Itsenko-Cushing's syndrome
Itsenko-Cushing's syndrome is a pathological combination of symptoms that arise against the background of a disease such as hypercortisolism. Hypercortisolism, in turn, develops as a result of disturbances in the adrenal cortex with the release of high doses of the hormone cortisol, or after prolonged intake of glucocorticoids. Itsenko-Cushing's syndrome should not be confused with Itsenko-Cushing's disease, since the disease is a consequence of disorders in the hypothalamic-pituitary system.
Steroid hormones - glucocorticoids are of great importance for the normal functioning of the human body. They are directly involved in metabolism, are responsible for maintaining a large number of physiological functions. So, the hormone ACTH is responsible for the work of the adrenal glands, which promotes the production of corticosterone and cortisol. The hormones produced by the hypothalamus - liberins and statins - are responsible for the functioning of the pituitary gland. As a result, the body functions as a whole, and if at least one link is disrupted, many processes fail, including an increase in the amount of glucocorticoid hormones produced. Against the background of this condition, Itsenko-Cushing's syndrome develops.
According to statistics, women are 10 times more likely to suffer from the manifestations of this syndrome than men. The age at which the pathology debuts can vary from 25 to 40 years.
Content:
- Symptoms of Itsenko-Cushing's syndrome
- Causes of Itsenko-Cushing's syndrome
- Diagnostics of the Itsenko-Cushing syndrome
- Treatment of Itsenko-Cushing's syndrome
Symptoms of Itsenko-Cushing's syndrome
The symptoms of Itsenko-Cushing's syndrome are diverse and are expressed in the following:
- More than 90% of patients are obese. Moreover, the distribution of fat goes according to a certain type, which in medicine is called cushingoid. The greatest accumulation of body fat is observed in the neck, face, abdomen, back and chest. In this case, the patient's limbs remain disproportionately thin.
- The patient's face is rounded, becomes like the moon. The complexion is purple-red, has a cyanotic hue.
- Often, patients develop a bison hump or a buffalo hump. This is the name of the fatty deposits in the region of the seventh cervical vertebra.
- The skin on the back of the palms becomes very thin and becomes transparent.
- As the disease progresses, muscle atrophy gains strength. This leads to the formation of myopathy.
- The symptom of "sloping buttocks" and "frog belly" is also characteristic of this pathology. In this case, the muscles of the buttocks and thighs lose volume, and the stomach hangs down due to the weakness of the muscles of the peritoneum.
- Often, a hernia of the white line of the abdomen develops, in which the bag protrudes along the midline of the peritoneum.
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The vascular pattern on the skin becomes more visible, the dermis itself acquires a marble pattern. Due to the increased dryness of the skin, there are areas of peeling. In this case, the sweat glands begin to function more strongly. The fragility of the capillaries leads to the fact that bruises easily appear on the patient's body.
- Crimson or cyanotic striae cover the shoulders, abdomen, chest, thighs, and buttocks of the patient. Stretch marks can be up to 80 mm long and 20 mm wide.
- The skin becomes prone to acne, and spider veins often begin to form. Hyperpigmented areas are observed.
- Osteoporosis is a frequent companion of patients with Itsenko-Cushing's syndrome. It is accompanied by thinning of bone tissue and is expressed in severe pain. People become more prone to bone fractures and deformities. Against the background of osteoporosis, scoliosis and kyphoscoliosis develop. More vulnerable in this regard are the thoracic and lumbar spine. As the compression of the vertebrae progresses, patients slouch more and more, eventually becoming shorter.
- If Itsenko-Cushing's syndrome debuts in childhood, then the child has a growth retardation in comparison with peers. This is due to the slow development of epiphyseal cartilage.
- Such symptoms of Itsenko-Cushing's syndrome as cardiomyopathies in combination with arrhythmias, heart failure, arterial hypertension can lead to death.
- Patients are often inhibited, prone to depressive moods, suffer from psychosis. Suicide attempts are not uncommon.
- Diabetes mellitus, which does not depend on pancreatic diseases, is diagnosed in 10-20% of cases. The course of steroid diabetes is rather mild and can be corrected with the help of specialized medications and dietary nutrition.
- Development of peripheral edema against the background of nocturia or polynuria is possible. In this regard, patients suffer from constant thirst.
- Men suffer from testicular atrophy, prone to feminization. They often have potency disorders, gynecomastia.
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In connection with the suppression of specific immunity, secondary immunodeficiency develops.
- Women often suffer from menstrual irregularities, infertility, amenorrhea and other disorders associated with hyperandrogenism.
Causes of Itsenko-Cushing's syndrome
The causes of Itsenko-Cushing's syndrome are identified by experts as follows:
- A pituitary microadenoma is a benign tumor of the glandular contents, which does not exceed 20 mm in size, but at the same time affects the increase in the amount of adrenocorticotropic hormone in the body. It is this reason that is considered by modern endocrinologists to be the leading one in terms of the development of Itsenko-Cushing's syndrome and causes pathological symptoms in 80% of cases.
