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  • Icenko-Cushing syndrome: symptoms, treatment and causes of the disease

    What is it - the syndrome Itenko-Cushing called a condition in which there is an increased concentration of glucocorticoid hormones in the blood of the adrenal cortex.

    This is not necessarily a disease of the adrenal glands or associated hypothalamus or pituitary gland - most often the syndrome is recorded with long-term administration of large doses of glucocorticoids for the treatment of various autoimmune pathologies.

    Pathology develops more often in women. Depending on the cause of the condition, both conservative and surgical treatment can be used.

    There is also the concept of "Itenko-Cushing's disease" - excessive production of the hypothalamus and pituitary gland of those hormones that "give the command" to the adrenal glands to synthesize more glucocorticoids. Disease is one of the constituent parts of the syndrome.

    Causes of development of Icenko-Cushing syndrome


    The syndrome develops due to internal or external causes.

    1) The external causes of the Itenko-Cushing syndrome are the long-term administration of injectable or tableted glucocorticoids: hydrocorizon, prednisolone, dexamethasone, and solu-medrol. Usually, these drugs are prescribed for the treatment of systemic diseases( rheumatoid arthritis, lupus erythematosus and so on), lung diseases( eg, fibrosis), kidneys( glomerulonephritis), blood( leukemia, thrombocytopathy).


    2) Causes of internal( organismic) nature:

    • is a pituitary tumor that produces the hormone ACTH;
    • adrenal disease: a benign or malignant tumor of the cortex of this organ that additionally produces glucocorticoids;an increase in the cell mass( hyperplasia) of the adrenal cortex, resulting in an increase in the production of all its hormones;
    • tumors in the body, capable of producing ACTH: small cell and ovate cell lung cancer, thymus cancer, neoplasm of testicles or ovaries.

    Mechanism of diseases occurring in the disease


    Glucocorticoid hormones, the level of which is significantly increased in Cushing's syndrome, in the body they act on almost all organs and systems:

    • increase the pigmentation of the skin and mucous membranes;
    • increase protein and carbohydrate metabolism;
    • reduce fat metabolism;
    • is excreted from the body by potassium;
    • increase blood pressure;
    • reduce the ability of bones to accumulate calcium, diluting them;
    • increase the synthesis of male sex hormones;
    • suppress immunity;
    • increase the level of prolactin.

    Symptoms of the Itenko-Cushing syndrome


    Syndrome Itenko-Cushing has bright clinical symptoms, as a rule, the appearance of a person with this disease has the characteristic features:

    • skin and mucous membranes acquire a darker color in places of its friction;
    • develops obesity;
    • the fat on the face is deposited in a special way, that it is like a moon-shaped, the skin on it is crimson-red;
    • fatty tissue deposited on the neck, forming a hump;
    • thus the skin on a back of a palm thin, transparent( there there is no fat);
    • fat is deposited over the clavicles;
    • legs are thin, their muscles are atrophied;
    • developed in children, the syndrome causes growth retardation;
    • on the abdomen, chest, hips appear red-violet stretch marks;
    • on the skin a lot of acne, pustules, vascular asterisks;
    • dark hair above the lip, on the chest, hips, abdomen and back - hirsutism;
    • swelling of the extremities due to a delay in the body of sodium and water.
    Other symptoms of the Itenko-Cushing syndrome are also noted:

    1. 1) Wounds heal for a long time because of deficiency of proteins and suppression of immunity.
    2. 2) Glucocorticoids are insulin antagonists, so when they increase, the concentration of sugar in the blood grows.
    3. 3) Elevated blood pressure figures are noted due to the fact that the cortical hormones "include" also that hormonal system of the kidneys, which is responsible for increasing the pressure.
    4. 4) The capillaries become brittle, which is manifested in the form of rapid bruising at the slightest trauma.
    5. 5) If the cause of Cushing's syndrome is an adenoma of the pituitary gland, and it grows, there is a mechanical compression of the mass of the optic nerves( they pass near the pituitary in the cranial cavity), which is manifested by blindness and headache.
    6. 6) There may be the expiration of milk from the nipples of a non-feeding woman.
    7. 7) Bone fractures after minor injuries - due to osteoporosis.
    8. 8) Disorders from the reproductive system: infertility, impotence, absence or irregularity of menstruation.
    9. 9) Propensity to form ulcers and erosions in the stomach.
    10. 10) Mental disorders: emotional imbalance, propensity for depression or euphoria.
    11. 11) Muscles of the abdomen, buttocks and thighs are atrophied.
    12. 12) Due to weak muscles and osteoporosis, scoliosis, kyphosis, and bone hump of the spine are formed.
    13. 13) Dysfunction of the heart.
    But if sharply to cancel reception of glucocorticoids for reduction of displays of a syndrome, the adrenal crisis can develop. This emergency condition, requiring first aid, and it manifests itself:

