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Adrenogenitales Syndrom und Wachstumshormonmangel

Betreuung beim Übergang zum Erwachsenenalter

Congenital adrenal hyperplasia and growth hormone deficiency

Special care in transition to adulthood

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Zusammenfassung

Patienten mit chronischen endokrinen Erkrankungen, die seit dem Kindesalter in pädiatrisch-endokrinologischen Zentren behandelt werden, bedürfen auch im Erwachsenenalter einer adäquaten endokrinologischen Betreuung. Um den Übergang in die Erwachsenenendokrinologie zu optimieren, wurde von pädiatrischen und internistischen Endokrinologen das Modell einer gemeinsamen Übergangssprechstunde entwickelt. Dieses Modell erlaubt es, Erfahrungen auszutauschen und die Kenntnisse und das Verständnis für die spezifischen Probleme der jeweils anderen Seite zu verbessern, mit dem Ziel, eine möglichst optimale Versorgung der Patienten zu gewährleisten. Am Beispiel von zwei endokrinen Erkrankungen, dem klassischen adrenogenitalen Syndrom mit Defekt der 21-Hydroxylase und dem Wachstumshormonmangel, der im Kindesalter diagnostiziert und behandelt und im Jugendalter bestätigt wurde, stellen wir exemplarisch die Probleme des Übergangs in die Erwachsenenmedizin dar. Spezielle Probleme bestehen dabei oft in der Loslösung vom bisher betreuenden Pädiater und in der Patientencompliance. Dies führt oft zu einer erheblichen Verschlechterung in der Qualität der Therapie.

Abstract

Children with chronically endocrine diseases should be treated as young adults by adult endocrinologists. To optimize the transfer from the pediatric to adult endocrinologist, the model of a common transition clinic has been developed. Within this setting it should be possible to exchange experiences, extend the knowledge and understanding of the disease with the other side, and to provide for the patient an optimal outpatient care. This model, however, has only been sporadically realized to date. To set an example for the problems of the transition into adult endocrinology, we used two different endocrine diseases, the classical congenital adrenal hyperplasia due to 21-hydoxylase deficiency, and the childhood-onset growth hormone deficiency. Specific problems for this transfer to adult care are the fixation of the patients to their pediatricians and the lack of comprehension in the need of a long term and continuous therapy. The consequence is a dramatic impairment in the quality of the therapy.

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Dörr, H., Schöfl, C. Adrenogenitales Syndrom und Wachstumshormonmangel. Internist 50, 1202–1212 (2009). https://doi.org/10.1007/s00108-009-2401-3

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