Die eingeschr\u00e4nkte Gerinnungssituation durch\n                    schwere Thrombozytopenien erschwerte die operative Anus praeter Anlage sowie\n                    Biopsie Entnahme zur Diagnosesicherung. In unserem Fallbeispiel konnte\n                    schlie\u00dflich der mTOR Inhibitor Sirolimus erfolgreich zur Verkleinerung des\n                    Gef\u00e4\u00dftumors und besonders zur Senkung des Thrombozyten- Verbrauchs eingesetzt\n                    werden.<\/jats:p>","DOI":"10.1055\/a-2262-8607","type":"journal-article","created":{"date-parts":[[2024,3,2]],"date-time":"2024-03-02T00:17:30Z","timestamp":1709338650000},"page":"298-302","source":"Crossref","is-referenced-by-count":1,"title":["Ein seltener Fall: Kasabach-Merrit-Syndrom bei\n                    VACTERL-Assoziation"],"prefix":"10.1055","volume":"228","author":[{"given":"Melanie","family":"Le","sequence":"additional","affiliation":[{"name":"Klinik und Poliklinik f\u00fcr Kinderchirurgie, Universit\u00e4tsklinikum\n                    Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katharina","family":"Wenke","sequence":"additional","affiliation":[{"name":"Klinik und Poliklinik f\u00fcr Kinderchirurgie, Universit\u00e4tsklinikum\n                    Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jochen","family":"Herrmann","sequence":"additional","affiliation":[{"name":"Abteilung f\u00fcr Kinderradiologie, Klinik und Poliklinik f\u00fcr Diagnostische\n                    und Interventionelle Radiologie, Universit\u00e4tsklinikum Hamburg-Eppendorf,\n                    Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dominique","family":"Singer","sequence":"additional","affiliation":[{"name":"Neonatologie und P\u00e4diatrische Intensivmedizin, Universit\u00e4tsklinikum\n                    Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mario","family":"Lange","sequence":"additional","affiliation":[{"name":"Neonatologie und P\u00e4diatrische Intensivmedizin, Universit\u00e4tsklinikum\n                    Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2024,3,1]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1063","DOI":"10.1001\/archpedi.1940.01990160135009","article-title":"Capillary Hemangioma with Extensive Purpura","volume":"59","author":"H H Kasabach","year":"1940","journal-title":"Am J Dis Child"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1111\/bjh.13557","article-title":"Recent advances in the pathobiology and management of Kasabach-Merritt\n                    phenomenon","volume":"171","author":"C O'Rafferty","year":"2015","journal-title":"Br J Haematol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"1179545X17699849","DOI":"10.1177\/1179545X17699849","article-title":"Kasabach-Merritt Phenomenon: Classic Presentation and Management Options","volume":"10","author":"P Mahajan","year":"2017","journal-title":"Clin Med Insights Blood Disord"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"1396","DOI":"10.1002\/pbc.22766","article-title":"Treatment of childhood kaposiform hemangioendothelioma with sirolimus","volume":"55","author":"J Blatt","year":"2010","journal-title":"Pediatr Blood Cancer"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"1579","DOI":"10.1007\/s00431-015-2572-y","article-title":"Sirolimus for the treatment of children with various complicated vascular\n                    anomalies","volume":"174","author":"H Lackner","year":"2015","journal-title":"Eur J Pediatr"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"949950","DOI":"10.3389\/fped.2022.949950","article-title":"Case report: Experience of a rare case of rebound of the Kasabach-Merritt\n                    phenomenon during sirolimus treatment in kaposiform hemangioendothelioma","volume":"10","author":"L Wang","year":"2022","journal-title":"Front Pediatr"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"322","DOI":"10.1007\/s12519-018-0171-5","article-title":"Kaposiform hemangioendothelioma in children: a benign vascular tumor with\n                    multiple treatment options","volume":"14","author":"I Schmid","year":"2018","journal-title":"World J Pediatr"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1111\/ddg.12987_g","article-title":"Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: successful\n                    treatment with sirolimus","volume":"15","author":"A Reichel","year":"2017","journal-title":"J Dtsch Dermatol Ges"}],"container-title":["Zeitschrift f\u00fcr Geburtshilfe und Neonatologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2262-8607.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,5]],"date-time":"2024-11-05T14:14:41Z","timestamp":1730816081000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2262-8607"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,1]]},"references-count":8,"journal-issue":{"issue":"03","published-online":{"date-parts":[[2024,6,11]]},"published-print":{"date-parts":[[2024,6]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2262-8607","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0948-2393","1439-1651"],"issn-type":[{"type":"print","value":"0948-2393"},{"type":"electronic","value":"1439-1651"}],"subject":[],"published":{"date-parts":[[2024,3,1]]}}}