    • cold shaky sweat, tremor, a sense of hunger - symptoms of lowering blood sugar;
    • sharp decrease in blood pressure, which is manifested by weakness, fainting, pallor, frequent and weak pulse;
    • with vomiting;
    • abdominal pain;
    • pains in the heart and a violation of its rhythm, which is very dangerous due to a delay in the body of potassium, and an elevated level of potassium in the blood can stop the heart.

    Diagnosis


    It is possible to suspect the diagnosis already by the characteristic appearance of a person. But this is not enough, because you also need to determine the severity, and the cause of the syndrome.

    It is important that a tumor that produces hormones can not be seen, and its level is determined precisely by hormonal tests.

    The following studies are needed to diagnose the Itenko-Cushing syndrome:

    • , a cortisol level( the main glucocorticoid hormone) in the blood for several days in a row;
    • determining the level of ACTH in day and night;
    • assessment of the level of 17-hydroxycorticosteroids( substances in which cortisol is converted) in the urine;
    • daily excretion of cortisol in urine;
    1) Test with dexamethasone( synthetic glucocorticoid): if given to a person with Cushing's syndrome at night, and in the morning to determine the level of cortisol in the blood, then it will be more than 1.8 μg%( in a person without this syndrome, the hormone level drops below thisfigures);

    2) The test with dexazone is standard when this hormone is given in low doses every 6 hours for 2 consecutive days, after which the level of cortisol in the blood and oxycorticoid in the urine is determined. With this syndrome, cortisol should be reduced;

    3) Test with corticoliberin - a hormone of the hypothalamus, which is the main stimulator for the production of cortisol. If this hormone is injected into the vein, and after 15 minutes, the blood level of ACTH and cortisol is determined, then with adrenal genesis of the syndrome, cortisol will be above 1.4 μg% if there is no response, this indicates a tumor located in non-endocrine organs( lungs,thymus, gonads);

    • MRI of the adrenal glands, pituitary gland;
    • CT of thoracic cavity;
    • is a radioisotope study, according to which one looks at the absorption of the drug administered by each of the adrenals.
    To determine the degree of severity of the syndrome, the level in the blood and urine of glucose, potassium, sodium is determined;also carry out radiography of the spine.

    Treatment of Isenko-Cushing syndrome


    The Itenko-Cushing syndrome and its treatment, in the first place, will depend on the cause that led to this disease.

    1. 1) With a drug syndrome - a gradual withdrawal of drugs, possibly - with the replacement of them with immunosuppressants;
    2. 2) With tumors of the pituitary gland, lungs, adrenal glands - their surgical removal, which can be done after the preliminary radiation therapy;
    3. 3) In case of cancer with metastasis, which is produced by ACTH, in case of impossibility to remove all these tumors, medicinal treatment with "Mitotan", "Aminoglutethimide", "Ketoconazole" is used. Additionally, radiotherapy courses are conducted;
    4. 4) The introduction of arterial obstruction directly into vessels carrying blood supply to tumors.
    Before the surgical treatment of the Itenko-Cushing syndrome, a person's medication is prepared for the operation:

    • insulin - to lower the glucose level;
    • drugs that reduce blood pressure: "Enalapril", "Captopril";
    • preparations of potassium: Asparcum, Panangin;
    • for reduction of bone tissue destruction - calcium preparations( "Calcium-D3"), "Pamidronate", "Clodronate";
    • to reduce protein catabolism - anabolic steroids;
    • antidepressants.
    Thus, Cushing's syndrome is a collective concept for a large number of pathologies complicated by an increase in the blood glucocorticosteroids, characterized by a violation of all types of metabolism, the effect on the function of a large number of organs and tissues.


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