Summary
Acute adrenal insufficiency or adrenal crisis is the life-threatening manifestation of glucocorticoid and mineralocorticoid deficiency. Generally, there are two types of adrenal failure: the first is caused by an adrenal disease, the second is the result of an impaired hypothalamic-pituitary-adrenal axis. A blunted mineralocorticoid production is the main pathogenic factor leading to dehydration and hypotensive shock. Diminished binding of the naturally occurring glucocorticoids on the mineralocorticoid receptor further aggravates the hemodynamic instability. More recently the important permissive effects of glucocorticoids on catecholamine action have been generally acknowledged and explain the poor responsiveness to catecholamines in untreated adrenal insufficiency. Clinical features of an adrenal crisis comprise fatigue, abdominal pain, nausea and fever. The severity of characteristic biochemical findings such as hyponatremia, hyperkalemia or hypoglycemia varies and depends on the corresponding hormone deficiencies. The diagnosis is usually confirmed by determination of circulating serum cortisol and plasma ACTH in addition to a standard corticotropin stimulation test. However, in an emergency setting laboratory results must not be awaited. After drawing the appropriate blood samples, 100 mg hydrocortisone has to be administered intravenously followed by a bolus of 100 mg every 8–12 h or by continuous infusion. The treatment has to be accompanied by sufficient fluid substitution. Replacement of mineralocorticoids in primary adrenal insufficiency is not required before the daily hydrocortisone dose has been tapered below 50 mg. Special attention has to be drawn on secondary adrenal insufficiency ensuing a glucocorticoid therapy in supraphysiologic doses. In this setting adrenal crisis develops when glucocorticoid treatment is tapered or stopped abruptly. Lacking controlled clinical studies dosage reduction of glucocorticoids is generally guided by empiricism and requires careful clinical observation.
Zusammenfassung
Die akute Nebennierenrindeninsuffizienz (NNR-Insuffizienz) oder adrenale Krise ist die Folge eines vital bedrohlichen Mangels an Glukound Mineralokortikoiden. Ursächlich liegt in der Mehrzahl der Fälle eine primär adrenale Störung zugrunde, seltener entwickelt sich die Erkrankung sekundär bei einer hypophysären oder hypothalamischen Läsion. Pathophysiologisch dominierend für die Entwicklung des Kreislaufversagens ist der Mangel an Mineralokortikoiden. Gleichzeitig ist aber auch das Fehlen der mineralokortikoiden Eigenwirkung der körpereigenen Glukokortikoide von Bedeutung. Dass Glukokortikoide unabdingbar für die Wirksamkeit von Katecholaminen sind, wurde erst in jüngerer Zeit erkannt und erklärt das schlechte Ansprechen der unbehandelten NNR-Insuffizienz auf die Gabe von Katecholaminen. Diagnostisch hinweisende Symptome wie Müdigkeit, Abdominalschmerzen, Übelkeit und Fieber sowie charakteristische Laborveränderungen wie Hyponatriämie, Hyperkaliämie und Hypoglykämie ergeben sich aus der Kombination der verschiedenen Hormonmangelsituationen. Die Diagnosesicherung mittels Cortisol-, ACTH-Bestimmung und ACTH-Kurztest kann vor Therapieeinleitung in aller Regel nicht abgewartet werden. Basis der Behandlung ist die sofortige Bolusinjektion von 100 mg Hydrocortison, gefolgt von einer hochdosierten Bolus- oder Dauerinfusion (100 mg Hydrocortison alle 8–12 h) und Volumenersatz bis zur klinischen Stabilisierung. Die an dritter Stelle stehende Substitution von Mineralokortikoiden ist nur bei primärer NNR-Insuffizienz und dabei erst nach Absenken der Hydrocortisondosis auf <50 mg/d erforderlich. Selten kann es auch unter einer supraphysiologischen Glukokortikoidtherapie zum Auftreten einer adrenalen Krise kommen, insbesondere wenn die Behandlung abrupt abgesetzt oder rasch ausgeschlichen wird. In Ermangelung klinischer Studien dienen hier empirisch gewonnene Modelle und eine engmaschige klinische Verlaufskontrolle der Prophylaxe dieser vermeidbaren Nebenwirkung.
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Serie: Die Notfall- und Intensivtherapie bei endokrinen und metabolischen Erkrankungen Herausgegeben von J. Schölmerich und Ulrike Woenckhaus (Regensburg)
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Woenckhaus, U., Vasold, A. & Bollheimer, L.C. Akute Nebenniereninsuffizienz („Addison-Krise“). Intensivmed + Notfallmed 42, 345–354 (2005). https://doi.org/10.1007/s00390-005-0546-4
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DOI: https://doi.org/10.1007/s00390-005-0546-4
Key words
- Adrenal insufficiency
- adrenal crisis
- hypothalamic-pituitary-adrenal axis
- corticotropin stimulation test
- treatment