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Symptoms and Treatment of Adrenal Insufficiency and Addisonian Crisis
Definition
Adrenal insufficiency is hypocortisolism due to dysfunction of the adrenal cortex, hypophysis, or hypothalamus with a potentially fatal outcome (Arlt et al., 2003).
Epidemiology
Prevalence 1/40.000, Mortality 0,3–1/100.000.
Etiology
- Diseases of the adrenals (Morbus Addison): after (bilateral) adrenalectomy, autoimmune disease, tuberculosis, adrenal metastases, sepsis. Medications: ketoconazole, mitotane, suramin. Rare causes are sarcoidosis, infections such as histoplasmosis, blastomycosis, or AIDS.
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Diseases of hypophysis and hypothalamus:
- Acute withdrawal from steroids (discontinuation of medication, adrenalectomy).
- Pituitary and hypothalamic diseases: Tumors, sarcoidosis, tuberculosis, radiation, and infarcts.
- Sheehan syndrome: ischemic necrosis of the hypophysis due to heavy postpartum bleeding and shock.
Signs and Symptoms
- Acute adrenal crisis: clinical deterioration, fever, vomiting, abdominal pain, hypotension, lethargy. Synonyms: Addisonian crisis.
- Chronic adrenal insufficiency: fatigue, weight loss, nausea and vomiting, hyperpigmentation of the skin and mucous membranes, abdominal discomfort, diarrhea, salt starvation, and hypotension.
Diagnosis of Adrenal Insufficiency
- Laboratory tests: hyponatremia, hyperkalemia, hypoglycemia. Urine cortisol decreased, serum cortisol decreased, and serum ACTH increased or decreased depending on the cause. Test for autoantibodies against the adrenal cortex.
- ACTH test: inadequate increase in serum cortisol 60 min after administration of ACTH (0.25 mg i.v.).
- CRH test: inadequate increase of ACTH and cortisol after administration of CRH (1 μg/kgKG i.v.).
- Imaging: MRI or CT of the head or abdomen, depending on the suspected diagnosis.
Treatment of Adrenal Insufficiency
Emergency Treatment of Addisonian Crisis:
- Rapid infusion of 0.9% NaCl (2–3 l).
- 100 mg of hydrocortisone is administered i.v. as a bolus, followed by a continuous infusion of another 100 mg over 24 hours.
- Treat hyponatremia.
- After stabilizing the patient, a short ACTH test is necessary to confirm the diagnosis.
Perioperative Substitution Therapy after Bilateral Adrenalectomy:
- OP day: 100 mg of hydrocortisone is administered i.v. as a bolus with begin of anesthesia, followed by a continuous infusion of another 100 mg over 24 hours.
- Day 1–3: hydrocortisone 100 mg/24 h i.v.
- Day 4: hydrocortisone 20-20-10 mg p.o.
- Following days: after uncomplicated surgery, reduce by 10 mg/d until the maintenance dose (hydrocortisone 20-10-0 mg/d) is reached. If hydrocortisone is below 50 mg/d, fludrocortisone 0.1 mg/d is additionally necessary.
- Control of blood pressure, electrolytes, and renin activity.
Substitution Therapy for Chronic Adrenal Insufficiency:
- Hydrocortisone 20-10-0 mg/d
- Fludrocortisone 0.1 mg/d
- An increase in dosage is vital in case of infections or surgery.
- Control of blood pressure, electrolytes and renin activity.
- A steroid emergency card should be given to all patients with primary adrenal insufficiency and those who are on a long-term oral steroid.
Hyperaldosteronism and Conn syndrome | Index | Adrenal incidentaloma |
Index: 1–9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
References
Arlt und Allolio 2003 ARLT, W. ; ALLOLIO, B.:
Adrenal insufficiency.
In: Lancet
361 (2003), Nr. 9372, S. 1881–93
Deutsche Version: Akute Addison Krise und Therapie der Nebenniereninsuffizienz
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