Chronic adrenal insufficiency

Adrenal insufficiency (synonym: hypocorticism) - reduction of glucocorticoid and mineralocorticoid functions of the adrenal glands is one of the most serious diseases of the endocrine system.

Isolate acute and chronic adrenal insufficiency. Distinguish primary hypocorticism, caused by direct damage to the cortical layer of the adrenal glands, and secondary hypocorticism associated with a pituitary or hypothalamic defect, accompanied by a deficiency of ACTH.

ICD-10 code

• E27.1 Primary insufficiency of the adrenal cortex.
• E27.3 Medication adrenal insufficiency.
• E27.4 Other and unspecified insufficiency of the adrenal cortex.

Causes of chronic adrenal insufficiency

The main cause of primary hypocorticism is the autoimmune destruction of the adrenal cortex. The appearance of autoantibodies to antigens of adrenal cells is often accompanied by production of other organ-specific autoantibodies, which leads to the development of polyendocrine syndrome - a combination of chronic adrenal insufficiency with autoimmune thyroiditis, diabetes mellitus, hypoparathyroidism, pernicious anemia, vitiligo, candidiasis. Another cause of primary adrenal involvement is tuberculosis, although tuberculosis etiology is less common in children than in adults. Sometimes primary hypokorticism can be caused by congenital hypoplasia of the adrenal cortex, a genetically determined disease with a recessive type of inheritance linked to the X chromosome (found only in boys).

The most common cause of secondary hypocorticism is destructive processes in the hypothalamic-pituitary system (tumor, trauma, infection).

Due to the lack of glucocorticoids, which ensure the activation of gluconeogenesis in a healthy organism, the glycogen reserves in muscles and liver decrease, the glucose content in blood and tissues decreases. Reducing the intake of glucose in the tissue leads to adynamia and muscle weakness. The lack of mineralocorticoids causes an increase in the excretion of sodium, chlorides and water, which leads to hyponatremia, hyperkalemia, dehydration and a decrease in blood pressure. Deficiency of adrenal androgens, accompanying adrenal insufficiency, is manifested by a delay in growth and sexual development. At the same time, the intensity of anabolic processes in bone and muscle tissues decreases. Clinical signs of chronic adrenal insufficiency are manifested when 90% of glandular cells are destroyed.

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Symptoms of chronic adrenal insufficiency

Symptoms of chronic adrenal insufficiency are primarily due to insufficiency of glucocorticoids. Congenital forms of hypocorticism appear from the first months of life. With autoimmune adrenalitis, the onset of the disease occurs more often after 6-7 years. Characteristic lack of appetite, weight loss, lower blood pressure, asthenia. Often noted pain in the abdomen, nausea, causeless vomiting.

Hyperpigmentation of the skin - pathognomonic clinical symptom of primary hypocorticism. Intensively colored natural folds of the skin, places of contact with clothing. Hyperpigmentation is caused by excessive secretion of ACTH and melanocyte-stimulating hormone. With secondary hypokorticism, there is no hyperpigmentation.

In some cases, hypoglycemic conditions develop, due to the lack of counterinsurgency of glucocorticoids.

With congenital chronic adrenal insufficiency, signs of the disease appear soon after birth. Characterized by rapid loss of body weight, hypoglycemia, anorexia, regurgitation. Typical pigmentation of the nipples, white belly line, external genitalia on the background of pale skin.

Classification of chronic adrenal insufficiency

I. Primary insufficiency of the adrenal cortex (Addison's disease)

• Congenital.
  • Congenital hypoplasia of the adrenal cortex.
  • Hypoaldosteronism.
  • Adrenoleukodystrophy.
  • Family isolated glycocorticoid deficiency.
  • Syndrome Allgrove.
• Acquired.
  • Autoimmune adrenaline.
  • Infectious adrenalitis (tuberculosis, syphilis, mycoses).
  • Amyloidosis.
  • Metastases of malignant tumors.

