Itsenko Cushing's disease (hypercorticism). Causes, symptoms, modern diagnosis and treatment of the disease. Cushing's syndrome (hypercorticism): causes, symptoms, treatment Obesity with symptoms of hypercorticism
Itsenko-Cushing syndrome- a pathological symptom complex resulting from hypercorticism, i.e., increased secretion of the hormone cortisol by the adrenal cortex or prolonged treatment with glucocorticoids. It is necessary to distinguish Itsenko-Cushing's syndrome from Itsenko-Cushing's disease, which is understood as secondary hypercortisolism that develops in the pathology of the hypothalamic-pituitary system. Diagnosis of Itsenko-Cushing's syndrome includes the study of the level of cortisol and pituitary hormones, dexamethasone test, MRI, CT and adrenal scintigraphy. Treatment of Itsenko-Cushing's syndrome depends on its cause and may consist in the abolition of glucocorticoid therapy, the appointment of steroidogenesis inhibitors, and the surgical removal of the adrenal tumor.
General information
Itsenko-Cushing syndrome- a pathological symptom complex resulting from hypercorticism, i.e., increased secretion of the hormone cortisol by the adrenal cortex or prolonged treatment with glucocorticoids. Glucocorticoid hormones are involved in the regulation of all types of metabolism and many physiological functions. The work of the adrenal glands is regulated by the pituitary gland by the secretion of ACTH - adrenocorticotropic hormone, which activates the synthesis of cortisol and corticosterone. The activity of the pituitary gland is controlled by the hormones of the hypothalamus - statins and liberins.
Such multi-stage regulation is necessary to ensure the coherence of body functions and metabolic processes. Violation of one of the links of this chain can cause hypersecretion of glucocorticoid hormones by the adrenal cortex and lead to the development of Itsenko-Cushing's syndrome. In women, Itsenko-Cushing's syndrome occurs 10 times more often than in men, developing mainly at the age of 25-40 years.
Hypersecretion of cortisol in Itsenko-Cushing's syndrome causes a catabolic effect - the breakdown of protein structures of bones, muscles (including heart), skin, internal organs etc., eventually leading to dystrophy and atrophy of tissues. Increased glucogenesis and absorption of glucose in the intestine causes the development of the steroid form of diabetes. Fat metabolism disorders in Itsenko-Cushing syndrome are characterized by excessive deposition of fat in some parts of the body and atrophy in others due to their different sensitivity to glucocorticoids. The effect of excess cortisol on the kidneys is manifested by electrolyte disorders - hypokalemia and hypernatremia and, as a result, an increase in blood pressure and aggravation of dystrophic processes in muscle tissue.
The heart muscle suffers the most from hypercortisolism, which manifests itself in the development of cardiomyopathy, heart failure and arrhythmias. Cortisol has a depressant effect on the immune system, causing patients with Itsenko-Cushing's syndrome to become prone to infections. The course of Itsenko-Cushing's syndrome can be mild, moderate and severe; progressive (with the development of the entire symptom complex in 6-12 months) or gradual (with an increase over 2-10 years).
Symptoms of Itsenko-Cushing's syndrome
The most characteristic symptom of Itsenko-Cushing's syndrome is obesity, detected in patients in more than 90% of cases. The redistribution of fat is uneven, according to the cushingoid type. Fat deposits are observed on the face, neck, chest, abdomen, back with relatively thin limbs ("colossus on clay feet"). The face becomes moon-shaped, red-purple in color with a cyanotic tinge (“matronism”). The deposition of fat in the region of the VII cervical vertebra creates the so-called "climacteric" or "buffalo" hump. In Itsenko-Cushing syndrome, obesity is distinguished by thin, almost transparent skin on the back of the palms.
On the part of the muscular system, muscle atrophy is observed, a decrease in muscle tone and strength, which is manifested by muscle weakness (myopathy). Typical signs that accompany Itsenko-Cushing's syndrome are "sloping buttocks" (reduction in the volume of the femoral and gluteal muscles), "frog belly" (hypotrophy of the abdominal muscles), hernia of the white line of the abdomen.
The skin of patients with Itsenko-Cushing's syndrome has a characteristic "marble" shade with a clearly visible vascular pattern, is prone to peeling, dryness, interspersed with areas of sweating. On the skin of the shoulder girdle, mammary glands, abdomen, buttocks and thighs, stripes of skin stretching are formed - striae of purple or cyanotic color, from a few millimeters to 8 cm long and up to 2 cm wide. Skin rashes (acne), subcutaneous hemorrhages, spider veins are observed, hyperpigmentation of certain areas of the skin.
With hypercortisolism, thinning and damage often develop bone tissue- osteoporosis, leading to severe pain, deformation and fractures of bones, kyphoscoliosis and scoliosis, more pronounced in the lumbar and thoracic spine. Due to compression of the vertebrae, patients become stooped and smaller in stature. In children with Itsenko-Cushing's syndrome, growth retardation is observed, caused by a slowdown in the development of epiphyseal cartilages.
Disorders of the heart muscle are manifested in the development of cardiomyopathy, accompanied by arrhythmias (atrial fibrillation, extrasystole), arterial hypertension and symptoms of heart failure. These formidable complications can lead to the death of patients. With Itsenko-Cushing's syndrome, the nervous system suffers, which is expressed in its unstable work: lethargy, depression, euphoria, steroid psychoses, suicidal attempts.
In 10-20% of cases, steroid diabetes mellitus develops during the course of the disease, which is not associated with lesions of the pancreas. Such diabetes proceeds quite easily, with a long-term normal level of insulin in the blood, quickly compensated by an individual diet and hypoglycemic drugs. Sometimes poly- and nocturia, peripheral edema develop.
Hyperandrogenism in women, accompanying Itsenko-Cushing's syndrome, causes the development of virilization, hirsutism, hypertrichosis, disorders menstrual cycle, amenorrhea, infertility. Male patients show signs of feminization, testicular atrophy, decreased potency and libido, and gynecomastia.
Complications of Itsenko-Cushing's syndrome
The chronic, progressive course of Itsenko-Cushing's syndrome with increasing symptoms can lead to the death of patients as a result of complications incompatible with life: cardiac decompensation, strokes, sepsis, severe pyelonephritis, chronic renal failure, osteoporosis with multiple fractures of the spine and ribs.
An emergency condition in Itsenko-Cushing's syndrome is an adrenal (adrenal) crisis, manifested by impaired consciousness, arterial hypotension, vomiting, abdominal pain, hypoglycemia, hyponatremia, hyperkalemia and metabolic acidosis.
As a result of a decrease in resistance to infections, patients with Itsenko-Cushing's syndrome often develop furunculosis, phlegmon, suppurative and fungal skin diseases. The development of urolithiasis is associated with osteoporosis of the bones and the excretion of excess calcium and phosphate in the urine, leading to the formation of oxalate and phosphate kidney stones. Pregnancy in women with hypercortisolism often ends in miscarriage or complicated delivery.
