home Consultations What is hypercortisolism syndrome characterized by and how to treat it. Endogenous hypercortisolism Cushing's syndrome in adults

What is hypercortisolism syndrome characterized by and how to treat it. Endogenous hypercortisolism Cushing's syndrome in adults

Itsenko-Cushing's disease is a complex of neuroendocrine symptoms that arise as a result of disruption of the pituitary gland, hypothalamus and adrenal glands. First of all, the hypothalamus is affected and begins to produce an increased amount of corticotropin-releasing hormone, which activates the increased production of corticotropin by the pituitary gland. This in turn causes secondary hyperplasia and hyperfunction of the adrenal glands. Together, all these pathological changes provoke the development of Itsenko-Cushing's disease (hypercortisolism).

The disease affects mainly middle-aged people (20-40 years old) and is diagnosed in most cases in the fair sex. Pathology is less commonly detected in children and the elderly.

Itsenko-Cushing's disease is classified into types according to different criteria. Depending on the severity of the disease, it is divided into several degrees:

The mild stage is characterized by moderate symptoms, and there is no osteoporosis.
The moderate degree is characterized by a clear manifestation of the clinical picture, but there are no complications.
A severe stage of the disease leads to an exacerbation of symptoms and the appearance of a number of complications: poor circulation, the development of osteoporosis and, as a consequence, frequent bone fractures, and mental disorders also occur.

Depending on the nature of the disease, a distinction is made between progressive and torpid disease. The first type is characterized by rapid development of symptoms and rapid complications. Patients quickly lose their ability to work and become disabled. The torpid course of the disease is characterized by a slow course and gradual development of the disease.

Causes of Itsenko-Cushing's disease

The exact causes of the development of Itsenko-Cushing's disease have not been established. Quite often, pathology develops in women after childbirth, which is due to hormonal imbalance and significant physiological changes in the body. In addition, in the fair sex, the disease can develop during pregnancy or menopause, which is also due to changes in the level of hormones in the body.

Also provoking factors for the disease are brain injuries, previous or meningitis, as well as damage to the central nervous system or severe intoxication of the body. It can become the root cause of hypercortisolism. Itsenko-Cushing's disease can also be caused by long-term use of hormonal medications.

As the disease develops, the process of production of biologically active substances is disrupted. First of all, the process of corticotropin synthesis is disrupted, which should decrease with an increase in cortisol. Impaired functioning of organs leads to increased levels of hormones.

Symptoms of Itsenko-Cushing's disease

The clinical picture of the disease is quite clear and is characterized by acute manifestation of symptoms.

Impaired carbohydrate tolerance , which can also cause an increase in the amount of adipose tissue in the body.

Quite often, patients with Itsenko-Cushing's disease exhibit problems with sexual function . In the fair sex, the menstrual cycle is disrupted, infertility or delayed sexual development develops. If the disease develops during pregnancy, it can lead to various pathologies and can cause miscarriage. Due to hormonal imbalances, a woman’s hair increases above her lip, along her abdomen, and on her limbs. In some cases, there is a pathological enlargement of the clitoris.

Cushing's disease also has a negative impact on men. Quite often there is a development and significant decrease in libido.

The disease leads to the development psycho-emotional disorders , which is due to increased levels of serotonin and corticotropin. Patients complain of sleep problems, memory loss and increased irritability. Patients develop a tendency to depression and often develop a hypochondriacal state.

As a result of damage to the digestive system, the patient develops heartburn, gastritis, duodenal and stomach ulcers.

Diagnosis of Itsenko-Cushing's disease

To diagnose and treat Itsenko-Cushing's disease, consultation with an endocrinologist, neurologist and psychologist is required. In some cases, additional help from other specialists is required - a nephrologist, cardiologist, etc. To obtain complete information about the patient’s health status, a number of laboratory tests and instrumental research methods are performed:

Treatment of Itsenko-Cushing's disease

The main goal of therapy for Itsenko-Cushing's disease is to eliminate the negative symptoms of the disease, normalize the level of hormones in the body (ACTH, corticosteroids and serotonin), and also activate metabolic processes. An integrated approach is used for treatment, which includes several areas - drug therapy, proton therapy of the hypothalamic-pituitary region and surgical intervention. Treatment is prescribed by the doctor after conducting a complete examination of the patient and establishing the extent of the disease.

Drug therapy is effective for mild to moderate Cushing's disease. For treatment, drugs that block the activity of the pituitary gland are used - Bromocriptine and Reserpine. In addition, medications are prescribed that suppress the synthesis of ACTH and steroid hormones. Products are used whose action is aimed at improving overall metabolism and electrolyte metabolism.

