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Der Internist

, Volume 57, Issue 5, pp 457–469 | Cite as

Morbus Addison

Primäre Nebenniereninsuffizienz
  • A. Pulzer
  • S. Burger-Stritt
  • S. HahnerEmail author
CME

Zusammenfassung

Die Nebenniereninsuffizienz (NNI) ist eine seltene Erkrankung, die durch eine verminderte oder gänzlich fehlende Synthese von Nebennierenhormonen bedingt ist. Die primäre NNI, bei der die Ursache im Bereich der Nebenniere selbst liegt, ist von sekundären Formen zu differenzieren, bei denen übergeordnete Zentren der kortikotropen Achse betroffen sind. Hauptursache der primären NNI ist insbesondere in den Industrienationen der Morbus Addison. Dessen Prävalenz liegt bei etwa 11 pro 100.000 Einwohner. Aufgrund einer meist unspezifischen klinischen Symptomatik wird die Diagnose häufig verzögert gestellt. Die Diagnosesicherung erfolgt mittels ACTH-Stimulationstest. Therapeutische Maßnahmen beinhalten die Substitution der fehlenden adrenokortikalen Hormone. Darüber hinaus sind eine endokrinologische Anbindung sowie regelmäßige Schulungen zur Prävention von Nebennierenkrisen essenziell. Jeder Patient mit NNI-Insuffizienz sollte einen Notfallausweis und eine Notfallausrüstung mit sich führen.

Schlüsselwörter

Adrenokortikotropes Hormon Hormonersatztherapie Hydrokortison Fludrokortison Dehydroepiandrosteron 

Addison’s disease

Primary adrenal insufficiency

Abstract

Adrenal insufficiency, a rare disorder which is characterized by the inadequate production or absence of adrenal hormones, may be classified as primary adrenal insufficiency in case of direct affection of the adrenal glands or secondary adrenal insufficiency, which is mostly due to pituitary or hypothalamic disease. Primary adrenal insufficiency affects 11 of 100,000 individuals. Clinical symptoms are mainly nonspecific and include fatigue, weight loss, and hypotension. The diagnostic test of choice is dynamic testing with synthetic ACTH. Patients suffering from chronic adrenal insufficiency require lifelong hormone supplementation. Education in dose adaption during physical and mental stress or emergency situations is essential to prevent life-threatening adrenal crises. Patients with adrenal insufficiency should carry an emergency card and emergency kit with them.

Keywords

Adrenocorticotropic hormone Hormone replacement therapy Hydrocortisone Fludrocortisone Dehydroepiandrosterone 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

S. Hahner erhielt 2015 ein Vortragshonorar der Firma Shire. A. Pulzer und S. Burger-Stritt geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  1. 1.Medizinische Klinik und Poliklinik I, Schwerpunkt Endokrinologie und DiabetologieUniversitätsklinikum WürzburgWürzburgDeutschland

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