The main clinical manifestation is hypotension with poor or no response to rehydration and antihypertensive drugs, in which case a high degree of suspicion of critical illness is required. Several factors contribute to the development of hypotension, including corticosteroid deficiency leading to reduced myocardial contractility; diminished vascular response to catecholamines; and aldosterone deficiency leading to hyponatremia and hypovolemia. Hypovolemia is more pronounced in primary than secondary adrenal insufficiency. Patients often have elevated renin and ADH due to volume deficit stimulation. Life-threatening to acute adrenal insufficiency are hypotension and hypoglycemia, which responds well to 5%-10% glucose.
This is followed by severe infections, adrenal insufficiency may occur in more than 50% of patients in septic shock, and systemic sepsis combined with extensive hemorrhagic infarction of the adrenal body, especially in meningococcal sepsis. In addition, adrenal hemorrhage is caused by trauma, anticoagulation therapy, tumor metastasis to the adrenal glands, heparin-induced thrombocytopenia, and antiphospholipid antibody syndrome. Adrenal insufficiency due to adrenal hemorrhage is fatal, and these patients often have severe systemic disease (e.g., heart attack, systemic infection, and burns) in combination with anticoagulant therapy.
Other causes of acute adrenocortical insufficiency are pituitary strokes, which present as severe headaches with neuro-ocular signs and symptoms, along with psychiatric changes. Most patients with pituitary stroke have a previous history of pituitary tumor. Postpartum hemorrhage with pituitary necrosis presents with total hypopituitarism and hyponatremia.
Electrolyte abnormalities: 88% of patients present with hyponatremia, 64% with hyperkalemia, 92% with hyponatremia or hyperkalemia, and 6%-33% with hypercalcemia. There are several mechanisms of hyponatremia: secondary to volume reduction or cortisol reduction, resulting in elevated DAH; reduced glomerular filtration resulting in reduced distal tubular filtrate, reducing sodium reabsorption; and finally, if there is concomitant aldosterone deficiency, resulting in increased urinary sodium loss. If the patient is on a high sodium diet, he or she is clinically asymptomatic; if the patient is on a low sodium diet, he or she will show clinical signs of volume deficiency.
Hyperkalemia comes from acidosis, aldosterone deficiency, and reduced glomerular filtration rate. Note that hyperkalemia causes heart rate disturbances. Hypercalcemia is relatively rare and the mechanism is unclear; it may be related to increased protein binding or volume deficit with concomitant increased renal tubular reabsorption, increased total calcium, and normal free calcium. Psychiatric symptoms can also be common, including depression, manic psychosis and even generalized convulsions.
Diagnosis
A random plasma cortisol < 18 μg/dL in a stressful situation is highly suggestive of cortical insufficiency. Normal values of plasma cortisol in the early morning (6:00-8:00 am) are 10-20 μg/dL. Adrenocortical insufficiency is excluded if cortisol binding protein (CBG) is normal and blood F 20 μg/dL. More authors now recommend a stimulation test with 1 μg cosyntropin, judged by the same criteria as 250 μg.
Alternatively, insulin hypoglycemia test, regular insulin 0, 1U/kg intravenously, measurement of plasma cortisol and ACTH at 0′, 30′, 60′, 90′, diagnostic criteria are the same as ACTH test.
Key Tip.
Since the clinical manifestations of adrenal crisis are non-specific, crossover with other diseases and there are no specific diagnostic criteria, it is not easy to make a clear clinical judgment. Acute adrenal crisis should be considered in the following cases.
(1) When there is a past history of chronic adrenal insufficiency or sudden discontinuation of long-term corticosteroid use, with sudden onset of fever, anorexia, nausea and vomiting, and abdominal pain
(2) For patients with unexplained shock or coma, after rehydration and antihypertensive drug treatment, the effect is not obvious
(3) Some thrombotic diseases, or anticoagulant treatment, or after major surgery, the condition suddenly worsens, and patients with a drop in blood pressure or shock with chest, abdominal and back pain.
If these patients are combined with hypotension, hypoglycemia, or hyponatremia, the diagnosis is more favorable.
Treatment
Treatment goals are.
(1) glucocorticoid supplementation
(2) Correction of electrolytes, metabolic abnormalities and hypovolemia
(3) Finding and managing the triggering factors
1. Glucocorticoid therapy
If adrenal insufficiency is not clinically established, corticosteroid supplementation is best after completion of the ACTH test. if time does not allow and the ACTH stimulation test is not completed, dexamethasone 4mg q6-8h can be supplemented intravenously. dexamethasone is 100 times more potent than cortisol and there is no crossover between it and cortisol measurement.
If the patient has established adrenal insufficiency, 100 mg hydrocortisone succinate is administered intravenously q6-8 h. If the intravenous route is difficult, cortisone acetate 100 mg is administered intramuscularly, but absorption is unstable and inferior to the intravenous preparation.
During severe stress, the adrenal glands can secrete cortisone 300-400mg/day and return to normal after three days, so three days of high-dose hormone therapy is safe and does not cause endogenous hypocorticism. Once the patient’s condition is stable, hydrocortisone 50-100 mg/day, the dose is gradually reduced, and when the patient can take it orally, it is changed to oral maintenance dose, cortisone 25mg in the morning and 12,5mg in the evening.
2.For the treatment of hypotension
Volume loss is more severe in patients with primary adrenocortical insufficiency than secondary, and volume loss in Addison patients often reaches 20%. If the patient has no specific contraindications to rehydration, the first hour infusion of isotonic saline 1000mL, the first 8 hours requires rehydration 3000mL, preferably with corticosteroid supplementation.
In the treatment of hypotension in patients with adrenal crisis, the effect of antihypertensive drugs alone is less than that of volume supplementation alone, and the best antihypertensive effect is achieved with corticosteroids combined with volume supplementation. Salt corticosteroids are not always necessary if adequate salt and water supplementation is available.
Blood volume and electrolytes need to be monitored during treatment to avoid excessive sodium supplementation by infusion leading to heart failure.
3.Treatment for hypoglycemia
Immediate treatment is needed without waiting. Severe patients need intravenous injection of 50% glucose 50-100mL, or immediate subcutaneous injection of glucagon 1-2mg if the intravenous route is difficult.
4.Correcting electrolyte abnormalities
Hyponatremia is usually corrected with saline. If the blood sodium rises unsatisfactorily after saline infusion and corticosteroid treatment, oral saline corticosteroid, 9a-fluhydrocortisone 0,05-0,2mg/24h can be considered. attention should also be paid to prevent high sodium and excessive rehydration from aggravating the heart burden and heart failure. Special attention should be paid to blood potassium. Symptomatic hyperkalemia needs to be treated with carbonates, insulin or calcium.
5.Other treatment measures
Pay attention to the patient’s combined presence of heart attack, asthma, infection and abnormal mental status. Blood, urine and stool cultures are required, and lumbar puncture is performed if necessary to exclude intracranial infections. Once infection is detected, treatment with antimicrobial agents is required.
Before the patient improves and is discharged, the patient and family need to be warned that the corticosteroid dose needs to be doubled, even up to 3-5 times, when there is high fever, surgery, trauma, and severe diarrhea.