If there is no tumor in the pituitary gland, proton therapy or x-ray therapy is performed. Quite often, stereotactic implantation of radioactive isotopes into the pituitary gland is used. After 2-3 months, weight and blood pressure normalize, a woman’s sexual function improves, the menstrual cycle and bone tissue condition are regulated.

In severe cases of the disease, surgery is performed during which the adrenal glands are removed. After surgery, the patient should undergo replacement therapy with mineralocorticoids and glucocorticoids for life.

If a pituitary adenoma is detected, complete resection of the tumor is performed. In most cases (80%), long-term remission is achieved. Recurrence of the disease is extremely rare.

Prevention of Itsenko-Cushing's disease

The prognosis of the disease depends on the severity of the disease, the age of the patient and his general health. If Itsenko-Cushing's disease is detected at a mild or moderate stage of development, then young people have a very high chance of complete recovery. An advanced form of the disease can be fatal, since the disease adversely affects the functioning of the heart muscle, respiratory and urinary systems.

In order to prevent the development of Itsenko-Cushing's disease, neuroinfections and intoxications should be treated in a timely manner. It is important to avoid traumatic brain injuries and limit excessive psycho-emotional experiences.

Hormones play an important role in many processes occurring in the body. Various glands are responsible for the synthesis of hormones. With a lack of hormone production or an excess of it, deviations develop that lead to disruptions in the functioning of the entire body.

Itsenko-Cushing's disease (hypercortisolism) is a neuroendocrine disorder characterized by excessive synthesis of adrenocorticotropic hormone (ACTH) in the pituitary gland, which regulates adrenal function. Excess ACTH leads to increased production of corticosteroids by the adrenal glands and to the development of hypercortisolism. The disease is rare and mainly affects women of childbearing age.

general information

The symptoms of the disease were first described by neurologist Nikolai Itsenko in the 20s of the last century. He argued that the cause of the disease was changes in the hypothalamus. A little later, the American Harvey Cushing argued that the disease was associated with tumors of the pituitary gland. They both turned out to be right. Hypercortisolism is a consequence of disruption of the hypothalamic-pituitary system.

Endocrinologists distinguish between the syndrome and Itsenko-Cushing's disease. They have common symptoms, but there are differences in the etiology of the process. The basis of the syndrome is the excessive production of glucocorticoids by tumors of the adrenal cortex or long-term use of exogenous hormonal drugs. In Itsenko-Cushing's disease, the process is concentrated at the level of the hypothalamus and pituitary gland. Thus, Itsenko-Cushing syndrome includes all pathological processes, the basis of which is hypercortisolism.

Causes and mechanism of occurrence

The pathology is associated with a malfunction of the mechanism of interaction between the adrenal glands, hypothalamus and pituitary gland. The hypothalamus receives nerve impulses that stimulate it to synthesize substances that activate the release of ACTH in the pituitary gland. In response to this, the pituitary gland releases a large concentration of the hormone into the blood. The adrenal glands react to the process and begin to produce excessive corticosteroids. Because of this, all metabolic processes in the body are disrupted.

Typically, NIC is accompanied by an increase in the volume of the pituitary gland (adenoma, tumor). This leads to a gradual enlargement of the adrenal glands. Sometimes a relationship is noted between the onset of the development of hypercortisolism with previous infections (meningitis, encephalitis), and skull injuries. In women, the disease may be associated with hormonal imbalances during pregnancy and menopause.

Excessive production of ACTH results in a range of adrenal and non-adrenal symptoms. A high concentration of corticosteroids affects the metabolism of proteins and carbohydrates, causes atrophy of connective and muscle tissue, hyperglycemia, insulin deficiency, and subsequently the development of steroid diabetes. When lipid metabolism fails, obesity develops. The effect of catabolism on bones accompanies the excretion and deterioration of calcium reabsorption in the gastrointestinal tract, resulting in osteoporosis.

Characteristic signs and symptoms

The development of pathology can be progressive and torpid. The progressive course develops quickly - within 6-12 months, symptoms increase, and complications arise. Torpid development of BIC is characterized by gradual (over 3-10 years) pathological changes.

About 90% of patients with Cushing's disease have varying degrees of obesity. This is one of the main symptoms of pathology. Fat deposits are concentrated in the face, neck, back, chest, and abdomen (Cushingoid appearance). The face takes on a puffy appearance and becomes purple-marbled. The skin of the face is dry and flaky. Striae form on the abdomen, mammary glands, and shoulders. Muscle mass and muscle tone gradually decrease, especially in the buttocks and thighs. After some time, hernial protrusions may be noticeable.