II. Secondary insufficiency of the adrenal cortex

• Congenital.
  • Isolated corticotropin deficiency.
  • Hypopituitarism.
• Acquired.
• Destructive damage to the pituitary gland (tumors, hemorrhage, infections, autoimmune hypophysitis).

III. Tertiary insufficiency of the adrenal cortex

• Congenital.
• Isolated insufficiency of corticoliberin.
• Multiple failure of the hypothalamus.
• Acquired.
• Destructive damage to the hypothalamus.

IV. Disorders of the reception of steroid hormones

• Pseudohypoaldosteronism.
• Iatrogenic insufficiency of the adrenal cortex.

[3], [4]

Complications of chronic adrenal insufficiency

Without treatment, the symptoms of chronic adrenal insufficiency rapidly increase, and the adrenal insufficiency crisis develops, characterized by symptoms of acute adrenal insufficiency. There is a sharp weakness, a drop in blood pressure, vomiting, loose stool, abdominal pain. There are clonic-tonic convulsions and meningeal symptoms. There are symptoms of dehydration, cardiovascular insufficiency. If untimely or inadequate treatment, a lethal outcome is possible.

[5], [6], [7]

Diagnosis of chronic adrenal insufficiency

The main diagnostic criterion of hypocorticism is a decrease in the content of cortisol and aldosterone in the blood serum. In the initial hypocorticism, low levels of cortisol and aldosterone are accompanied by an increase in the content of ACTH and renin in the blood plasma.

Deficiency of glucocorticoids leads to hypoglycemia. Mineralocorticoid insufficiency is characterized by hyperkalemia and hyponatremia.

With the erased forms of chronic adrenal insufficiency, a stimulation test with ACTH is carried out: after blood sampling for the determination of the basal level of cortisol, the drug AKTG of prolonged action of tetracosactides or fast action of syncorpine, corticotropin is introduced at 8:00 in the morning. Then, the level of cortisol is repeatedly determined 12-24 hours after the administration of tetracosactide or 60 minutes after corticotropin. In healthy children, the content of cortisol after the introduction of ACTH is 4-6 times higher than the basal level. The lack of response to stimulation indicates a decrease in the reserves of the adrenal cortex.

[8], [9], [10], [11]

Differential diagnostics

Differential diagnosis of chronic adrenal insufficiency should be carried out with neurocirculatory dystonia according to the hypotonic type, essential arterial hypotension. The combination of arterial hypotension with weight loss can be with peptic ulcer of stomach, anorexia nervosa, oncological pathology. In the presence of hyperpigmentation, differential diagnosis is performed with dermatomyositis, scleroderma, pigment-papillary dystrophy of the skin, poisoning with salts of heavy metals.

[12], [13], [14], [15]

Treatment of chronic adrenal insufficiency

Treatment of the crisis is aimed at eliminating electrolyte disorders and hypoglycemia. Infusion therapy includes 0.9% sodium chloride solution and 5% glucose solution. The total volume of fluid is calculated on the basis of physiological needs, taking into account the losses.

At the same time begin substitution therapy - preference is given to glucocorticosteroids with mineralocorticoid activity, for example, hydrocortisone. The drug is administered fractionally within a day intramuscularly; in severe cases, water-soluble preparations of hydrocortisone are administered intravenously. Subsequently, after arresting the crisis of adrenal insufficiency, patients are transferred to constant replacement therapy with tableted preparations (cortef, cortineff). Adequacy of treatment is assessed by the parameters of physical and sexual development, blood pressure, electrolyte level in blood plasma, ECG.

Forecast

With adequate replacement therapy, the prognosis for life is favorable. With intercurrent illnesses, injuries, stressful situations, there is a risk of developing a crisis of adrenal insufficiency. In cases of presumed risk, you should increase the daily dose of glucocorticosteroids 3-5 times. During the surgical intervention, the drugs are administered parenterally.

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