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Adenoma, adenocarcinoma, adenomatosis of the adrenal cortex leads to the development of Itsenko-Cushing's syndrome in 18% of cases. Tumor growths disrupt the normal structure and functioning of the adrenal cortex, provoking a malfunction of the whole organism.
- Corticotropinoma of the lungs, ovaries, thyroid gland, prostate, pancreas and other organs causes the development of the pathological syndrome in no more than 2% of cases. Corticotropinoma is a tumor that produces a corticotropic hormone that causes hypercortisolism.
- The development of the disease is possible with prolonged use of drugs that include glucocorticoids or ACTH.
The course of the disease can be progressive with the full development of symptoms over a period of six months to a year, or gradual with an increase in symptoms over a period of 2 to 10 years.
Diagnostics of the Itsenko-Cushing syndrome
If the patient has a suspicion of Itsenko-Cushing's syndrome, but at the same time the intake of corticoid hormones from the outside is completely excluded, then it is necessary to determine the true cause of hypercortisolism.
For this, modern specialists have the following screening tests, thanks to which the diagnosis of Itsenko-Cushing's syndrome is reliable:
- Study of daily urine in order to determine the excretion of the hormone in it. The presence of the syndrome is confirmed by an increase in cortisol in the urine by 3 or more times.
- Taking a small dose of dexamethasone. Normally, dexamethasone has a neutralizing effect on cortisol, reducing it by more than 50%. This will not happen if the syndrome is present.
- Taking a large dose of dexamethasone can distinguish Itsenko-Cushing's disease from the syndrome. If the cortisol level remains unchanged from baseline, then the patient is diagnosed with a syndrome, not a disease.
- Urinalysis reveals an increase in 11-hydroxyketosteroids and a drop in 17-KS.
- A blood test reveals hypokalemia, an increase in hemoglobin, cholesterol and erythrocytes.
- MRI, CT scan of the pituitary gland and adrenal glands can determine the presence of a tumor, which is the source of hypercortisolism.
- X-ray and CT of the spine and chest can determine the presence of complications of the pathological condition.
Treatment of Itsenko-Cushing's syndrome
Treatment of Itsenko-Cushing's syndrome is in the competence of an endocrinologist. If it has been established that certain drugs are the cause of the development of the syndrome, then they must be excluded. Cancellation of glucocorticoid drugs should be carried out gradually, followed by replacement with other immunosuppressants.
If hypercortisolism is of an endogenous nature, then it is necessary to take medications that affect the production of cortisol. These can be drugs such as: Aminoglutethimide, Ketoconazole, Chloditan, Mitotan.
Surgical intervention is necessary if a tumor is found in one or another part of the body. If it is not possible to remove the formation, then the removal of the organ as a whole is indicated (most often it is an operation to remove the adrenal glands). Or the patient is prescribed a course of radiation therapy for the hypothalamus and pituitary gland. Radiation therapy is carried out both separately and in combination with medical correction or after surgery. This allows you to achieve the best effect and minimize the risk of recurrence.
Depending on the symptomatology of Itsenko-Cushing's syndrome, patients receive appropriate therapy aimed at stopping the clinical signs of pathology.
Prescription of the following medicines is possible:
- Antihypertensive drugs (Spironolactone, ACE inhibitors);
- Potassium preparations;
- Medicines to lower blood sugar levels;
- Osteoporosis medications (calcitonins);
- Vitamin D;
- Anabolic steroid;
- Antidepressants;
- Sedatives, etc.
Compensation for impaired metabolism is important.
If the patient had to undergo surgery to remove the adrenal glands, then hormone replacement therapy is prescribed for life.
As for the prognosis, the lack of treatment in 50% of cases leads to death, since irreversible consequences occur in the body. In other cases, the prognosis depends on the cause of the development of hypercortisolism. In the presence of a benign corticosteroma, the prognosis is favorable and in 80% of cases, after removal of the tumor, the adrenal gland begins to function in full.
Five-year survival after removal of the malignant tumor ranges from 20 to 25%. If there is chronic adrenal insufficiency, then the reception of hormone replacement therapy is carried out throughout life. Patients with Itsenko-Cushing's syndrome need to abandon difficult working conditions and adhere to a normal daily routine.
Author of the article: Lebedev Andrey Sergeevich | Urologist
Education: Diploma in the specialty "Andrology" received after completing residency at the Department of Endoscopic Urology of the Russian Medical Academy of Postgraduate Education in the urological center of the Central Clinical Hospital No. 1 of JSC Russian Railways (2007). Postgraduate studies were completed here by 2010.
