Hypercorticism syndrome

In 1912, this syndrome was described by the American physician Harvey Cushing , who called it English. polyglandular syndrome ^[1] , and independently of it in 1924, the Odessa neurologist Nikolai Mikhailovich Itsenko . Summing up his observations in 1932, Cushing published the work “Basophilic pituitary adenomas and their clinical manifestations” ^[2] .

Cushing's syndrome can be caused by various conditions. Most often, hypercorticism syndrome (excessive formation of hormones of the adrenal cortex) is caused by increased production of the adrenocorticotropic hormone of the pituitary gland ( Itsenko-Cushing's disease ). This hormone can be produced by the pituitary microadenoma or ectopic (not located in the usual place) corticotropinoma . Ectopic malignant corticotropinoma can be located in the bronchi , testicles , ovaries .

Less commonly, Cushing's syndrome occurs with primary damage to the adrenal cortex ( benign or malignant tumors of the adrenal cortex, hyperplasia of the adrenal cortex). A hormone-active tumor of the adrenal cortex is called a corticosteroma . It produces an excessive amount of glucocorticoids in the blood. Moreover, due to the excessive amount of glucocorticoids in the blood, the amount of adrenocorticotropic hormone of the pituitary gland decreases and the remaining adrenal tissue undergoes atrophic changes.

Hypercorticism syndrome can occur in the treatment of various diseases using hormones of the adrenal cortex (glucocorticoids) if an overdose of the drug occurs. Quite often, cortisol hypersecretion is observed in obesity, chronic alcohol intoxication, pregnancy, and some mental and neurological diseases - a condition called “Pseudo-Cushing's syndrome” or “functional hypercorticism” that is not caused by tumors, but the clinical picture is observed as with true Itsenko’s syndrome - Cushing.

The basis of the pathogenesis of pathological changes that develop with Cushing's syndrome on the part of most organs and systems is primarily the overproduction of cortisol. Cortisol in non-physiological concentrations hasthe catabolic effect on protein structures and matrices of most tissues and structures (bones, muscles, including smooth and myocardium, skin, internal organs, etc.), in which pronounced dystrophic and atrophic changes gradually develop. Disorders of carbohydrate metabolism consist in the persistent stimulation of gluconeogenesis and glycogenolysis in the muscles and liver, which leads to hyperglycemia (steroid diabetes). Fat metabolism changes in a complex way: excess deposits occur in some parts of the body, and atrophy of fatty tissue in others, which is explained by the different sensitivity of individual fat sections to glucocorticoids. An important component of the pathogenesis of Cushing's syndrome are electrolyte disorders (hypokalemia, hypernatremia), which are caused by the effect of an excess of aldosterone on the kidneys. A direct consequence of these electrolyte shifts is arterial hypertension and aggravation of myopathy, primarily cardiomyopathy, which leads to the development of heart failure and arrhythmias. The immunosuppressive effect of glucocorticoids causes a tendency to infections.

The most common variant of Cushing's syndrome, which is faced by doctors of most specialties, is exogenous Cushing's syndrome, which develops during glucocorticoid therapy. ^[3] Persons aged 20–40 years are mainly affected.

Hypercorticism syndrome is characterized by typical manifestations:

• the metabolism of proteins, fats and carbohydrates is disturbed;
• protein breakdown is activated, an excess amount of free fat in the blood is formed, the amount of glucose in the patient’s blood rises, which can lead to the development of steroid diabetes .

Manifestations of Cushing's disease are caused by excessive secretion of hormones of the adrenal cortex, primarily glucocorticoids .

The earliest sign of the disease is obesity , with a typical deposition of fatty tissue in the face, neck, chest, and abdomen. The face at the same time looks rounded, moon-shaped. A purple blush appears on the cheeks.

Glucocorticoids cause an increase in appetite and change metabolism, increasing blood glucose and increased fat deposition. The limbs, on the contrary, become thin due to a decrease in muscle mass.

On the skin in the abdomen, shoulder girdle, buttocks and hips appear crimson-cyanotic stretch bands - striae . This is the result of stretching the skin with excessive deposition of fats and impaired protein metabolism, which makes the skin thin and easily stretched. Acne appears on the skin . Wounds and cuts heal slowly.

In places of friction in the collar area on the neck, in the abdomen, elbows, enhanced coloring of the skin occurs. In these places, melanin is deposited, the amount of which increases with the amount of adrenocorticotropic hormone.

In women, hirsutism (excessive hair growth) occurs. Hair appears on the upper lip, chin, chest. Excessive hair growth in women is due to increased production of male androgen hormones androgens by the adrenal cortex, which in women also causes menstrual irregularities. In men, impotence occurs.

At first, a periodic, then a constant increase in blood pressure appears. Bone tissue rarefaction gradually develops - osteoporosis , which is manifested first by pain in the bones and joints, then spontaneous fractures of the ribs and limbs can occur. Excess glucocorticoids both cause a violation of the formation of the protein base of bones, and enhances the release of calcium from bone tissue.

Patients complain of weakness, headaches, weight gain. Sometimes patients with a disease with hypercorticism experience mental disorders . Depression , various sleep disorders, psychoses appear.

The goal of treatment is to normalize the level of hormones in the adrenal cortex in the blood. Used drugs that reduce the production of hormones in the adrenal cortex - ketoconazole , mamomite - for a long time.

Symptomatic treatment is carried out aimed at correcting disorders of protein and carbohydrate metabolism, normalizing blood pressure levels, and treating heart failure. If the patient has a corticosteroma, unilateral adrenal gland removal is performed (unilateral adrenalectomy ).

• Itsenko - Cushing's disease
• Pituitary Adenoma
• Adrenal adenoma
• Nelson's syndrome
• Corticotropinoma

• Itsenko-Cushing's Syndrome: Monograph / Ed. V. G. Baranova , A. I. Nechaya. - M .: Medicine , 1988 .-- 224 p. (in per.)