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The Syndrome Itsenko-Kushinga

The syndrome Itsenko-Kushinga is a pathological combination of symptoms that occur on the background of such diseases as hyperadrenocorticism. Hyperadrenocorticism in turn, develops as a result of disorders in the adrenal cortex with the release of high doses of the hormone cortisol, or after long-term administration of glucocorticoids. The syndrome Itsenko-Kushinga should not be confused with the disease Itsenko-Kushinga, as the disease is a consequence of disturbances in the hypothalamic-pituitary system.

Steroid hormones – glucocorticoids are of great importance for the normal functioning of the human body. They take a direct part in metabolism, are responsible for maintaining a large number of physiological functions. So, in the adrenal glands respond to the hormone ACTH, which promotes the development of corticosterone and cortisol. For the functioning of the pituitary gland are responsible hormones produced by the hypothalamus – liberina and statins. As a result, the body functions as a whole and for violation in the operation of at least one of the link fails, a variety of processes, including, it is possible to increase the quantity produced by glucocorticoid hormones. Against this background, the state is developing the syndrome Itsenko-Kushinga.

According to statistics, women are 10 times more likely to suffer from manifestations of this syndrome than men. The age at which to debut the pathology may vary in the range from 25 to 40 years.

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The symptoms of the syndrome Itsenko-Kushinga

The symptoms of the syndrome Itsenko-Kushinga diverse and are expressed in the following:

  • More than 90% of patients suffer from obesity. Moreover, the distribution of fat is a specific type which is called in medicine cushingoid. The greatest accumulation of fat occurs in the neck, face, abdomen, back and chest. In this extremity the patient remain disproportionately thin.
  • The patient's face is rounded, becoming similar to the moon. The complexion is crimson red, has a cyanotic hue.
  • Often patients formed the Buffalo hump or the hump of a Buffalo. The so-called fat deposits in the area of the seventh cervical vertebra.
  • The skin on the palms from the back side becomes very thin,gets the transparency.
  • Progression of disease is gaining strength muscular atrophy. This leads to the formation of myopathy.

  • The symptom of "beveled buttocks"and"frog's belly" is also characteristic of this pathology. While the muscles of the buttocks and thighs lose volume, and the abdomen hangs down due to the weakness of the muscles of the peritoneum.
  • Often develop a hernia white line of the abdomen, in which there is bulging of the bag along the midline of the abdomen.
  • Vascular pattern on the skin becomes more visible, the dermis becomes a marble pattern. Due to excessive dryness of the skin, there are areas of flaking. In this case, sweat glands begin to function stronger. Capillary fragility leads to the fact that the body of the patient can easily see the bruises.
  • Purple or cyanotic striae cover the shoulders, stomach, Breasts, thighs and buttocks of the patient. Stretch can reach a length of 80 mm and width 20 mm.
  • The skin becomes prone to acne, often begin to form spider veins. Observed hyperpigmentaion areas.
  • Osteoporosis is a frequent companion of patients with syndrome Itsenko-Kushinga. It is accompanied by a thinning of bone tissue and is expressed in severe pain. People become more prone to fractures and bone deformities. On the background of osteoporosis, scoliosis, kyphoscoliosis. More vulnerable thoracic and lumbar spine. With the progression of the compression of the vertebrae, patients increasingly slouch, eventually becoming shorter.
  • If the syndrome Itsenko-Kushinga is making its debut in children, the child lags behind in growth compared to peers. This is due to the slow development of the epiphyseal cartilage.
  • To death can cause such symptoms of the syndrome Itsenko-Kushinga, as cardiomyopathy in combination with arrhythmias, heart failure, arterial hypertension.
  • Patients are often lethargic, prone to depressive moods, suffer from psychosis. Frequent suicide attempts.

  • Diabetes, independent of disease of the pancreas, is diagnosed in 10-20% of cases. For steroid diabetes is quite easy and can be changed by using receiving targeted medications and diet.
  • Perhaps the development of peripheral edema on the background of nocturia orpolyuria. In this regard, the patients suffer from persistent thirst.
  • Men suffer from atrophy of the testes, tend towards the feminization. They often have erectile dysfunction, gynecomastia.
  • In connection with the inhibition of specific immunity is the development of secondary immunodeficiency.
  • Women often suffer from menstrual disorders, infertility, amenorrhea, and other disorders on the background of hyperandrogenism.