Diagnosis of Itsenko-Cushing's syndrome
If a patient is suspected of Itsenko-Cushing's syndrome on the basis of amnestic and physical data and exclusion of an exogenous source of glucocorticoid intake (including inhalation and intraarticular), the cause of hypercorticism is first of all clarified. For this, screening tests are used:
- determination of the excretion of cortisol in daily urine: an increase in cortisol by 3-4 times or more indicates the reliability of the diagnosis of Itsenko-Cushing's syndrome or disease.
- a small dexamethasone test: normally, taking dexamethasone reduces cortisol levels by more than half, and with Itsenko-Cushing's syndrome, there is no decrease.
Differential diagnosis between the disease and Itsenko-Cushing's syndrome allows a large dexamethasone test. In Itsenko-Cushing's disease, taking dexamethasone leads to a decrease in the concentration of cortisol by more than 2 times from the original; in the syndrome of low cortisol does not occur.
With the iatrogenic (medicinal) nature of Itsenko-Cushing's syndrome, a gradual abolition of glucocorticoids and their replacement with other immunosuppressants is necessary. With the endogenous nature of hypercortisolism, drugs that suppress steroidogenesis (aminoglutethimide, mitotane) are prescribed.
In the presence of a tumor lesion of the adrenal glands, pituitary gland, and lungs, surgical removal of neoplasms is performed, and if it is impossible, one- or two-sided adrenalectomy (removal of the adrenal gland) or radiation therapy of the hypothalamic-pituitary region is performed. Radiation therapy is often combined with surgical or medical treatment to enhance and sustain the effect.
Symptomatic treatment for Itsenko-Cushing's syndrome includes the use of antihypertensive, diuretic, hypoglycemic drugs, cardiac glycosides, biostimulants and immunomodulators, antidepressants or sedatives, vitamin therapy, drug therapy for osteoporosis. Compensation of protein, mineral and carbohydrate metabolism is carried out. Postoperative treatment of patients with chronic adrenal insufficiency who underwent adrenalectomy consists of continuous hormone replacement therapy.
Forecast of Itsenko-Cushing's syndrome
If the treatment of Itsenko-Cushing's syndrome is ignored, irreversible changes develop, leading to death in 40-50% of patients. If the cause of the syndrome was a benign corticosteroma, the prognosis is satisfactory, although the functions of a healthy adrenal gland are restored only in 80% of patients. When diagnosing malignant corticosteromas, the five-year survival prognosis is 20-25% (average 14 months). In chronic adrenal insufficiency, lifelong replacement therapy with mineral and glucocorticoids is indicated.
In general, the prognosis of Itsenko-Cushing's syndrome is determined by the timeliness of diagnosis and treatment, the causes, the presence and severity of complications, the possibility and effectiveness of surgical intervention. Patients with Itsenko-Cushing's syndrome are under dynamic observation by an endocrinologist, they are not recommended for heavy physical exertion, night shifts at work.
Itsenko-Cushing's disease- a disease resulting from lesions at the level of the brain in the hypothalamus or pituitary gland. This causes an increase in the production of ACTH (adrenocorticotropic hormone produced by the pituitary gland), which excessively increases the work of the adrenal glands (paired glands that lie on the upper side of the kidneys). In a person with this pathology, blood pressure rises, the skin becomes marble-colored, and the face becomes moon-shaped, crimson-red stripes appear in the abdomen and thighs, fat deposits occur in certain areas of the body, the menstrual cycle is disturbed, etc. The disease is 5-10 times more common in women than in men aged 20-40 years. It can occur in adolescents during puberty and sometimes in children and people over 50 years of age. The symptoms of the disease were first described in 1924 by the Odessa neuropathologist Nikolai Itsenko and in 1932 by the Canadian neurosurgeon Harvey Cushing. The first scientist suggested that the reason lies in the disruption of the hypothalamus, and the second - associated it with a pituitary tumor. It has now been proven that pathology occurs after failures in the functions of both brain structures.
How do the hypothalamus, pituitary and adrenal glands work?
In order to understand how the symptoms of Itsengo-Cushing's disease are related to the hypothalamus - pituitary - adrenal glands, we will talk a little about the anatomy and physiology of these body structures. Let's start with a few words:- A hormone is a biologically active substance produced by special organs or cells in one part of the body and regulating the activity of organs and tissues in other parts of the body.
- An endocrine gland (endocrine gland) is an organ that produces and releases hormones or other substances into the blood.
- The hypothalamus is a part of the brain weighing 4 g that controls metabolism, the work of the endocrine and gonads.
- The pituitary gland is an endocrine gland located at the base of the human brain and affects the growth, development, and metabolic processes of the body.
- The adrenal glands are paired human endocrine glands that lie on the upper side of the kidneys.
The hypothalamus regulates the following processes in the body:
- Feelings of thirst, hunger and satiety.
- Sleep and emotional states.
- sexual function.
- Respiration and blood supply.
- The processes of memorization and learning.
- Thermoregulation - a set of physiological processes aimed at maintaining a relatively constant temperature of the human body.
- Diuresis - the amount of urine excreted in a certain time. In humans, daily diuresis averages 1200-1600 ml.
The anterior lobe accounts for 75% of the total mass of the pituitary gland. It contains 3 types of cells:
- Acidophilic (eosinophilic) - cells contained in 30-35%, which are stained with acid dyes in red and Orange color.
- Basophilic - cover up to 10% of the total mass. Alkaline dyes are used to detect them. Some of the cells - corticotropocytes - produce ACTH (adrenocorticotropic hormone).
- Chromophobic - precursors of eosinophilic and basophilic cells. Their adenohypophysis contains 60%.
ACTH (adrenocorticotropic hormone, corticotropin) produced by the anterior pituitary gland. It stimulates the function of the adrenal cortex. Thus, it controls metabolic processes and increases the resistance of the human body under the influence of adverse conditions. In addition, it contributes to the formation of melanin, a pigment that gives the skin a brownish tint. The production of ACTH by the pituitary gland is under the action of a special releasing factor of the hypothalamus - corticoliberin. Under its influence, there is an increase in the formation of corticotropin.
adrenal glands are laid at 6-8 weeks of gestation. Their final formation occurs in the third year of life. They have different shape: the left one is presented in the form of a crescent, the right one - in the form of a pyramid. The size of each is approximately 6 × 3 × 1 cm, weight - about 4-5 g. In the adrenal glands, the surface (cortical layer) and internal (medulla) parts are isolated. Both glands are encased in a connective tissue capsule. The cortical layer is divided into 3 areas:
- Glomerular (outer) zone - occupies 15% of the cortex, forms mineralocorticoids.