In women, endocrine disorders affect the menstrual cycle. There may be excessive hair growth on the body and face. In men, libido decreases and impotence may occur. If BIC began to develop in early childhood, then there may be a delay in puberty due to a decrease in the production of gonadotropic hormones.

Due to the effect on the skeletal system and the development of osteoporosis, patients experience frequent deformities and bone fractures. From the cardiovascular system there is hypertension, tachycardia, cardiopathy, heart failure. Lesions of the urinary organs are manifested by the development,.

Note! Mental disorders in BIC are manifested by depressive, hypochondriacal, neurasthenic and other syndromes. The patient's memory deteriorates, intellectual activity decreases, frequent emotional fluctuations are observed, and there may be suicidal thoughts.

Diagnostics

If there is a suspicion of Itsenko-Cushing's disease, several specialists (endocrinologist, neurologist, and others) need to be involved in the work.

To diagnose the disease, a set of screening tests is performed:

determination of daily excretion of cortisol in urine;
small dexamethasone test - the cortisol level is determined in stages before and after the use of dexamethasone; if there is hypercortisolism, the cortisol level will not fall;
the large dexamethasone test differs from the previous test only in the dosage of dexamethasone and is used to differentiate pathogenetic types of BIC and adrenal corticosteroma;
biochemical examination of urine and blood to diagnose the state of electrolyte metabolism.

Instrumental diagnostics include:

adrenal glands and pituitary gland;
radiography of the spinal column, chest;
adrenal glands

Treatment methods for Itsenko-Cushing disease

The main task with BIC is to remove hypothalamic shifts, normalize the production of ACTH and corticosteroids, and metabolic processes.

Therapy may include various activities:

drug treatment;
radiation therapy;
X-ray therapy;
proton therapy;
surgical intervention.

Medications are prescribed at the initial stages of the disease. To block pituitary hyperactivity and reduce ACTH secretion, use:

Bromocriptine;
Reserpine.

Symptomatic therapy may consist of taking the following groups of drugs:

diuretics;
cardiac glycosides;
immunomodulators;
antidepressants;
sedatives;
drugs that lower glucose levels and others.

If there is a tumor in the area of the adrenal glands or pituitary gland, then the most effective method of treating them is adrenalectomy (removal of the adrenal gland). A contraindication to the operation is a very severe form of hypercorticism and the presence of somatic pathologies in the patient. In 70-80% of patients, remission occurs after surgery. And less than 20% have recurrences after adrenalectomy. After bilateral adrenal removal, lifelong hormone replacement therapy is required.

Radiation therapy is often used. It is usually combined with drug or surgical treatment for greater effectiveness. As a rule, several months of radiation therapy lead to stable remission. Patients' blood pressure normalizes, body weight decreases, bone and muscle tissue return to normal. In the absence of a pituitary tumor, gamma therapy, radiotherapy, and proton therapy can be prescribed.

Prevention measures

There are no special measures to prevent the disease. You can reduce the risk of developing pathology by adhering to general preventive recommendations:

Healthy food;

eliminate alcohol consumption;

avoid stressful situations and excessive physical activity;

prevent traumatic brain injuries;

promptly treat infectious diseases;

undergo regular medical examinations to monitor your health and check your hormone levels.

Itsenko-Cushing's disease is a dangerous disease that is characterized by polymorphism of symptoms in many body systems. If the disease lasts for a long time, it can lead to serious complications, even death. If at least one sign appears that indicates problems of an endocrine nature, you need to undergo a comprehensive examination and consult with several specialists. With timely and adequate treatment, there is a high chance of complete recovery.

Video. Elena Malysheva about the causes of the development of Itsenko-Cushing disease and the features of treatment of the disease:

What is Itsenko-Cushing's disease? We will discuss the causes, diagnosis and treatment methods in the article by Dr. E. I. Vorozhtsov, an endocrinologist with 14 years of experience.

Definition of disease. Causes of the disease

Itsenko-Cushing's disease(BIC) is a severe multisystem disease of hypothalamic-pituitary origin, the clinical manifestations of which are caused by hypersecretion of hormones of the adrenal cortex. It is an ACTH-dependent form of hypercortisolism (a disease associated with excessive secretion of hormones by the adrenal cortex).

The main cause of the disease is corticotrophic microadenoma (benign tumor) of the pituitary gland. The occurrence of the latter can be associated with many factors. Often the disease develops after a brain injury, pregnancy, childbirth, or neuroinfection.

Itsenko-Cushing's disease is a rare disease, the incidence of which is 2-3 new cases per year per one million population.

Symptoms of Itsenko-Cushing's disease

The clinical signs of this disease are due to hypersecretion of corticosteroids. In most cases, the first clinical sign is obesity, which develops in the face, neck, trunk and abdomen, with thin limbs. Later, skin changes appear - thinning of the skin, difficult-to-heal wounds, bright stretch marks (stretch marks), skin hyperpigmentation, hirsutism (excessive hair on the face and body).

