{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,4,2]],"date-time":"2022-04-02T10:18:13Z","timestamp":1648894693580},"reference-count":25,"publisher":"Georg Thieme Verlag KG","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Fortschr Neurol Psychiatr"],"published-print":{"date-parts":[[2021,12]]},"abstract":"Zusammenfassung<\/jats:title>\n Hintergrund Der Morbus Pompe ist eine lysosomale Multisystemerkrankung mit\n pr\u00e4dominanter Myopathie, f\u00fcr die eine Enzymersatztherapie (EET) mit rekombinanter\n \u03b1-Glucosidase verf\u00fcgbar ist. Diese muss aktuell zweiw\u00f6chentlich lebenslang erfolgen. Um\n die Belastung durch diese Behandlungsform gering zu halten, besteht bei vielen Betroffenen\n der Wunsch, die EET zu Hause als sog. Heiminfusionstherapie durchzuf\u00fchren.<\/jats:p>\n Ziele und Methoden Im Rahmen zweier Deutsch-\u00d6sterreichisch-Schweizerischer\n Konsensus-Expertentreffen in den Jahren 2019 und 2020 wurde diskutiert, welche\n Voraussetzungen gegeben sein m\u00fcssen, damit eine Heiminfusionstherapie medizinisch\n vertretbar erfolgen kann, wie diese sachgem\u00e4\u00df durchzuf\u00fchren ist und wie die Rechtslage f\u00fcr\n delegierende \u00c4rzte aussieht.<\/jats:p>\n Ergebnisse und Diskussion Prinzipiell ist bei Patienten mit Morbus Pompe eine\n Heiminfusionstherapie m\u00f6glich, wenn bestimmte Voraussetzungen erf\u00fcllt sind. Die\n Entscheidung muss f\u00fcr jeden Patienten individuell getroffen werden. Der behandelnde Arzt\n delegiert die Durchf\u00fchrung der Heiminfusionstherapie ad personam an eine\n Pflegekraft. Die Endverantwortung f\u00fcr die korrekte Durchf\u00fchrung der EET verbleibt aber\n beim Arzt. Die Heiminfusionstherapie muss durch speziell geschultes Pflegepersonal\n erfolgen. Hauptrisiko ist das Auftreten allergischer Reaktionen. Deren ad\u00e4quate\n Erstbehandlung muss ebenso sichergestellt sein wie die Verf\u00fcgbarkeit einer\n notfallmedizinischen Versorgung. Um die psychosoziale Belastung von Patienten mit Morbus\n Pompe in deutschsprachigen L\u00e4ndern zu minimieren und delegierenden \u00c4rzten gr\u00f6\u00dfere\n Rechtssicherheit zu geben, sind klarere Regelungen f\u00fcr die Durchf\u00fchrung einer\n Heiminfusionstherapie w\u00fcnschenswert.<\/jats:p>","DOI":"10.1055\/a-1365-8977","type":"journal-article","created":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T23:45:29Z","timestamp":1612914329000},"page":"630-636","source":"Crossref","is-referenced-by-count":0,"title":["Heiminfusionstherapie bei Morbus Pompe: Konsensusempfehlungen f\u00fcr den\n deutschsprachigen Raum"],"prefix":"10.1055","volume":"89","author":[{"given":"Andreas","family":"Hahn","sequence":"additional","affiliation":[{"name":"Abteilung Kinderneurologie, Sozialp\u00e4diatrie und Epileptologie,\n Universit\u00e4tsklinikum Gie\u00dfen und Marburg GmbH Standort Gie\u00dfen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina","family":"Lampe","sequence":"additional","affiliation":[{"name":"Abteilung Kinderneurologie, Sozialp\u00e4diatrie und Epileptologie,\n Universit\u00e4tsklinikum Gie\u00dfen und Marburg GmbH Standort Gie\u00dfen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthias","family":"Boentert","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Neurologie mit Institut f\u00fcr Translationale Neurologie,\n Universit\u00e4tsklinikum M\u00fcnster; Klinik f\u00fcr Innere Medizin, Bereich Neurologie,\n UKM-Marienhospital Steinfurt, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Hundsberger","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Neurologie, Kantonsspital St. Gallen, Schweiz"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"L\u00f6scher","sequence":"additional","affiliation":[{"name":"Department Neurologie, Medizinische Universit\u00e4t Innsbruck,\n \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephan","family":"Wenninger","sequence":"additional","affiliation":[{"name":"Friedrich-Baur- Institut der Neurologischen Klinik , Klinikum der Universit\u00e4t\n M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Ziegler","sequence":"additional","affiliation":[{"name":"Sektion f\u00fcr Neurop\u00e4diatrie und Stoffwechselmedizin, Zentrum f\u00fcr Kinder- und\n Jugendmedizin, Universit\u00e4tsklinikum Heidelberg, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florian","family":"Lagler","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinik f\u00fcr Kinder- und Jugendheilkunde Paracelsus Medizinische\n Privatuniversit\u00e4t Salzburg, \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diana","family":"Ballhausen","sequence":"additional","affiliation":[{"name":"Unit\u00e9 p\u00e9diatrique des maladies m\u00e9taboliques, D\u00e9partement Femme-M\u00e8re-Enfant,\n Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Schweiz"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Schlegel","sequence":"additional","affiliation":[{"name":"Kanzlei f\u00fcr Medizinrecht (Prof. Schlegel, Hohmann & Partner), Frankfurt am Main, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benedikt","family":"Schoser","sequence":"additional","affiliation":[{"name":"Friedrich-Baur- Institut der Neurologischen Klinik , Klinikum der Universit\u00e4t\n M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2021,2,9]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.1016\/S0140-6736(08)61555-X","article-title":"Pompe\u2019s disease","volume":"372","author":"AT van der Ploeg","year":"2008","journal-title":"Lancet"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1016\/j.ymgme.2010.08.001","article-title":"Differences in the predominance of lysosomal and autophagic pathologies between infants\n and adults with Pompe disease: Implications for therapy","volume":"101","author":"N Raben","year":"2010","journal-title":"Mol Genet Metab"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1212\/01.wnl.0000251268.41188.04","article-title":"Recombinant human acid alpha-glucosidase. 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