- The bundle (middle) zone - makes up 75% of the cortical layer, produces glucocorticoids and a small amount of androgens.
- The reticular (lower) zone - produces androgens, a little estrogens and glucocorticoids.
- Androgens (androstenedione, dehydroepiandrosterone, testosterone, etc.),
- Estrogens (estrogen, estradiol, estrone, etc.),
- Mineralocorticoids (aldosterone, deoxycorticosterone),
- Glucocorticoids (corticosterone, hydrocortisone, cortisol).
Each of the above groups performs its functions:
- Androgens - in women and men develops secondary sexual characteristics, libido.
- Estrogens - stimulate the growth and development of female genital organs and secondary sexual characteristics in women.
- Mineralocorticoids - retain water in the body (in the space between cells).
- Glucocorticoids - contribute to the decomposition of proteins and fats into simpler components, reduce the absorption of calcium from the intestines and the body's immunity, increase arterial pressure, increase the secretion of gastric juice, forming "steroid" ulcers.
In addition, one of the important functions is the control of the hormone of the hypothalamus - corticoliberin. The high content of glucocorticoids, namely cortisol in the blood, delays the production of corticoliberin, in turn, it reduces the formation of ACTH in the pituitary gland. Ultimately, ACTH inhibits the work of the adrenal cortex, and the content of cortisol in the blood decreases. If there are few glucocorticoids in the blood, then the whole process described above will go the other way around. The hormone of the hypothalamus will increase, it in turn will increase the production of ACTH, and the latter will increase cortisol in the blood. This is the mechanism of regulation of the hypothalamic-pituitary-adrenal system.
Causes of Itsenko-Cushing's disease
It is often difficult to find out the source of the disease. But over the course of many years of practice of highly qualified specialists, several factors have been identified. First of all, these are periods of rapid restructuring of the body, which can cause damage at the level of the hypothalamus or pituitary gland: puberty, pregnancy, childbirth, menopause. Secondly, severe bruises, severe craniocerebral and mental injuries of the skull, various infections of the brain and intoxication (poisoning) of the body contribute to the appearance of this pathology. Thirdly, scientists consider the tumor (adenoma) formed from basophilic and chromophobic cells of the anterior pituitary gland to be the main cause. In people suffering from Itsego-Cushing's disease, it occurs in 85%.Each of these factors can trigger the following mechanism: corticoliberin (hormone of the hypothalamus) begins to be produced in large volumes, this causes increased production of ACTH (the pituitary hormone), and the latter increases the work of the adrenal cortex by 4-5 times. Normally, elevated blood levels of cortisol (adrenal hormone) should reduce the formation of corticoliberin. But the reason that caused the disease does not allow it to be done. As a result, all hormones continue to be synthesized in large quantities.
Symptoms of Itsenko-Cushing's disease
There are many clinical signs of this pathology. In order to remember everything and not to miss anything, we will consider them in stages for all body systems.Leather. The skin becomes dry, purple-marble, flaky. In places of strong friction, increased pigmentation is noted. In the area of the buttocks, thighs, abdomen, mammary glands, armpits, wide bands of blue-violet to dark red shades stand out. Their appearance is associated with the destruction of proteins under the influence of increased production of glucocorticoids (adrenal hormones). The skin thins to the point where blood vessels are visible. The vessels themselves are breaking, that any impact on them causes damage. For this reason, the patient can see bruises and wounds of various sizes and depths, which take a very long time to heal. It should be noted 2 important symptoms - hirsutism and virilism - both develop as a result of an increased content of androgens. Hirsutism is male-pattern hair growth in women. Mustaches, a beard, vegetation on the chest and abdomen appear.
virilism- a phenomenon when the face and body of a person, regardless of gender, is covered with a large amount of hair, exceeding the norm by several times. And on the head they fall out and form bald patches. Often on the body you can notice pustular rashes, ulcers and boils (inflammation of the sweat and sebaceous glands of the skin).
Subcutaneous adipose tissue and obesity. Obesity is one of the main symptoms and occurs in 95% of cases. Its redistribution throughout the body is not the same. The face acquires a rounded shape resembling the moon ("moon face"). Another distinguishing feature may be the deposition of fat in the region of the seventh cervical vertebra in the form of a small mound ("climacteric hump" or "buffalo type"). This vertebra, or rather its process, can be felt at the back of the neck (the most protruding part), if you tilt your head strongly forward. A significant thickening of the subcutaneous fat layer also occurs in the abdomen and chest. All these signs are explained by the action in the increased content of cortisol (adrenal hormone), which contributes to the accelerated formation of lipids (fats) in the body.
Bone system. With Itsengo-Kushin's disease, the skeletal system is affected in 95%. The increased content of glucocorticoids (adrenal hormones), on the one hand, destroys the proteins that are the basis of the bone, and on the other hand, reduces the absorption of calcium in the intestine. Thus, calcium, which gives strength to the skeletal system, enters it in small quantities. As a result, a disease called osteoporosis develops. It covers mainly the bones of the face, skull, spine, feet and hands. There is a change in their size and shape, which is most often accompanied by severe fractures and severe pain. In adults, there is a decrease in growth by 10-20 cm, and in children - its complete delay.
Muscular system. As in the previous case, an increased amount of glucocorticoids also destroys muscle proteins. This leads to weight loss of the arms and legs, weakness appears in them to such an extent that the patient cannot lift a small load or stand up without someone else's support.
Respiratory system. Immunity is reduced under the influence of adrenal hormones. The body becomes susceptible to all infections. Against this background, bronchitis (inflammation of the bronchi), pneumonia (a disease that affects the lungs and small bronchi), tuberculosis (an infectious disease, often chronic, caused by a special Koch bacterium and affecting the lungs, bones, joints, intestines and other organs) develop.
The cardiovascular system. One of the first signs of Itsenko-Cushing's disease in 95% of cases is an increase in blood pressure. If measures are not taken to lower it to normal, a person may develop heart failure. It is characterized by the following: the heart, contracting less often, slows down the movement of blood through the vessels of the body, and all organs and tissues begin to experience a lack of oxygen necessary for their work. This condition of the patient can lead to death.
Digestive system. With this disease, there is heartburn (burning) in the esophagus, "steroid" ulcers of the stomach and the initial part of the intestine, caused by glucocorticoids. The liver also suffers: it cannot perform all its proper functions.
Urinary system. There are several causes that create problems in the kidneys and urinary tract. One of them - reduced immunity increases their susceptibility to infections. Pyelonephritis is formed - a disease that affects the kidneys and is characterized by high body temperature and back pain. The second reason is the leaching of calcium from the bones, which enhances its excretion in the urine. This contributes to the formation of stones in the urinary system and the development of urolithiasis. The third factor - high blood pressure destroys the kidneys in such a way that connective tissue grows in their place. This is how nephrosclerosis develops, which raises the pressure. Formed vicious circle and all processes are repeated one after another, worsening the general condition of the body.
