Acute adrenal insufficiency

Acute adrenal insufficiency is a syndrome that develops as a result of a sharp decrease or complete cessation of hormone production by the adrenal cortex.

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Causes of Acute Adrenal Insufficiency

In children of the first 3 years of life, due to the anatomical and physiological immaturity of the adrenal glands, acute adrenal insufficiency can develop under the influence of even minor exogenous factors (stress, acute respiratory viral infections, infectious diseases, etc.). The development of acute hypocorticism is accompanied by such conditions as congenital dysfunction of the adrenal cortex, chronic adrenal insufficiency, bilateral hemorrhage in the adrenal glands, including Waterhouse-Friderichsen syndrome in meningococcemia.

Acute adrenal insufficiency is observed in autoimmune adrenalitis, adrenal vein thrombosis, congenital adrenal tumors, tuberculosis, herpes, diphtheria, cytomegalovirus, toxoplasmosis, listeriosis. Acute hypocorticism can be caused by treatment with anticoagulants, adrenalectomy, acute pituitary insufficiency, and withdrawal of glucocorticosteroids. In the neonatal period, hypocorticism is a consequence of birth trauma to the adrenal glands, usually during breech delivery.

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Pathogenesis of acute adrenal insufficiency

In acute adrenal insufficiency, due to the lack of synthesis of gluco- and mineralocorticoids, there is a loss of sodium and chloride ions, a decrease in their absorption in the intestine, which leads to dehydration and a secondary transition of water from the extracellular space into the cell. Due to sharp dehydration, the volume of circulating blood decreases and shock develops. The concentration of potassium in the blood serum, in the intercellular fluid and in the cells increases and leads to a violation of the contractility of the myocardium.

In the absence of glucocorticoids, hypoglycemia develops, glycogen reserves in the liver and muscles decrease. A decrease in the filtration and reabsorption functions of the kidneys is characteristic.

In Waterhouse-Friderichsen syndrome, bacterial shock develops, leading to acute vascular spasm, necrosis and hemorrhage in the cortex and medulla of the adrenal glands. Adrenal lesions can be focal and diffuse, necrotic and hemorrhagic.

Symptoms of Acute Adrenal Insufficiency

Initial symptoms of acute adrenal insufficiency: adynamia, muscle hypotonia, reflex inhibition, pallor, anorexia, decreased blood pressure, tachycardia, oliguria, non-localized abdominal pain of varying intensity, including acute abdomen syndrome. Without treatment, hypotension progresses rapidly, signs of microcirculation disorders appear in the form of acrocyanosis, "marbling" of the skin. Heart sounds are muffled, the pulse is threadlike. Vomiting and frequent loose stools occur, leading to exsicosis and anuria.

The clinical picture, including coma, develops quite suddenly, sometimes without any prodromal phenomena (bilateral hemorrhage in the adrenal glands of various origins, glucocorticosteroid withdrawal syndrome). Addison's disease (fulminant form) manifests itself much less frequently, and central forms of adrenal insufficiency manifest themselves extremely rarely. Acute hypocorticism against the background of an infectious disease is accompanied by the development of severe cyanosis, dyspnea, convulsions, and sometimes petechial rash on the skin.

Decompensation of chronic adrenal insufficiency is characterized by gradual, over a week or more, increase in pigmentation of the skin, general weakness, loss of appetite, nausea, frequent vomiting, abdominal pain. Adynamia, severe depression, prostration develop, and with increasing cardiovascular insufficiency the patient falls into a coma.

Diagnosis of acute adrenal insufficiency

The development of adrenal insufficiency is likely in any acutely ill child, especially at an early age, with symptoms of shock, collapse, and a rapid pulse of weak filling. The disease is also possible in children with signs of malnutrition, developmental delays, hyperthermia, hypoglycemia, and convulsions.

Chronic adrenal insufficiency is characterized by hyperpigmentation in the area of extensor and large folds, external genitalia, along the white line of the abdomen, areolae. Differential diagnosis is carried out with intestinal infections, poisoning, comatose states of various origins, acute surgical diseases of the abdominal organs, pyloric stenosis. Regurgitation and vomiting from the first days of life are possible with disaccharidase deficiency, glucose-galactose malabsorption, CNS pathology of hypoxic, traumatic or infectious origin, salt-wasting form of adrenogenital syndrome. Abnormal, and especially hermaphroditic, structure of the genitals should always be a reason for excluding various variants of congenital dysfunction of the adrenal cortex. Ineffectiveness of infusion therapy with vasopressors in children with acute diseases usually indicates the adrenal nature of the crisis.

Minimal diagnostic testing for suspected acute adrenal insufficiency includes determination of serum electrolytes (hyponatremia, hypochloremia, hyperkalemia). Hyponatremia and hyperkalemia, according to rapid electrolyte analysis, are characteristic of mineralocorticoid deficiency, and isolated hyponatremia may be one of the manifestations of glucocorticoid deficiency.

The characteristic hormonal profile in acute adrenal insufficiency is a decrease in serum cortisol and/or aldosterone levels, as well as serum 17-hydroxyprogesterone levels. ACTH levels are elevated in primary hypocorticism and decreased in secondary hypocorticism. In addition, low levels of 17-OCS and 17-KS are noted in daily urine.

On the ECG with hyperkalemia - ventricular extrasystole, splitting of the P wave, biphasic T wave with the first negative phase. Ultrasound of the adrenal glands reveals the presence of hemorrhages or hypoplasia.

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Differential diagnostics

Differential diagnosis is carried out with comatose states of various origins, acute surgical diseases of the abdominal organs.

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Treatment of acute adrenal insufficiency

Treatment of acute adrenal insufficiency is carried out in the intensive care unit. To correct hypoglycemia and salt loss, 0.9% sodium chloride and 5% glucose solution are administered - for children under 1 year old in a ratio of 1:1, for children over 1 year old - a physiological solution containing 5% glucose. At the same time, a water-soluble preparation of hydrocortisone is administered intravenously by drip at 10-15 mg / kg of body weight per day. Half of the daily dose can be administered at one time, then half the dose is distributed evenly throughout the day.

It should be remembered that excessive administration of sodium-containing drugs in combination with high doses of mineralocorticoids can contribute to the development of cerebral and pulmonary edema, arterial hypertension with intracranial hemorrhage. In this regard, careful monitoring of arterial pressure and sodium ion concentration in blood plasma is necessary.

Insufficient glucose administration against the background of high doses of glucocorticosteroids contributes to the development of metabolic acidosis.

Emergency care for acute adrenal insufficiency

In case of pronounced clinical signs of exsicosis, it is necessary first of all to carry out infusion therapy in the volume of age-related daily requirement. The rate of administration of infusion solutions (0.9% sodium chloride solution and 5-10% glucose solution - in a ratio of 1:1, plasma, albumin 10%) is mediated by the values of arterial pressure and in case of shock hemodynamic disorders varies from 10-20 to 40 ml / (kg h) or more, with the connection, if necessary, of vasopressors and inotropic support drugs: mesaton 10-40 mcg / kg h min), dobutamine or dopamine from 5-8 mcg / (kg h min) to 15 mcg / (kg h min) in young children, as well as epinephrine - 0.1-1 mcg / kg h min).

In case of severe acidosis (pH <7.2), sodium bicarbonate solution (taking into account BE) is administered under the control of acid-base balance parameters. In acute adrenal insufficiency, the administration of any infusion media containing potassium is contraindicated. As soon as oral rehydration becomes possible, infusion therapy is stopped.

The starting daily dose of hydrocortisone succinate (Solu-Cortef) is 10-15 mg/kg, prednisolone - 2.5-7 mg/kg.

During the first day, treatment is carried out according to the following scheme:

• 10% glucose solution - 2-4 ml/kg (at glycemia level <3 mmol/l);
• 0.9% sodium chloride solution - 10-30 ml/kg;
• hydrocortisone intravenously (50 mg in 50 ml of 0.9% sodium chloride solution): 1 ml/h - newborns, 2 ml/h - preschool children, 3 ml/h - schoolchildren;
• hydrocortisone intramuscularly (2-3 injections): 12.5 mg - for children under 6 months, 25 mg - from 6 months to 5 years, 50 mg - for patients 5-10 years old, 100 mg - over 10 years old.

On the second day:

• hydrocortisone is administered intramuscularly - 50-100 mg (2-3 injections);
• deoxycorticosterone acetate (deoxycortone), intramuscularly once - 1-5 mg.

The transition from intravenous to intramuscular administration is possible immediately after the disappearance of microcirculatory disorders. Subsequently, in the absence of clinical and laboratory signs of acute adrenal insufficiency, the dose is reduced by 30-50% with a simultaneous decrease in the frequency of injections - every 2-3 days up to the maintenance dose, followed by a transfer to enteral drugs in equivalent doses. When using prednisolone, the enteral mineralocorticoid 9-fluorocortisone is prescribed in age-appropriate doses immediately after vomiting has ceased. If hydrocortisone is administered, 9-fluorocortisone is usually prescribed only after the maintenance dose of hydrocortisone has been reached. In Waterhouse-Friderichsen syndrome, despite the severity of the condition, adrenal insufficiency, as a rule. transient, and there is no mineralocorticoid deficiency, therefore only glucocorticosteroids are used for 1-3 days, focusing on the state of hemodynamics.

Replacement therapy for chronic hypocorticism is carried out for life: prednisolone 5-7.5 mg/day, fludrocortisone (cortineff) 50-100 mcg/day (in the absence of hypertension and/or hypokalemia).

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