Another important and common symptom is muscle weakness and the development of osteoporosis.

Itsenko-Cushing's disease is accompanied by immunodeficiency, which manifests itself in the form of recurrent infection (most often chronic pyelonephritis occurs).

Frequency of clinical manifestations of NIC:

moon-shaped face - 90%;
impaired glucose tolerance - 85%;
abdominal type of obesity and hypertension - 80%;
hypogonadism (lack of testosterone) - 75%;
hirsutism - 70%;
osteoporosis and muscle weakness - 65%;
striae - 60%;
swelling of the joints and fat deposition in the area of the VII cervical vertebra - 55%;
back pain - 50%;
mental disorders - 45%;
deterioration of wound healing - 35%;
polyuria (excessive urine production), polydipsia (excessive thirst) - 30%;
kyphosis (curvature of the spine) - 25%;
and polycythemia (increased concentration of red blood cells in the blood) - 20%.

Pathogenesis of Itsenko-Cushing's disease

The pathogenesis of this disease is multistage.

In the corticotrophs of the pituitary gland, characteristic receptor-postreceptor defects arise, which further lead to the transformation of normal corticotrophs into tumor ones with hyperproduction of adrenocorticotropic hormone and the subsequent development of hypercortisolism.

The transformation of normal corticotrophs into tumor ones is influenced by hypothalamic hormones and local growth factors:

Chronically elevated ACTH concentrations lead to increased secretion of cortisol, which causes the characteristic clinical picture of BIC.

Classification and stages of development of Itsenko-Cushing disease

BIC classification by severity:

mild form - symptoms are moderate;
medium form - symptoms are clearly expressed, there are no complications;
severe form - in the presence of all the symptoms of NIC, various complications arise.

BIC classification with the flow:

torpid (pathological changes develop gradually over 3-10 years);
progressive (an increase in symptoms and complications of the disease occurs within 6-12 months from the onset of the disease);
cyclical (clinical manifestations occur periodically, inconsistently).

Complications of Itsenko-Cushing's disease

Vascular and. This complication develops in half of patients after 40 years. This pathology can lead to death. In most cases, this occurs due to acute heart failure and pulmonary edema.
Pathological osteoporetic fractures. The most common fractures are the spine, ribs, and tubular bones.
Chronic renal failure. In 25-30% of patients, glomerular filtration and tubular reabsorption (reverse absorption of fluid) decrease.
Steroid diabetes mellitus. This complication is observed in 10-15% of patients with this disease.
Muscle atrophy, resulting in severe weakness. Patients experience difficulties not only when moving, but also when standing up.
Visual impairment(cataracts, exophthalmos, visual field impairment).
Mental disorders. The most common symptoms are depression, panic attacks, paranoid states, and hysteria.

Diagnosis of Itsenko-Cushing's disease

The diagnosis of Itsenko-Cushing's disease can be established on the basis of characteristic complaints, clinical manifestations, hormonal and biochemical changes and instrumental research data.

Extremely important hormonal indicators for this pathology will be ACTH and cortisol. ACTH levels increase and range from 80 to 150 pg/ml. An increase in the level of cortisol in the blood is not always informative, since an increase in this indicator may be due to a number of other reasons (stress, alcoholism, pregnancy, endogenous depression, family resistance (tolerance) to glucocorticoids, etc.). In this regard, methods have been developed to study the level of free cortisol in daily urine or saliva. The most informative is the study of free cortisol in saliva at 23:00.

The small dexamethasone test is used to prove hypercortisolism. With NIR, this test will be negative, since cortisol is not suppressed when taking 1 mg of dexamethasone.

A biochemical blood test can reveal numerous changes:

increased blood serum levels of cholesterol, chlorine, sodium and globulins;
decrease in blood concentrations of potassium, phosphates and albumin;
decreased alkaline phosphatase activity.

After confirmed hypercortisolism, instrumental research methods (MRI of the pituitary gland, CT scan of the adrenal glands) are necessary.

With NIR, in 80-85% of cases a pituitary microadenoma (tumor up to 10 mm) is detected, in the remaining 15-20% - a macroadenoma (benign neoplasm from 10 mm).