Nervous system and psyche. In previous systems, osteoporosis was described, which destroys the bones, presses on the nerves passing near them. This action causes such severe pain, especially in the upper parts of the arms and legs, that the patient cannot make the slightest movement. On the part of the psyche, there is a decrease in memory and intelligence, frequent depression, increased emotionality, or vice versa - indifference to everything around. With a long course of the disease with a significant deterioration, thoughts of suicide appear.
Endocrine and reproductive systems. In 35-40% of patients, "steroidal" diabetes mellitus caused by glucocorticoids is observed. Diabetes mellitus is a disease resulting from endocrine disorders that increase blood sugar and disrupt the metabolism of proteins, fats, carbohydrates, minerals, water and salts. With Itsenko-Cushing's disease, both sexes experience a decrease in libido. In women, in 70% of cases, there is no menstruation, the organs themselves (uterus, ovaries) decrease in size. Pregnancy in such situations is almost impossible. And in the case when it happens, it always ends in a miscarriage. If this pathology manifested itself in childhood, sexual development child stops.
Diagnosis of Cushing's disease
- To make a diagnosis of Itsenko-Cushing's disease, our first step will be to assess the presence of the symptoms listed above. The most common of them: obesity, high blood pressure, specific hair growth and skin coloration, osteoporosis and pain, menstrual disorders and decreased libido, mental disorders. This pathology has several forms of manifestation of symptoms: mild (symptoms are mild), moderate (most symptoms can be detected, but complications will not be observed), severe (all symptoms with complications). According to the course of the disease, two forms are distinguished: progressive (develops for several months) and torpid (lasts for several years).
- The second step will be the study of blood and urine different methods, where an increased content of hormones from the hypothalamus - pituitary gland - adrenal glands and their end products of decomposition will be found.
- The third is examination methods that should confirm Itsenko-Cushing's disease and exclude all other pathologies, which are very similar to it. These include: radiography of the bones of the skull, spine, limbs; magnetic resonance imaging (MRI) and computed tomography (CT) of the brain - can confirm the destruction of the skeletal system and the presence of a pituitary tumor. Ultrasound, MRI, CT, angiography, radioisotope imaging of the adrenal glands will show an increase in their size and assess the degree of functioning of each layer. Diagnostic tests with metopyrone and dexamethasone will finally eliminate doubts about the correctness of the diagnosis.
Treatment of Itsenko-Cushing's disease
There are two types of treatment: etiopathogenetic (eliminates the cause of the disease and restores the normal functioning of the hypothalamus - pituitary - adrenal glands) and symptomatic (correction of the work of all affected organs).- Treatment causative factor diseases can be divided into surgical, radiation and medical methods, which can be combined or used individually, depending on the form and severity of the disease.
- Of the surgical ones, adrenalectomy is distinguished (unilateral or bilateral removal of the adrenal glands), destruction of the adrenal glands (by introducing a contrast agent that destroys their structure) and excision of the pituitary tumor.
- From radiation - use X-ray, gamma and proton therapy.
- The drug method includes two groups of drugs: dopamine receptor agonists (bromocriptine (parlodel), reserpine, difenin, cyproheptadine), and antitumor agents, steroid synthesis inhibitors (chloditan, elipten, mitotane, mamomit). The first group of drugs activates dopamine receptors nervous systems s, and the hypothalamus begins to "understand" that there are too many corticosteroids in the blood, so it needs to reduce the release of its corticoliberin in order to reduce the work of the pituitary gland, which depresses the function of the adrenal glands. The second - delays the synthesis of all hormones of the adrenal cortex.
- symptomatic treatment. In the presence of diabetes, they follow a strict diet and take drugs that reduce blood sugar - bigunides and sulfonamides. In severe cases, use insulin. With arterial hypertension, antihypertensive substances of central action (reserpine, adelfan, etc.) and diuretic drugs are prescribed. In heart failure, cardiac glycosides or digitalis preparations are required. For the treatment of osteoporosis, vitamin D, calcitonins, bisphosphonates are used, which restore the protein structure of the bone, increase calcium absorption from the intestine and thereby increase its inclusion in protein matrices. Fluorine salts and anabolic steroids increase bone formation. With a decrease in immunity, drugs are prescribed that increase it due to increased growth and maturation of the main components - lymphocytes.
What is the difference between Cushing's disease and Cushing's syndrome?
Cushing's syndrome and Itsenko-Cushing's disease are often confused not only because of the similar name, but also because of very similar symptoms. However, these concepts in medicine are far from identical. In both cases, we are talking about hypercortisolism - increased secretion of hormones from the cells of the adrenal cortex ( primarily cortisol.). It is these hormones that begin to affect various organs and entire systems, change metabolism ( metabolism of certain substances). These changes lead to the appearance of certain symptoms. Cushing's syndrome is understood as the totality of all changes that occur in the body with increased release of corticosteroids, as well as the external manifestations of this disorder. From the point of view of medicine, this concept coincides with the concept of "hypercorticism". Cushing's syndrome can have various causes. We are talking about any internal or external effect on the body that stimulates the production of corticosteroids.
The causes of Cushing's syndrome may be the following disorders:
- hormone-producing adrenal gland tumor;
- paraneoplastic syndrome ( rare, with tumors of the lungs, mediastinum and some other organs);
- long-term use of large doses of corticosteroid drugs - the so-called drug hypercorticism;
- pituitary tumor ( Cushing's disease).
In Itsenko-Cushing's disease, the effect on the cells of the adrenal glands occurs as follows:
- The hypothalamic-pituitary system in the brain normally produces the so-called adrenocorticotropic hormone (ADH). ACTH). With Itsenko-Cushing's disease in the pituitary gland ( the pineal gland of the brain that controls the endocrine system) a microtumor is formed, which increases the release of ACTH.
- ACTH enters the bloodstream and acts on cells in the adrenal cortex. With an excess of this hormone, cells increase in volume and number.
- Spurred on by adrenocorticotropic hormone, the adrenal cortex produces more corticosteroid hormones and hypercortisolism occurs ( Cushing's syndrome).
- An increase in blood corticosteroid levels should be picked up by the pituitary gland and cause a decrease in ACTH production ( feedback mechanism). However, due to a tumor or pathological changes in the gland, this does not happen.
- Gradually increases both the level of ACTH and the level of corticosteroids in the blood. They act on target cells, causing disturbances in the functioning of various organs and systems and leading to the appearance of characteristic symptoms.
