Itenko-cushing's disease

Itenko-cushing's disease is characterized by impaired function of the hypothalamic-pituitary-adrenal system and symptoms of increased production of corticosteroid hormones. It is observed at any age, but more often in 20-40 years; Women suffer 10 times more often than men.

Etiology. Tumors of the pituitary gland (micro- and macroadenomas), inflammatory processes of the brain; In women often develops after childbirth. Tumors of the adrenal gland (glucosteroids, glucoandrosteromas), tumors of the lungs, bronchi, mediastinum, pancreas secreting ACTH, the syndrome of ectopic ACTH produce Cushing's syndrome characterized by similar clinical symptom-complexes.

Pathogenesis. Violation of the dopaminergic and serotonergic mechanism of ACTH secretion, increased production of ACTH by the pituitary and corticosteroids by the adrenal glands.

Symptoms, course. Excessive fat deposition in the face, neck, trunk area. The face becomes lunate. The limbs are thin. The skin is dry, thinned, on the face and in the chest area - purple-cyanotic color. Acrocyanosis. There is a pronounced venous pattern on the chest and extremities, stretch bands on the skin of the abdomen, thighs, inner surfaces of the shoulders. Often there is hyperpigmentation of the skin, often in places of friction. On the skin of the face, the extremities of women have hypertrichosis. Propensity to furunculosis and development of erysipelas. Blood pressure is increased. Osteoporotic changes in the skeleton (in severe cases, there are fractures of the ribs, spine). Steroid diabetes is characterized by insulin resistance. Hypokalemia of varying severity. Steroid myopathy and cardiopathy. The number of erythrocytes, hemoglobin and cholesterol is increased. Mental disorders (depression, euphoria).

There are light, moderate and severe forms of the disease; The course can be progressive (development of all symptoms for 6-12 months) and torpid (symptoms gradually increase over 3-10 years).

The diagnosis is based on the following data: an increase in the content of 17-ACS in daily urine, cortisol and ACTH in blood plasma (most significantly nighttime increase); With macroadenomas of the pituitary gland - an increase in the size of the Turkish saddle; Osteoporosis of the spine and other bones of the skeleton; Given oxysuprasprenography or computed tomography-an increase in both adrenal glands, and in the presence of a tumor of the adrenal gland, its image on the corresponding side is visible; According to adrenal scintigraphy, after the introduction of 19-iodine-cholesterol there is an increase in the contours of two adrenals, in the presence of a tumor - one. Diagnostic tests to exclude a tumor of the adrenal cortex are samples with dexamethasone, methopyron and ACTH: in the presence of an adrenal tumor, the content of 17-ACS in the daily urine does not change with the administration of these substances, and with bilateral hyperplasia, the level of 17-ACS decreases after the administration of dexamethasone (more than 50%) and increases after the introduction of ACTH and metopyrone (2-3 times from the baseline level).

Treatment. With a light and moderate current, radiation therapy of the interstitial-pituitary region (gamma-therapy or proton therapy) is performed; After the gamma therapy prescribe reserpine - 1 mg / day (4-6 months). In the absence of the effect of radiotherapy, one adrenal gland is removed or a course of treatment with chloditan (inhibitor of biosynthesis of hormones in the adrenal cortex) in combination with the administration of reserpine, parlodel, diphenin, and peritol.

In severely ill patients, bilateral adrenalectomy is used; After the operation chronic adrenal insufficiency develops, which requires constant replacement therapy.

Symptomatic therapy is aimed at compensating protein (anabolic steroids), mineral (potassium, verospilone) and carbohydrate (biguanide in combination with insulin preparations) metabolism; Antihypertensive drugs (reserpine), diuretics, cardiac glycosides.