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.

[1], [2], [3], [4], [5], [6],

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 with the influence of even minor exogenous factors (stresses, acute respiratory 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 to the adrenal glands, including the Waterhouse-Frideriksen syndrome in meningococcemia.

Acute adrenal insufficiency is observed in autoimmune adrenal, adrenal thrombosis, congenital adrenal tumors, tuberculosis, herpes, diphtheria, cytomegaly, toxoplasmosis. Listeriosis. Acute hypocorticism can result in treatment with anticoagulants, adrenalectomy, acute pituitary insufficiency, elimination of glucocorticosteroids. During the newborn period, hypocorticism is a consequence of the birth trauma of the adrenal glands, as a rule, during birthing in the pelvic presentation.

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Pathogenesis of acute adrenal insufficiency

In acute adrenal insufficiency, because of the lack of synthesis of gluco- and mineralocorticoids, loss of sodium and chloride ions occurs, a decrease in their absorption in the intestine, which leads to dehydration and a secondary transition of water from extracellular space into the cell. In connection with severe 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 stores in the liver and muscles decrease. Characteristic decrease in filtration and reabsorption functions of the kidneys.

With the Waterhouse-Frideriksen syndrome, a bacterial shock develops, leading to acute vascular spasm, necrosis, and hemorrhage into the cortical and cerebral layers of the adrenal glands. Lesions of the adrenal glands can be focal and diffuse, necrotic and hemorrhagic.

Symptoms of acute adrenal insufficiency

Initial symptoms of acute adrenal insufficiency: adynamia, muscle hypotension, oppression reflexes, pallor, anorexia, lowering blood pressure, tachycardia, oliguria, non-localized abdominal pain of varying intensity, including acute stomach syndrome. Without treatment, hypotension progresses rapidly, signs of microcirculation disorders appear in the form of acrocyanosis, the "marbling" of the skin. The heart sounds are deaf, the pulse is threadlike. There is vomiting, a frequent loose stool leading to an exsicosis and anuria.

The clinical picture up to the coma unfolds quite suddenly, sometimes without any prodromal phenomena (bilateral hemorrhage into the adrenal glands of various genesis, the syndrome of glucocorticosteroid withdrawal). Significantly less often manifests addison's disease (fulminant form), extremely rarely - the central forms of adrenal insufficiency. Acute hypocorticism against the background of an infectious disease is accompanied by the development of a sharp cyanosis, dyspnea, convulsions, and sometimes a petechial rash on the skin.

Decompensation of chronic adrenal insufficiency is characterized by gradual, for a week or more, increased pigmentation of the skin, general weakness, loss of appetite, nausea, frequent vomiting, abdominal pain. Developed adynamy, pronounced depression, prostration, 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 of an early age, with phenomena of shock, collapse, a frequent pulse of weak filling. The disease is possible in children with signs of malnutrition, lagging behind in development, with hyperthermia, hypoglycemia and seizures.

Chronic adrenal insufficiency is characterized by hyperpigmentation in the region of extensor and major folds, external genitalia, along the white abdominal line, areola. Differential diagnosis is carried out with intestinal infections, poisonings, coma of various genesis, acute surgical diseases of the abdominal cavity, pyloric stenosis. Regurgitation and vomiting from the first days of life are possible with disaccharidic insufficiency, malabsorption of glucose-galactose, pathology of the hypoxic, traumatic or infectious genesis of the central nervous system, the salt-losing form of adrenogenital syndrome. The wrong, and even more hermaphroditic, structure of the genitals should always be an excuse for excluding various variants of congenital dysfunction of the adrenal cortex. The ineffectiveness of infusion therapy with the use of vasopressors in children with acute diseases usually indicates the adrenal nature of the crisis.

The minimal diagnostic examination for suspected acute adrenal insufficiency includes the determination of serum electrolytes (hyponatremia, hypochloraemia, hyperkalemia). Hyponatremia and hyperkalemia, according to the rapid analysis of electrolytes, are characteristic of mineralocorticoid insufficiency, and isolated hyponatremia may be one of the manifestations of glucocorticoid insufficiency.

The characteristic hormonal profile in acute adrenal insufficiency: a decrease in the level of cortisol and / or aldosterone in the blood serum, as well as the level of 17-hydroxyprogesterone in the blood serum. The content of ACTH is increased with primary hypocorticism and decreased - in the secondary. In addition, daily urine is marked by a low level of 17-ACS and 17-CS.

On the ECG with hyperkalaemia - ventricular ekstrasistoliya, splitting of the tooth P, a two-phase tooth T 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 different genesis. Acute surgical diseases of the abdominal cavity.

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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 loss of salt, sodium chloride 0.9% and 5% glucose solution are administered to children under 1 year in a ratio of 1: 1, children older than one year - saline containing 5% glucose. At the same time, a water-soluble hydrocortisone preparation is dripped intravenously at a dose of 10-15 mg / kg of body weight per day. You can enter half the daily dose at one time, then distribute half the dose evenly throughout the day.

It should be remembered that the excessive introduction of drugs containing sodium, combined with high doses of mineralocorticoids, can promote the development of cerebral edema, lungs, arterial hypertension with intracranial hemorrhages. In this regard, careful monitoring of blood pressure and concentration of sodium ions in blood plasma is necessary.

Insufficient injection of glucose against the background of high doses of glucocorticosteroids promotes the development of metabolic acidosis.

Emergency care for acute adrenal insufficiency

With the expressed clinical signs of exsicosis, first of all it is necessary to conduct the infusion therapy in the volume of the age-related daily requirement. The rate of administration of infusion solutions (0.9% sodium chloride solution and 5-10% glucose solution - 1: 1 ratio, plasma, albumin 10%) is mediated by blood pressure values and for shock disorders of hemodynamics varies from 10-20 to 40 ml / (kghh) or more, with the inclusion of vasopressors and inotropic support in cases of need: 10-10 μg / kghmin mezaton), dobutamine or dopamine from 5-8 μg / kg kg to 15 μg / kg kgmin in young children, as well as epinephrine - 0.1-1 μg / kghmin).

In the case of severe acidosis (pH <7.2), under the control of the acid-base state indicators, a solution of sodium bicarbonate (with BE taken into account) is introduced. In acute adrenal insufficiency, the administration of any infusion medium containing potassium is contraindicated. As soon as there is a possibility of oral rehydration, infusion therapy is stopped.

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

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

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

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. In the future, 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 number of injections - every 2-3 days up to a maintenance dose, with subsequent transfer to enteric drugs in equivalent doses. When using prednisolone, enteric mineralocorticoid 9-fluorocortisone is prescribed in the dose levels immediately after cessation of vomiting. If hydrocortisone is administered, then 9-fluorocortisone is prescribed, as a rule, only after reaching a maintenance dose of hydrocortisone. With Waterhouse-Frideriksen syndrome, despite the severity of the condition, adrenal insufficiency, as a rule. Transient, and there is no mineralocorticoid deficiency, so only glucocorticosteroids are used for 1-3 days, focusing on the state of hemodynamics.

Substitution therapy for chronic hypokorticism 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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