Treatment of Itsenko-Cushing's disease

Drug therapy

Currently, there are no effective drugs to treat this pathology. In this regard, they are used either in the presence of contraindications for surgical treatment, or as an auxiliary therapy. The following medications are used:

In addition to drugs from these groups, patients are prescribed symptomatic therapy to reduce the symptoms of hypercortisolism and improve the patient’s quality of life:

antihypertensive therapy (ACE inhibitors, calcium antagonists, diuretics, beta-blockers);
antiresorptive therapy for the development of steroid osteoporosis;
drugs that correct carbohydrate metabolism disorders (metformin, sulfonylureas, insulin);
therapy aimed at correcting dyslipidemia;
antianginal therapy.

Operative therapy

Radiosurgery

Gamma knife surgery. During surgery, a beam of radiation directed directly at the pituitary adenoma destroys its cells. This treatment prevents tumor growth with a single dose of radiation. The procedure lasts about two to three hours. It does not affect the surrounding brain structures and is performed with an accuracy of 0.5 mm. Complications of this therapy are hyperemia (increased blood flow) in the irradiation area, alopecia (hair loss).

Forecast. Prevention

The prognosis of BIC depends on several indicators: the form and duration of the disease, the presence of complications, the state of immunity and other factors.

Full recovery and restoration of ability to work is possible with a mild form of the disease and a short period of illness.

In moderate and severe forms of BIC, the ability to work is extremely reduced or absent. After a bilateral adrenalectomy, chronic adrenal insufficiency develops, which dictates lifelong use of gluco- and mineralcorticoids.

In general, any treatment, as a rule, leads to an improvement in the patient’s quality of life, but it remains lower than in persons without this pathology.

Itsenko-Cushing's disease is a serious chronic disease that, if not treated in a timely manner, can cause death. The mortality rate for this pathology is 0.7%.

The five-year survival rate for BIC without treatment is 50%, but is markedly improved even if only palliative treatment is performed (with bilateral adrenalectomy, survival increases to 86%).

There is no primary prevention for NIC. Secondary prevention of the disease is aimed at preventing relapse of the disease.

Bibliography

Itsenko-Cushing's disease is an endocrine disease of the pituitary gland. One of the common causes of increased cortisol levels is Cushing's disease. With this disease, a tumor formation is detected in the pituitary gland, which in 80% of cases is small in size (no more than 10 mm, more often 3-5 mm).

Itsenko-Cushing's disease accounts for 70% of cases of hypercortisolism (increased cortisol levels). The prevalence of this disease is 5-10 cases per 1 million population, and women get sick 4-5 times more often than men. Cushing's disease should be distinguished from Cushing's syndrome. The cause of the syndrome is tumors of the adrenal cortex, as well as ectopic ACTH syndrome. I wrote about this in my .

Most often, in Itsenko Cushing's disease, the pituitary tumor synthesizes only ACTH, i.e. the tumor is monoclonal. This happens in 85-90% of cases. But sometimes there is a tumor that contains cells of different types, which means that they synthesize different hormones.

Excessive secretion of ACTH by the pituitary gland stimulates the adrenal glands to produce excess glucocorticoids (cortisol) and androgens. ACTH does not affect the synthesis of aldosterone. Therefore, with Itsenko-Cushing's disease, the level remains the same. By the way, the synthesis of aldosterone is controlled by the renin-angeotensin-aldosterone system of the kidneys.

So, with prolonged stimulation of the adrenal glands by the pituitary hormone ACTH, hypertrophy of the adrenal tissue occurs (bilateral macronodular hyperplasia of the adrenal glands, in other words - secondary adenomatosis).

This change in the adrenal glands can be mistakenly considered the cause of hypercortisolism. This can lead to incorrect diagnosis and incorrect treatment.

Symptoms of Itsenko-Cushing's disease

Symptoms Itsenko-Cushing's disease are no different from the symptoms of hypercortisolism caused by another cause. And the symptoms caused by the presence of a tumor in the pituitary gland are so insignificant that they practically do not attract attention against the backdrop of a bright clinic of hypercortisolism.

So, first of all, the appearance of a patient suffering from Cushing’s disease attracts attention. Such patients are characterized by:

Dysplastic (uneven) type of obesity (excessive fat deposition on the face, neck, torso with thin limbs)
The face is moon-shaped (round), the skin is purple-red, the so-called “matronism”.
The skin is generally dry, thinned, with a characteristic marble pattern (blood vessels are visible).
The presence of characteristic stretch marks (stretch marks). Striae are purplish-bluish, wide (up to several cm), located on the abdomen, inner thighs, and shoulders. Striae occur more often in young patients; they almost never occur in people over 50 years of age.
Skin tendency to acne, pustules, fungal diseases
Hyperpigmentation (darkening) of the skin and mucous membranes, but this symptom is not as pronounced as with
Increased hair growth in androgen-dependent areas (face, chest, shoulders, back)
"Sheep smell" coming from the patient.

