Zusammenfassung
Das hypophysäre Koma ist ein seltener Notfall und wird durch die kortikotrope (ACTH) und thyreotrope (TSH) Insuffizienz dominiert. Ein hypophysäres Koma entsteht häufiger durch die insuffiziente Substitution einer bekannten Hypophyseninsuffizienz als durch einen unbekannten Hypophysentumor. Leitsymptome sind neben dem stuporösen bis komatösen Zustand eine Hypotonie, Bradykardie, Hautblässe ("alabasterfarbig"), Muskelschwäche, Übelkeit, Erbrechen, Obstipation, Hypothermie und Hypoventilation. Ein postpartal ausbleibender Milchfluss (Agalaktie) kann bereits das erste Symptom eines Sheehan-Syndroms sein. Im Gegensatz zur primären Nebennierenrindeninsuffizienz (M. Addison) fehlen beim hypophysären Koma die Hyperpigmentation der Haut und Schleimhäute, Hyperkaliämie und Salzverlust. Bei Verdacht auf ein hypophysäres Koma ist unverzüglich die Therapie mit 100 mg Hydrokortison i.v., 100 mg Hydrokortison über 24 h, anschließend 500 µg Levothyroxin i.v. sowie eine Flüssigkeitssubstitution einzuleiten. Erst nach der klinischen Rekompensation wird der ACTH-Test, der CRH-Test und ggf. der Insulinhypoglykämietest durchgeführt, um die hypophysären Partialfunktionen detailliert beurteilen zu können.
Abstract
Pituitary coma is a rare case of emergency and primarily due to ACTH and TSH deficiency. Pituitary coma occurs more often in patients with well-known pituitary deficiency than in patients with intrasellar tumor. Clinical manifestations are hypotonia, bradycardia, decreased skin and nipple pigmentation, muscle weakness, vomitus, nausea, obstipation, hypothermia, and hypoventilation. A postpartal agalactia is often the first sign of Sheehan's syndrome. Unlike primary adrenal insufficiency (Addison's disease) ACTH deficiency does not cause hyperpigmentation, hyperkaliemia, or salt loss. The suspicion of pituitary coma requires replacement with 100 mg hydrocortisone iv, 200 mg hydrocortisone iv/24 h, 500 µg levothyroxine iv and fluid substitution. Since thyroxine accelerates the degradation of cortisol and can precipitate adrenal crisis in patients with limited pituitary reserve, hydrocortisone replacement should always precede levothyroxine therapy. ACTH stimulation test, CRH stimulation test and insulin tolerance test (optional) should be performed after therapeutic compensation to determine pituitary function.
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Herrmann, B.L., Mann, K. Das hypophysäre Koma. Internist 44, 1253–1259 (2003). https://doi.org/10.1007/s00108-003-1047-9
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DOI: https://doi.org/10.1007/s00108-003-1047-9