Causes of the syndrome Itsenko-Kushinga

Causes of the syndrome Itsenko-Kushinga experts distinguish the following:

  • Microadenoma of the pituitary gland is a benign tumor of glandular contents, which in size does not exceed 20 mmbut has an impact on the increase in the body amount of adrenocorticotropic hormone. This reason is considered a modern leading endocrinologists in the development of the syndrome Itsenko-Kushinga and causes pathological symptoms in 80% of cases.
  • Adenoma, adenocarcinoma, adenomatosis of the adrenal cortex leads to the development of the syndrome Itsenko-Kushinga in 18% of cases. Tumor proliferation disrupts the normal structure and functioning of the adrenal cortex, causing malfunction of the whole organism.
  • Kortikotropinom lung, ovarian, thyroid, prostate, pancreas, and other organs becomes the cause of development of pathological syndrome is not more than 2% of cases. Kortikotropinom is a tumor that produces corticotropin hormone, causing Cushing.
  • May develop the disease with prolonged use of drugs, which include corticosteroids or ACTH.

The disease can be progressing with the full development of symptoms over a period of six months to one year, or with gradual increase of symptoms over a period of 2 to 10 years.

Diagnosis of the syndrome Itsenko-Kushinga

If the patient is suspected syndrome Itsenko-Kushinga, but it completely excludes the receipt of corticoid hormones from outside, it is necessary to determine the true cause of the hyperadrenocorticism.

To do this, armed with modern specialists have the following screeningtests, thanks to which diagnosis of the syndrome Itsenko-Kushinga is true:

  • The study of daily urine to determine its excretion of the hormone. Confirms the presence of the syndrome increased cortisol in the urine in 3 and more times.
  • A small dose of dexamethasone. In normal, dexamethasone exerts a neutralizing effect on cortisol, reducing it by more than 50%. In the presence of the syndrome that will not happen.
  • Taking a large dose of dexamethasone allows to distinguish disease Cushing's syndrome. If cortisol levels remain unchanged from the original, then the patient is diagnosed with a syndrome, not a disease.
  • Urine analysis reveals the growth of 11-oxyclozanide and drop 17-KS.
  • A blood test gives hypokalemia, the increase of hemoglobin, cholesterol and red blood cells.
  • MRI, CT scan of the pituitary and adrenal glands allow us to determine the presence of the tumor, which is the source of hyperadrenocorticism.
  • Radiography and CT of the spine and thorax to determine the presence of complications of the pathological condition.

Treatment of the syndrome Itsenko-Kushinga

Treatment of the syndrome Itsenko-Kushinga is in the competence of the endocrinologist. If it was established that the cause of the development of the syndrome are, certain medicines, it is necessary to exclude them. The abolition of glucocorticoid drugs should be gradually followed by replacement with other immunosuppressants.

If the hyperadrenocorticism is of endogenous nature, the necessary taking drugs that affect cortisol production. It can be drugs such as Aminoglutethimide, Ketoconazole, Klodian, Mitotan.

Surgery was necessary in case of detection of tumors in different parts of the body. If you remove the education fails, it shows the removal of the organ as a whole (often this is surgery to remove the adrenal glands). Either the patient is administered a course of radiation therapy area of the hypothalamus and pituitary gland. Radiation therapy alone or in combination with medicamentous correction or after surgery. This allows you to achieve the best effect and to minimize the risk of relapse.

Depending on symptoms of the syndrome Itsenko-Kushinga, patients undergo appropriate therapy aimed at relief of clinical signs of disease.

Perhaps the appointment of the following drugs:

  • Antihypertensive drugs(Spironolactone, ACE inhibitors);
  • Potassium supplements;
  • Medicines to reduce blood sugar levels;
  • Drugs for the treatment of osteoporosis (calcitonin);
  • Vitamin D;
  • Anabolic steroids;
  • Antidepressants;
  • Sedatives, and St.

Important compensation of disturbed metabolism.

If the patient had to undergo an operation to remove the adrenal glands, he life prescribed hormone replacement therapy.

As for the forecast, the lack of treatment in 50% of cases leads to death, as in the body there are irreversible consequences. In other cases, the prognosis depends on the causes of hyperadrenocorticism. In the presence of benign korticosteroma the prognosis is favorable in 80% of cases after removal of the tumor, the adrenal gland begins to function fully.

Five-year survival after resection of malignant tumor is in the range from 20 to 25%. If you have chronic adrenal insufficiency, the reception of hormone replacement therapy is carried out throughout life. Patients with syndrome Itsenko-Kushinga, you must abandon heavy working conditions and to adhere to a normal routine.