Differential diagnosis of the causes of hypercortisolism
| Criterion | Itsenko-Cushing's disease | Other Causes of Hypercortisolism (Cushing's syndrome) |
| Blood ACTH level | Increased due to pathological changes in the pituitary gland. | Normal or reduced ( because the feedback mechanism can operate). |
| Instrumental Research | Magnetic resonance imaging ( MRI) often reveals tumors or hyperplasia ( cell proliferation) pituitary gland. | CT scan ( CT), MRI and X-ray reveal hyperplasia or tumor of the adrenal glands, but no changes in the pituitary gland. |
| Large dexamethasone test | The level of corticosteroids after the introduction of dexamethasone is reduced by more than half, compared with the original. | The introduction of dexamethasone does not cause a pronounced decrease in the level of corticosteroids. |
| Influence of other factors | Unable to identify the influence of other factors ( besides ACTH) on the adrenal glands. | Careful examination reveals other tumors ( causing paraneoplastic syndrome) or any other causes of the syndrome. |
Thus, Itsenko-Cushing's disease is only one of the possible causes of Cushing's syndrome. These concepts cannot be identified, since there are differences in the mechanism of their development, diagnosis and treatment tactics, although the external manifestations in both cases completely coincide.
Does hypercortisolism occur in children?
Hypercortisolism ( Cushing's syndrome) in children is considered a fairly rare disease. Its cause in childhood and adolescence hyperplasia becomes more frequent ( increase and increase in activity) adrenal glands or incorrect intake of corticosteroid drugs ( drug-induced hypercortisolism). Itsenko-Cushing's disease, which is also a variant of hypercortisolism, is very rare in childhood and adolescence. Cushing's syndrome is a significant increase in the concentration of corticosteroid hormones in the blood. These hormones regulate many metabolic processes in the body and can affect the functioning of a variety of organs. In childhood, when many tissues of the body are still at the stage of growth and development, hypercortisolism is especially dangerous.
Most often, doctors or doctors can reduce the level of corticosteroid hormones with medication or surgery. However, if their action was long enough, some complications and residual effects may develop, which will affect health and life in general in the future. That is why it is believed that hypercortisolism in children is more severe, although statistically lethal outcome in children and adolescents is less common than in adults.
The consequences of hypercortisolism in children may be the following disorders:
- Bone curvature. The hormone that causes major problems in the body in Cushing's syndrome is cortisol. Under its influence, calcium begins to be washed out of the bones ( osteoporosis develops). Because of this, the bone tissue loses its strength. The growth centers that are in the bones of children and adolescents disappear, and the bones themselves become more fragile. Usually flat bones are affected first ( skull bones, pelvic bones ) and only in severe cases - tubular ( limb bones). In the midst of the disease, cases of bone fractures are not uncommon. Due to poor absorption of calcium, these fractures heal slowly and the bones may not heal properly. Therefore, people who suffered from hypercortisolism in childhood may show visible cosmetic defects associated with bone curvature.
- growth retardation. Growth retardation is also associated with osteoporosis, described above. The problem is that not only flat and tubular bones stop growing, but also the vertebrae. No urgent effective treatment they can change their shape, shrink, causing pain associated with pinching of the spinal nerves. Since the full treatment of hypercortisolism and the normalization of metabolic processes in the body usually require a certain amount of time ( months, sometimes years, depending on the cause of Cushing's syndrome), children begin to lag far behind their peers in growth.
- Morbid obesity. Under the influence of cortisol, there is a redistribution of fatty tissue in the body. The child begins to gain weight despite the stunting). Obesity can remain after the normalization of hormone levels and present certain problems in the future. Muscle at the same time, it gradually atrophies, due to which the limbs become somewhat thinner.
- Skin problems. Excess corticosteroids can cause dry skin, flaking, and acne in adolescents. In severe cases, more serious infectious processes develop ( pyoderma etc.). As a rule, these diseases do not pose a serious danger to health or life, but they can affect the psychological state of a child and, especially, a teenager.
- Psycho-emotional disorders. In part, their appearance is facilitated by cosmetic defects that accompany the course of the disease. On the other hand, with hypercortisolism, there is also a direct effect of hormones on the central nervous system. A number of patients develop depression, depression, and sometimes thoughts of suicide ( more typical for teenagers). In these cases, the intervention of a psychologist in the treatment process is not enough. It is important to reduce the level of hormones, because without this psychological help will not have the desired effect.
- Puberty disorders. Patients with hypercortisolism often develop excessive and early hair growth. Moreover, hair grows in a male pattern. With a high concentration of hormones and the absence of treatment, the development of hirsutism is possible. This syndrome consists in the growth of male-type hair in girls ( on the upper lip, chin, chest, back). Hypertrophy may be seen increase) genital organs - clitoris and penis. At the same time, puberty itself is often delayed or, conversely, accelerated.
Treatment of children with hypercortisolism requires special attention. They are usually handled by a pediatric endocrinologist. The main directions in treatment are similar to those in adults. However, accompanying psychological support should be provided. For a long time limit sports and exercise in general. The fact is that they increase the risk of fractures or bone deformities, slow down the growth of the child. Timely diagnosis and intervention of doctors, as well as a properly selected course of treatment, can prevent complications or residual effects in the future.
What is the diet for Itsenko-Cushing's disease?
Itsenko-Cushing's disease is characterized by an increase in the blood level of corticosteroid hormones ( cortisol and others), which cause significant changes in the functioning of various organs and tissues. Diet therapy cannot greatly affect the production of these hormones, so its role in treatment is often underestimated. However, in many cases it is dietary changes that allow patients to improve their health. This is due to the fact that cortisol affects the metabolism of various substances. This can cause various complications, which lead to the appearance of severe symptoms of the disease. Compliance with a certain diet partly regulates metabolism. Thus, diet therapy can be considered a symptomatic treatment of complications, although it does not directly affect hormonal disruptions in Itsenko-Cushing's disease. A specific diet should be followed in case of the following complications:
- Osteoporosis and urolithiasis ( stones in the kidneys) . Both of these complications are associated with leaching of calcium from bone tissue ( demineralization) and its accumulation in the renal pelvis after blood filtration. To normalize calcium levels, it is recommended not to consume saturated fatty acids ( main sources - margarine, butter, mayonnaise). The proportion of foods containing vitamin D should be increased, since it is it that promotes the absorption of calcium. Such products are eggs, dairy products, cheese. The use of cottage cheese, milk and other foods rich in calcium will have an ambiguous effect. On the one hand, it can strengthen bones, on the other hand, it can accelerate the formation of kidney stones.
- Obesity. Excess cortisol affects the formation of adipose tissue and electrolyte balance, contributing to edema. To alleviate the condition, it is recommended to reduce the amount of sodium consumed ( First of all, salt), animal fats, and in severe cases, fluids ( up to 1.5 - 2 liters per day).
