Ontology type: schema:ScholarlyArticle
2018-04
AUTHORSH.-G. Dörr, M. Bettendorf, G. Binder, J. Dötsch, B. Hauffa, K. Mohnike, H. L. Müller, J. Woelfle
ABSTRACTNach 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... »
PAGES317-324
http://scigraph.springernature.com/pub.10.1007/s00112-017-0267-3
DOIhttp://dx.doi.org/10.1007/s00112-017-0267-3
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"description": "Nach Diagnosestellung sollten kleinw\u00fcchsige Kinder im Rahmen der in Deutschland zugelassenen Indikationen auch rechtzeitig mit Wachstumshormon (HGH) behandelt werden. Was passiert aber, wenn die Diagnose sp\u00e4t gestellt wird und die Kinder bei Therapiebeginn schon relativ alt sind? Eine Expertengruppe p\u00e4diatrischer Endokrinologen hat sich diese Frage gestellt und f\u00fcr die zugelassenen Indikationen, anhand der Evidenz aus klinischen Studien, die daraus resultierenden Konsequenzen evaluiert. Bei Kindern mit Wachstumshormonmangel (GHD) ist ein sp\u00e4ter Therapiebeginn in der Regel ab einem Alter \u00fcber 12 Jahre und nach Pubert\u00e4tsbeginn der Fall. Die publizierten Daten zeigen insbesondere bei Kindern mit idiopathischem GHD eine deutlich reduzierte Wirksamkeit. F\u00fcr eine Empfehlung zur Anhebung der HGH-Dosis in der Pubert\u00e4t bei GHD ist die Evidenz unzureichend. Kinder mit postnatalem Kleinwuchs bei \u201esmall for gestational age\u201c (SGA), die erst nach Einsetzen der Pubert\u00e4t vorgestellt werden, sollten nur in gut begr\u00fcndeten Ausnahmef\u00e4llen eine HGH-Therapie erhalten. Bei M\u00e4dchen mit Ullrich-Turner-Syndrom konnten bei einem Therapiebeginn nach einem chronologischen Alter \u00fcber 12 Jahren noch vergleichbare Endgr\u00f6\u00dfen wie bei j\u00fcngeren M\u00e4dchen erzielt werden. Bei der Indikation Short-stature-homeobox-gene(SHOX)-Syndrom und bei Kindern mit Prader-Willi-Syndrom (PWS) liegen keine aussagekr\u00e4ftigen Daten zu einem sp\u00e4ten Therapiebeginn vor. Neue Daten zeigen, dass der Therapiebeginn bei Kindern mit PWS bereits im S\u00e4uglingsalter erfolgen soll. F\u00fcr Patienten mit chronischer Niereninsuffizienz ergibt die begrenzte Datenlage f\u00fcr den HGH-Therapiebeginn in der fr\u00fchen Pubert\u00e4t einen vergleichbaren Effekt wie bei einem pr\u00e4pubert\u00e4ren Therapiebeginn. Zusammenfassend muss die Option eines sp\u00e4ten HGH-Therapiebeginns individuell, anhand der zu der betreffenden Indikation jeweils verf\u00fcgbaren Daten, \u00fcberpr\u00fcft werden. Weitere Untersuchungen und Analysen sind notwendig, da bei einigen Fragen zum sp\u00e4ten HGH-Therapiebeginn die Datenlage noch unbefriedigend ist.",
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]Download the RDF metadata as: json-ld nt turtle xml License info
JSON-LD is a popular format for linked data which is fully compatible with JSON.
curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1007/s00112-017-0267-3'
N-Triples is a line-based linked data format ideal for batch operations.
curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1007/s00112-017-0267-3'
Turtle is a human-readable linked data format.
curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1007/s00112-017-0267-3'
RDF/XML is a standard XML format for linked data.
curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1007/s00112-017-0267-3'
This table displays all metadata directly associated to this object as RDF triples.
286 TRIPLES
20 PREDICATES
77 URIs
19 LITERALS
7 BLANK NODES