Patients who became ill in childhood are characterized by growth retardation. And excess androgens lead to premature puberty.

Excess of adrenal hormones also affects:

Circulatory system: increased blood pressure, palpitations, arrhythmias, heart failure, thromboembolism.
Skeletal system: osteoporosis, compression fractures, scoliosis.
Digestive system: gastritis, ulcers, fatty liver, pancreatitis.
Urinary system: kidney stones, frequent urination (usually at night), frequent urinary tract infections.
Muscles: muscle atrophy, characterized by atrophy of the muscles of the fingers (“spider fingers”) and gluteal muscles (“flat” buttocks). Patients find it difficult to climb stairs and get out of chairs without using their hands.
Reproductive system: women experience various types of menstrual irregularities, atrophy of the uterus and ovaries, mammary glands; men have testicular atrophy, decreased sexual hair, decreased libido and erectile dysfunction.
Nervous system: 50% have mental disorders, which are manifested by memory loss, emotional lability, irritability, sleep disturbance, stupor, depression, steroid psychoses.
Immune system: decreased immunity and decreased resistance to infections, chronicity of their course, poor wound healing.
on carbohydrate metabolism: impaired glucose tolerance, steroid diabetes mellitus occurs.

Diagnosis of Itsenko-Cushing's disease

Diagnosis Itsenko-Cushing's disease" diagnosed on the basis of characteristic complaints (changes in appearance, muscle weakness, arterial hypertension), as well as on the basis of laboratory and instrumental research methods.

If Cushing's disease is suspected, the daily excretion of free cortisol in the urine is determined.

IMPORTANT! The determination of free cortisol in the blood is not clinically significant, since the production of cortisol is influenced by many factors, and a single determination of cortisol in the blood does not provide an objective picture.

If an increased result is obtained, a small dexamethasone test is performed, which makes it possible to clarify the type of hypercortisolism (endogenous or exogenous), since an increase in cortisol can occur not only with Itsenko Cushing’s syndrome and disease.

Radiation treatment of Itsenko-Cushing's disease

As a primary method of treating Cushing's disease, it can be used in the following cases:

in patients under 20 years of age
in case of refusal of surgical treatment
with contraindications to surgical treatment
when Itsenko-Cushing's disease is proven, but there are no clear signs of a tumor on MRI

Radiation treatment of Itsenko-Cushing's disease includes several methods:

proton irradiation
gamma therapy
gamma knife

At proton irradiation Narrow beams of protons are used, which have high penetrating power. Irradiation is performed once. Improvement in the condition and normalization of hormonal levels are observed after 2-3 months, and complete remission occurs after 6-12 months. Until complete remission is achieved, drugs are prescribed that inhibit the synthesis of adrenal hormones. Remission is achieved in 88-92% in patients under 20 years of age, in 75-8 5% in adults; after 40 years, the effectiveness of this method decreases significantly.

Gamma therapy these days it is rarely performed, mainly when it is impossible to perform surgery or proton therapy. The effectiveness of gamma therapy is low (about 66%) and remission is achieved after 15-24 months.

Advantage gamma knife is a one-time use and rapid onset of remission. A disadvantage is the low prevalence of the method.

Drug treatment of Itsenko-Cushing's disease

Drug treatment of Itsenko-Cushing's disease is used as an additional method:

in preparation for surgical treatment
in the period after irradiation until stable remission
with severe hypercortisolism and severe condition

For this purpose use:

aminoglutethimide
ketoconazole

The dose is selected individually, taking into account the daily excretion of cortisol in the urine. The drug is discontinued if urinary cortisol levels are low or if adrenal insufficiency occurs.

After treatment of Itsenko-Cushing's disease, the patient should be monitored by an endocrinologist (at least 6 months). Correction of the manifestations of hypercortisolism is required: treatment of osteoporosis, control of carbohydrate metabolism, treatment of metabolic disorders. Since relapses of the disease are observed after surgical treatment, examination is required annually.

After irradiation, examinations are carried out after 3 and 6 months. And when complete remission develops, an annual examination is indicated to exclude the development of hypopituitarism.

With warmth and care, endocrinologist Dilyara Lebedeva

Itsenko-Cushing's disease- a disease in which an excess amount of adrenocorticotropic hormone (ACTH) is produced, which regulates the functioning of the body. With an excess of ACTH, the adrenal glands increase in size and intensively produce their own corticosteroids, which leads to the disease.

The symptoms of this disease were described at different times by two scientists - American neurosurgeon Harvey Cushing and Odessa neuropathologist Nikolai Itsenko.

An American specialist associated the disease with, a Soviet scientist suggested that the cause of the disease lies in changes in the area of the brain responsible for interaction and. Today, scientists have come to the conclusion that Itsenko-Cushing's disease occurs as a result of malfunctions in the hypothalamic-pituitary system - both researchers were right.

Itsenko-Cushing's disease, or otherwise hypercortisolism- a severe neuroendocrine disease that occurs in case of dysregulation of the hypothalamic-pituitary-adrenal system, when an excess of adrenal hormones - corticosteroids - is formed.

Distinguish Itsenko-Cushing syndrome And Itsenko-Cushing's disease. Itsenko-Cushing syndrome refers to all pathological conditions with one common symptom complex, which is based on hypercortisolism, i.e. increased secretion of cortisol by the adrenal cortex.

Itsenko-Cushing syndrome includes:

Itsenko-Cushing's disease, which occurs as a result of increased production of adrenocorticotropic hormone by the pituitary gland (pituitary ACTH-dependent syndrome).
ACTH- or corticoliberin-ectopic syndrome.
iatrogenic or drug syndrome various hyperplastic tumors of the adrenal cortex - adenomatosis, adenoma, adenocarcinoma.

What provokes / Causes of Itsenko-Cushing's Disease:

Cause of Cushing's syndrome There can be various conditions, but most often hypercortisolism is caused by increased production of adrenocorticotropic hormone by the pituitary gland (Cushing's disease). Adrenocorticotropic hormone can be produced by pituitary microadenoma (a microadenoma is a small space-occupying formation in the pituitary gland, usually benign, not exceeding 2 cm in size, hence its name) or ectopic corticotropinoma - a tumor that produces large quantities of corticotropic hormone, but develops slowly. Ectopic corticotropinoma, which has an oncogenic or other malignant nature, can be located in the bronchi, testicles, and ovaries.

Pathogenesis (what happens?) during Itsenko-Cushing's Disease:

A cascade of hormonal changes in the body is launched.

It all starts with the fact that mysterious nerve impulses are received that cause his cells to produce too many substances that activate the release of adrenocorticotropic hormone in.

In response to such powerful stimulation, the pituitary gland releases a huge amount of this same adrenocorticotropic hormone (ACTH). It, in turn, affects: it forces them to produce their own corticosteroids in excess. This is where complete chaos begins, because corticosteroids affect all metabolic processes.

As a rule, with Cushing's disease, the pituitary gland is enlarged in size (tumor, or pituitary adenoma). As the disease progresses, the adrenal glands also enlarge.

Symptoms of Cushing's Disease:

Diagnosis of the disease, as a rule, does not present much difficulty, because The data from the physical examination of the patient are of paramount importance. In more than 90% of patients, it is observed to one degree or another, and special attention is paid to the distribution of fat - dysplastic (Cushingoid type): on the abdomen, chest, neck, face (moon-shaped face of a purplish-red color, sometimes with a cyanotic tint - t so-called matronism) and back (the so-called “menopausal hump”). Cushing's syndrome is distinguished from other forms of obesity by thin, almost transparent skin on the back of the hand.

Amyotrophy- a decrease in the volume of muscle mass, accompanied by a decrease in muscle tone and muscle strength, especially the gluteal and femoral muscles (“skewed buttocks”) - a typical sign of Itsenko-Cushing syndrome. Atrophy of the muscles of the anterior abdominal wall (“frog belly”) is also observed, and over time, hernial protrusions appear along the white line of the abdomen.

Leather The patient’s skin becomes thinner, has a “marbled” appearance with a clear vascular pattern, is prone to dryness, alternates with areas of regional sweating, and flakes off.

Very often on stretch marks appear on the skin- stretch stripes of crimson or purple color. Mostly, stretch marks are located on the skin of the abdomen, the inner and outer surfaces of the thighs, mammary glands, shoulders, and can range from several centimeters to several millimeters. The skin is replete with various acne-type rashes, manifested as a result of inflammation of the sebaceous glands, numerous subcutaneous microhemorrhages, and spider veins.

In some cases, with Cushing's disease and ectopic ACTH syndrome, hyperpigmentation can be observed - an uneven distribution of pigment, manifested in the form of dark and lighter spots that differ from the basic color of the skin. A disease associated with damage (thinning) of bone tissue, leading to fractures and bone deformations - osteoporosis - one of the most severe complications accompanying Cushing's syndrome. As a rule, osteoporosis develops in the vast majority of patients. Fractures and bone deformations, accompanied by severe pain, eventually lead to scoliosis and kyphoscoliosis.

With hypercortisolism, it often develops cardiomyopathy- a group of diseases in which the heart muscle primarily suffers, which is manifested by dysfunction of the heart muscle.

There are several reasons that can cause cardiomyopathy, the main ones being the catabolic effects of steroids on electrolyte changes and arterial hypertension. Manifestations of cardiomyopathy are cardiac arrhythmias and heart failure. The latter in most cases can lead to death of the patient.

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You? It is necessary to take a very careful approach to your overall health. People don't pay enough attention symptoms of diseases and do not realize that these diseases can be life-threatening. There are many diseases that at first do not manifest themselves in our body, but in the end it turns out that, unfortunately, it is too late to treat them. Each disease has its own specific signs, characteristic external manifestations - the so-called symptoms of the disease. Identifying symptoms is the first step in diagnosing diseases in general. To do this, you just need to do it several times a year. be examined by a doctor, in order not only to prevent a terrible disease, but also to maintain a healthy spirit in the body and the organism as a whole.

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Other diseases from the group Diseases of the endocrine system, nutritional disorders and metabolic disorders:

Addisonian crisis (acute adrenal insufficiency)

Breast adenoma

Adiposogenital dystrophy (Perchkranz-Babinski-Fröhlich disease)

Adrenogenital syndrome

Acromegaly

Nutritional insanity (nutritional dystrophy)

Alkalosis

Alkaptonuria

Amyloidosis (amyloid dystrophy)

Amyloidosis of the stomach

Intestinal amyloidosis

Pancreatic islet amyloidosis

Liver amyloidosis

Amyloidosis of the esophagus

Acidosis

Protein-energy malnutrition

I-cell disease (mucolipidosis type II)

Wilson-Konovalov disease (hepatocerebral dystrophy)

Gaucher disease (glucocerebroside lipidosis, glucocerebrosidosis)

Krabbe disease (globoid cell leukodystrophy)

Niemann-Pick disease (sphingomyelinosis)

Fabry disease

Gangliosidosis GM1 type I

Gangliosidosis GM1 type II

Gangliosidosis GM1 type III

Gangliosidosis GM2

Gangliosidosis GM2 type I (amaurotic idiocy of Tay-Sachs, Tay-Sachs disease)

GM2 gangliosidosis type II (Sandhoff's disease, Sandhoff's amaurotic idiocy)

Gangliosidosis GM2 juvenile

Gigantism

Hyperaldosteronism

Hyperaldosteronism secondary

Primary hyperaldosteronism (Conn's syndrome)

Hypervitaminosis D

Hypervitaminosis A

Hypervitaminosis E

Hypervolemia

Hyperglycemic (diabetic) coma

Hyperkalemia

Hypercalcemia

Hyperlipoproteinemia type I

Hyperlipoproteinemia type II

Hyperlipoproteinemia type III

Hyperlipoproteinemia type IV

Hyperlipoproteinemia type V

Hyperosmolar coma

Hyperparathyroidism secondary

Primary hyperparathyroidism

Hyperplasia of the thymus (thymus gland)

Hyperprolactinemia

Testicular hyperfunction

Hypercholesterolemia

Hypovolemia

Hypoglycemic coma

Hypogonadism

Hypogonadism hyperprolactinemic

Hypogonadism isolated (idiopathic)

Primary congenital hypogonadism (anorchism)

Primary acquired hypogonadism

Hypokalemia

Hypoparathyroidism

Hypopituitarism

Hypothyroidism

Glycogenosis type 0 (aglycogenosis)

Glycogenosis type I (Gierke's disease)

Glycogenosis type II (Pompe disease)

Glycogenosis type III (Measles disease, Forbes disease, limit dextrinosis)

Glycogenosis type IV (Andersen's disease, amylopectinosis, diffuse glycogenosis with liver cirrhosis)

Glycogenosis type IX (Haga's disease)

Glycogenosis type V (McArdle disease, myophosphorylase deficiency)

Glycogenosis type VI (Hers disease, hepatophosphorylase deficiency)

Glycogenosis type VII (Tarui disease, myophosphofructokinase deficiency)

Glycogenosis type VIII (Thomson's disease)

Glycogenosis type XI

Glycogenosis type X

Deficiency (insufficiency) of vanadium

Magnesium deficiency (insufficiency)

Manganese deficiency (insufficiency)

Copper deficiency (insufficiency)

Deficiency (insufficiency) of molybdenum

Deficiency (insufficiency) of chromium

Iron deficiency

Calcium deficiency (nutritional calcium deficiency)

Zinc deficiency (dietary zinc deficiency)

Diabetic ketoacidotic coma

Ovarian dysfunction

Diffuse (endemic) goiter

Delayed puberty

Excess estrogen

Involution of the mammary glands

Dwarfism (short stature)

Kwashiorkor

Cystic mastopathy

Xanthinuria

Lactic acidemic coma

Leucinosis (maple syrup disease)