- peptic ulcer. Diet for peptic ulcer caused by hypercortisolism ( elevated levels of corticosteroids), does not differ from the diet for ulcers of other origins. Frequent fractional meals are recommended. When choosing products, you should focus on table number 1, 1a and 1b according to Pevzner. He excludes rich, salty, sour, smoked or fatty foods, as they stimulate the secretion of gastric juice and aggravate the course of the disease. Also, you can not drink strong tea, coffee, alcohol. During an exacerbation, boiled meat, cereals, low-fat soups and other light foods are recommended.
- Heart failure. In heart failure, the goal of the diet is to normalize the volume of circulating blood. To alleviate the symptoms, you should “unload” the heart, which cannot cope with its work. Patients need to follow the treatment table number 10 according to Pevsner. First of all, limit salt intake ( up to 1.5 - 2 g per day) and water ( up to 1 - 1.5 liters per day).
- steroid diabetes. Steroid diabetes occurs due to disturbances in the production of insulin and insensitivity of tissue receptors to the action of this hormone. The basic principles of diet therapy for this disease correspond to table number 9 according to Pevzner. The patient's diet should be slightly increased in protein content ( 100 - 110 g), but the proportion of carbohydrates is reduced ( no more than 200 g) and fats ( no more than 40 g).
It should be understood that with Itsenko-Cushing's disease, all of the above processes ( calcium leaching, electrolyte disturbances, ulcer formation, and increased sugar levels) occurs simultaneously, although the patient may not have as many symptoms. In this regard, to prevent possible complications, the final diet is discussed with the attending physician. Usually it is focused on the complication that is most pronounced in a particular patient.
Also, in parallel with dieting, it is necessary to fight the increased level of hormones with medicinal or surgical methods. Without eliminating the underlying cause of the disease, diet therapy will only be a temporary measure that will allow for some time to eliminate the symptoms and manifestations of the disease.
Is it possible to treat hypercortisolism with folk remedies?
Hypercortisolism is an increase in the level of corticosteroid hormones in the blood. In about 70% of cases, this syndrome is caused by Itsenko-Cushing's disease or improper use of hormonal drugs. Often, tumors in the pituitary gland are the root cause of the disease ( iron in the brain), adrenal cortex or other organs. Eliminate such a serious pathology only by methods traditional medicine impossible. This requires a serious and long course of drug treatment, and often surgery. However, traditional medicines can sometimes help to relieve the symptoms and manifestations of the disease. Certain medicinal plants can, for example, lower blood sugar levels in steroid diabetes, relieve stomach ulcers, improve heart function in heart failure. However, all these measures will be only auxiliary. Traditional medicine is much weaker in effect than modern pharmaceuticals.
For auxiliary treatment for hypercortisolism, the following folk remedies can be used:
- Sea buckthorn juice. Drink 40 - 50 ml an hour before meals ( three times a day). Due to its enveloping properties, it protects the gastric mucosa from the action of gastric juice, relieves the symptoms of peptic ulcer.
- Sea buckthorn oil. The mechanism is similar to the action of sea buckthorn juice. It is also taken 3 times a day before meals, but 0.5 - 1 teaspoon.
- Kefir. Before going to bed, it is recommended to drink 1 glass of fresh kefir at room temperature. Add a tablespoon before use. vegetable oil and mix well. The tool promotes healing of ulcers within 30 - 40 days. However, for a good effect, hypercortisolism, the cause of their formation, should also be fought.
- Linden honey. Just like sea buckthorn juice, it has enveloping properties and protects the gastric mucosa. To relieve peptic ulcer, it is enough to drink 3 cups of warm boiled water a day, in which 1 tablespoon of honey is dissolved.
- Radish juice with honey. Used for urolithiasis kidney stones). Freshly squeezed radish juice is mixed with honey in equal proportions. The remedy is taken 1 teaspoon three times a day ( before meals).
- watermelon rinds. Dried watermelon peels are crushed, poured with boiling water and continue to simmer for another 20-25 minutes. The remedy is drunk chilled to room temperature, 1 glass three times a day. For 1 - 2 weeks of admission, the symptoms of gallstone disease are removed.
- Potato poultices. Used for severe back pain renal colic). After easing the pain, you need to take a warm bath, take a horizontal position and put on your back ( in the area of the kidneys) warm squashed boiled potatoes ( do not peel off).
- Infusion of garlic. It is used in steroid diabetes to lower sugar levels. A few cloves of garlic are rubbed, poured with boiling water ( half liter) and insist for 20 - 30 minutes. The infusion is drunk half a cup 2-3 times a day in small sips.
- Buckwheat. Also effective in lowering sugar levels. The grains are crushed ( in a coffee grinder or mortar) and pour kefir. For 200 ml of kefir, 3-4 full teaspoons of chopped buckwheat are needed. The remedy should be taken twice a day half an hour before meals. For a visible effect, treatment is continued for at least 2 weeks.
- Infusion of blueberries. For 4 teaspoons of dried berries, you need 1 cup of boiling water. Infusion lasts 8 - 10 hours, after which the infusion is drunk during the day in small portions. This medicine lowers blood pressure often elevated in hypercortisolism) and protects the retina from degeneration due to diabetes. In addition, a large amount of vitamins from blueberries will strengthen weakened immunity.
- Infusion of rowan fruits. For 1 tablespoon of dried fruits, you need a glass of boiling water. The infusion lasts until the water cools to room temperature. The remedy is taken three times a day for half a cup. The action is similar to the infusion of blueberries ( without such pronounced protection of the retina).
Why is hypercortisolism dangerous?
Hypercortisolism is a very serious disease, which under certain conditions can lead to the death of the patient. The problem is that the adrenal glands start to produce too much corticosteroid hormones ( mainly cortisol). These substances in excess can seriously disrupt the functioning of various organs and systems in the body. The longer the period of hypercortisolism lasts ( due to late diagnosis or incorrect treatment), the greater the risk of serious complications. Hypercortisolism is dangerous for the following reasons:
- Violation of calcium metabolism. Excess cortisol affects the absorption of calcium by the body. Because of it, this substance is not absorbed by the bones, and with prolonged hypercortisolism it is even washed out of the bone tissue. This leads to the development of osteoporosis, a disease in which the patient's bones become more fragile. Excess calcium travels through the bloodstream to the kidneys, where it often contributes to the formation of stones ( urolithiasis). Osteoporosis and urolithiasis are rarely fatal ( there is a risk only with the addition of an infection and the absence of medical care), but often cause disability.
- Increase in glucose levels. Under the influence of cortisol, insulin production worsens, and blood sugar levels begin to rise. This condition is called steroid diabetes. It is not as severe as ordinary diabetes mellitus, but still, without adequate treatment, it can greatly worsen the patient's condition. In particular, small vessels are affected, blood supply to tissues worsens, and the work of the nervous system is affected. It also increases the risk of a heart attack.
- Stunted growth in children and adolescents. Growth retardation is associated mainly with osteoporosis of the vertebrae. Normally, bones should grow and gain strength, however, against the background of hypercortisolism, they often break, bend, which can lead to irreversible deformities after the disease itself is cured. In addition, children and adolescents often suffer from obesity. Long-term treatment leaves an imprint on the development of the psyche.
- Damage to the cardiovascular system. Patients with hypercortisolism most often develop hypertension ( increased blood pressure). With concomitant atherosclerosis or other vascular and heart diseases, this greatly increases the risk of myocardial infarction or the development of cardiomyopathy. The result can be chronic heart failure and heart rhythm disturbances. Complications related to the work of the heart are the most common causes of death in hypercortisolism.
- Gastrointestinal tract injury gastrointestinal tract) . At the level of the gastrointestinal tract, a number of serious complications can develop, the most common of which are gastritis and peptic ulcer. Since these diseases are characterized by structural changes in the mucous membrane of the organ, they will not go away even after a decrease in the level of corticosteroid hormones. The immediate danger lies in the fact that, without the necessary treatment, the ulcer may be complicated by perforation ( perforation of the stomach wall), peritonitis ( inflammation of the peritoneum) and other surgical problems.
- Immune suppression. Cortisol in large quantities can have a depressing effect on immune system. The danger lies in the fact that the body becomes more susceptible to various infectious diseases. The person becomes sickly, often catches a cold. The problem is that with a weak immune system, even a common cold can lead to pneumonia and pose a threat to life.
Thus, hypercortisolism can be considered a very dangerous disease. This is due to multiple disorders and the need for long, difficult treatment. Statistically, almost half of patients die within the first 5 years of diagnosis. Most often this is due to the severe course of the disease ( for example, Itsenko-Cushing's disease, not responding to treatment) or errors in diagnosis and treatment.
What is drug-induced hypercortisolism?
Medical ( or iatrogenic) hypercortisolism is a disease caused by excessive or improper use of medications based on corticosteroid hormones. This disease is characterized by an increase in the level of these hormones in the blood and the development of Cushing's syndrome. By and large, drug-induced hypercortisolism can be considered side effect or a possible complication from the use of certain pharmacological drugs. This disease is most often associated with taking the following drugs:
- prednisolone;
- hydrocortisone;
- betamethasone;
- dexamethasone.
Cushing's syndrome, which combines the manifestations of hypercortisolism, develops gradually. The higher the dose of drugs entering the body, the faster the disease will manifest. As a rule, the highest doses come when they are administered as injections ( injections) or inside ( in tablets or capsules). It is important that these high doses are taken regularly over a period of several months. During this period, there are corresponding changes in the hypothalamic-pituitary system and adrenal glands. Hormones, on the other hand, begin to have a pronounced effect on other systems, causing the development of drug-induced hypercortisolism.
Long-term treatment with corticosteroid drugs is usually prescribed for the following diseases:
- chronic hirsutism, virilism), obesity develops, osteoporosis ( demineralization of bones with their gradual weakening). Other organs and systems are also affected.
The problem is that in some cases, when no medical errors were made, and the patient strictly followed the specialist's instructions, it is still not possible to cancel corticosteroid drugs. They hold back the development of another, more dangerous disease. In these cases, one has to look for compromise doses or prescribe symptomatic treatment. Patients are managed by endocrinologists. At the first symptoms of hypercortisolism, it is checked for other causes ( Itsenko-Cushing's disease, tumors of the adrenal glands). The dose of corticosteroid drugs that the patient is taking is recalculated and rechecked.
What is the prognosis for Itsenko-Cushing's disease?
Itsenko-Cushing's disease is a rather serious disease in which it is difficult to make a specific prognosis for the patient. This is due to the fact that the hormones of the adrenal cortex, the level of which is elevated in this disease, can cause disturbances in the work of various organs and systems. With a severe course of the disease and the absence of qualified treatment, it may well end in death or serious irreversible changes in the patient's body. At the same time, a timely appeal to a specialist often helps to save not only life, but also the ability to work. It is statistically shown that mortality among patients with Itsenko-Cushing's disease is about 40 - 50% within 5 years ( in the absence of treatment). Most often this is due to the gradual development of diabetes mellitus, suppression of the immune system and disorders in the functioning of the cardiovascular system. In other words, the most severe consequences are observed not from Itsenko-Cushing's disease itself, but from secondary developed diseases.
The development of the following complications can greatly affect the prognosis for the patient:
- Osteoporosis. Caused by leaching of calcium from bone tissue. It can lead to chronic pain, contributes to frequent bone fractures, curvature of the spine. May lead to disability.
- Heart failure. Caused by irreversible changes in the heart muscle. Left untreated, it can lead to disability and even death.
- Stomach ulcer. Caused by metabolic disorders at the level of the gastric mucosa. Causes chronic epigastric pain central upper quadrant of the abdomen), periodic bleeding, digestive problems.
- Obesity. Increases the risk of diseases such as heart attack or stroke. Reduces the quality of life of patients.
- Mental changes. A long-term increase in cortisol levels often leads to the development of depression in patients.
- Weakened immunity. Leads to frequent pustular skin lesions, which are a cosmetic defect. Also, patients with Itsenko-Cushing's disease more often than ordinary people suffer from infectious diseases.
Regarding the prognosis for children, it can be noted that for them the period of onset of irreversible changes is shorter. This is due to the rapid growth of the body in childhood and accelerated metabolism. The leaching of calcium from the bones, for example, leads to stunted growth and curvature of the bones. This creates a serious cosmetic defect that cannot be corrected in the future. In addition, inhibition of the development of the spine or chest can affect the functioning of internal organs. With a prolonged increase in corticosteroid levels, it is also possible to slow down mental development. In children, all the same complications can occur as in adults, but they usually come on faster.
In general, the following factors influence the prognosis for Itsenko-Cushing's disease:
- Patient's age. As mentioned above, children have a number of serious complications that are more common than adults. For them, the prognosis is usually worse.
- Development of complications. Serious complications can lead to disability or death of the patient. Of course, their development worsens the prognosis.
- Timely treatment. The sooner the diagnosis is made, the shorter the period of hypercortisolism. Hormones do not have time to have a significant impact on the body and lead to irreversible changes in tissues. This improves the prognosis.
- Type of neoplasm. Most often, Itsenko-Cushing's disease is caused by neoplasms in the pituitary gland ( iron in the brain). The type of formation, its size and location predetermine the method of treatment ( surgical removal, symptomatic treatment).
- Treatment tactics. Symptoms of hypercortisolism in Itsenko-Cushing's disease can be eliminated in several ways. They largely affect the prognosis for the patient. For example, surgical removal of pituitary tumors is associated with certain risks during surgery ( complications occur in 2-3% of cases, and postoperative mortality reaches 1%) and a difficult postoperative period, but the disease can be completely defeated. Removal of the adrenal gland adrenalectomy) reduces hormone levels, relieving symptoms, but is only a partial solution to the problem. If it is impossible to fight the disease, the patient will have to constantly take medications for symptomatic treatment ( blood pressure lowering, blood sugar lowering, etc.).
- Compliance with doctor's orders. Treatment of Itsenko-Cushing's disease can last for months and even years. During this period, the patient must strictly follow all the prescriptions and recommendations of the doctor, regularly come for consultations and take tests, take a large number of different medications. In this case, we are talking about cases where the problem cannot be quickly solved by surgery. Interruption of treatment, even for a short time, again leads to an increase in cortisol levels, the return of symptoms and the risk of severe complications.
What are the complications of Itsenko-Cushing's disease?
Itsenko-Cushing's disease is manifested by the so-called Cushing's syndrome or hypercortisolism. This syndrome includes a variety of symptoms and manifestations caused by elevated levels of cortisol in the blood. The problem is that prolonged exposure to this hormone can cause serious changes in the structure and function of various organs and tissues. Because of this, disorders that were previously regarded as symptoms turn into full-fledged independent complications of the disease. Some of them do not go away even after the cure of Itsenko-Cushing's disease itself and require a separate course of treatment. It is the development of complications that becomes the most common cause of disability or even death in patients with this disease. The most serious complications of this disease are:
- Diabetes. Against the background of Itsenko-Cushing's disease, the so-called steroid diabetes mellitus may develop. This usually occurs in 10-20% of patients. The disease proceeds much easier than ordinary diabetes mellitus. Glucose levels can often be controlled with the right diet, without regular medication. However, in advanced cases, the level of sugar can rise greatly, leading to serious violations. After fixing the problem neoplasms in the pituitary gland) and curing Itsenko-Cushing's disease, the sugar level may still fluctuate for some time, but gradually stabilizes.
- Osteoporosis. Osteoporosis is one of the most common complications, the signs of which are found in almost 90% of patients. Under the action of hormones, calcium is washed out of the bones, and they lose strength. In this regard, fractures and curvature of bones become more frequent. Against the background of changes in the vertebrae, severe pain may appear due to compression of the spinal roots. Bone changes in osteoporosis are irreversible, that is, they do not disappear after the treatment of Itsenko-Cushing's disease and the normalization of cortisol levels. Therefore, patients with this complication often lose their ability to work and become disabled.
- Heart failure. It is the most dangerous of the complications of Itsenko-Cushing's disease. Hormonal failure affects heart cells, causing cardiomyopathy and rhythm disturbances. Because of this, the blood is pumped worse through the vessels, and heart failure occurs. It is she who usually causes death in patients who have not received the necessary treatment. If the disease was cured, the changes in the myocardium still do not disappear, and the patient suffers from chronic heart failure until the end of his life. He is not able to perform heavy physical exertion, he gets tired faster from everyday work.
- Weakened immunity. The increased release of corticosteroids depresses the immune system over time. Because of this, the body becomes more susceptible to various infections. Patients often suffer from pustular skin diseases, respiratory infections. The immune system is not able to quickly produce enough antibodies to defeat microbes. Even the treatment of such banal infections with antibiotics lasts longer than in healthy people. After curing and normalizing the level of cortisol in the blood, immunity is gradually restored.
- stomach ulcer. Metabolic disorders also affect the gastric mucosa. It becomes more susceptible to the aggressive effects of hard, spicy and acidic foods, as its defense mechanisms are weakened. First of all, the production of mucus, which normally envelops the walls of the stomach, decreases. The rate of regeneration of mucosal cells also slows down. As a result, patients with hypercortisolism may develop gastric ulcers or ( less often) of the duodenum. An additional factor, accelerating this process, is malnutrition and non-compliance with the diet prescribed by the doctor. After the normalization of hormone levels, the patient will have to treat an ulcer for a long time, which does not disappear immediately. However, the prognosis for this complication will already be favorable, because the root cause that caused disturbances at the level of the gastric mucosa has been eliminated.
- Obesity. Obesity is one of the most common symptoms of hypercortisolism ( including in Itsenko-Cushing's disease). In some cases, it can be regarded as a specific complication of this disease. The fact is that excess body weight increases the risk of myocardial infarction and a number of other diseases. In addition, after fixing the problem ( pituitary neoplasms) excess weight comes off gradually, and the patient does not always return to his former forms.
- Urolithiasis of the kidneys. The formation of kidney stones, like osteoporosis, is due to the leaching of calcium compounds from the bones. Calcium enters the bloodstream, is not absorbed by bone tissue and is filtered in large quantities in the kidneys. In the renal pelvis, where urine accumulates before entering bladder, stones begin to form from calcium compounds. If the period of hypercortisolism lasts several years, then very large formations form in the kidneys. They injure the delicate epithelium of the renal pelvis, disrupt the outflow of urine into the ureters, cause severe pain ( renal colic) and predispose to the development of infections ( pyelonephritis). Usually, the symptoms of urolithiasis appear on one side, but a careful examination always reveals damage to both kidneys.
Cushing's syndrome (hypercorticism) rarely diagnosed in men and children. This disease is more likely to affect women in the age group from 25 to 40 years.
Hormonal imbalance caused by various reasons leads to pathological changes in metabolism, which is reflected in the appearance.
The main cause of Cushing's disease is overproduction of the hormone cortisol - a product of the adrenal cortex. And the violation of the work of this body can contribute to several factors at once, given below.
Exogenous hypercortisolism
Caused by an overdose or long-term use of steroid drugs (prescribed in the treatment of asthma, rheumatoid arthritis and in the postoperative period after organ transplantation).
Endogenous hypercortisolism
Caused by internal discord in the body. Pituitary dysfunction (increased production of adrenocorticotropic hormone) provokes the release of cortisol by the adrenal cortex. The causes of the disease can be hyperplasia of the adrenal cortex and malignant tumors-corticotropinoma. Places of possible localization - bronchi, ovaries, testicles.
Pseudo Cushing's Syndrome
Symptoms similar to hypercorticism can be obesity, chronic alcohol intoxication, pregnancy, stress, depression, and sometimes taking oral contraceptives.
"To reduce the risk of death and achieve the fastest results in the treatment process, it is advisable to seek help in the first 5 years from the onset of the disease. "
Symptoms of Cushing's disease
1. Rapid and characteristic weight gain. Areas of fat accumulations - face (round and ruddy), abdomen, cervicothoracic region. The arms and legs look disproportionately thin.
2. Muscular atrophy of the shoulder girdle and legs, accompanied by increased weakness and fatigue.
3. