Effekte eines späten Beginns einer Therapie mit Wachstumshormon View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2018-04

AUTHORS

H.-G. Dörr, M. Bettendorf, G. Binder, J. Dötsch, B. Hauffa, K. Mohnike, H. L. Müller, J. Woelfle

ABSTRACT

Nach Diagnosestellung sollten kleinwüchsige Kinder im Rahmen der in Deutschland zugelassenen Indikationen auch rechtzeitig mit Wachstumshormon (HGH) behandelt werden. Was passiert aber, wenn die Diagnose spät gestellt wird und die Kinder bei Therapiebeginn schon relativ alt sind? Eine Expertengruppe pädiatrischer Endokrinologen hat sich diese Frage gestellt und für die zugelassenen Indikationen, anhand der Evidenz aus klinischen Studien, die daraus resultierenden Konsequenzen evaluiert. Bei Kindern mit Wachstumshormonmangel (GHD) ist ein später Therapiebeginn in der Regel ab einem Alter über 12 Jahre und nach Pubertätsbeginn der Fall. Die publizierten Daten zeigen insbesondere bei Kindern mit idiopathischem GHD eine deutlich reduzierte Wirksamkeit. Für eine Empfehlung zur Anhebung der HGH-Dosis in der Pubertät bei GHD ist die Evidenz unzureichend. Kinder mit postnatalem Kleinwuchs bei „small for gestational age“ (SGA), die erst nach Einsetzen der Pubertät vorgestellt werden, sollten nur in gut begründeten Ausnahmefällen eine HGH-Therapie erhalten. Bei Mädchen mit Ullrich-Turner-Syndrom konnten bei einem Therapiebeginn nach einem chronologischen Alter über 12 Jahren noch vergleichbare Endgrößen wie bei jüngeren Mädchen erzielt werden. Bei der Indikation Short-stature-homeobox-gene(SHOX)-Syndrom und bei Kindern mit Prader-Willi-Syndrom (PWS) liegen keine aussagekräftigen Daten zu einem späten Therapiebeginn vor. Neue Daten zeigen, dass der Therapiebeginn bei Kindern mit PWS bereits im Säuglingsalter erfolgen soll. Für Patienten mit chronischer Niereninsuffizienz ergibt die begrenzte Datenlage für den HGH-Therapiebeginn in der frühen Pubertät einen vergleichbaren Effekt wie bei einem präpubertären Therapiebeginn. Zusammenfassend muss die Option eines späten HGH-Therapiebeginns individuell, anhand der zu der betreffenden Indikation jeweils verfügbaren Daten, überprüft werden. Weitere Untersuchungen und Analysen sind notwendig, da bei einigen Fragen zum späten HGH-Therapiebeginn die Datenlage noch unbefriedigend ist. More... »

PAGES

317-324

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    http://scigraph.springernature.com/pub.10.1007/s00112-017-0267-3

    DOI

    http://dx.doi.org/10.1007/s00112-017-0267-3

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    https://app.dimensions.ai/details/publication/pub.1084018666


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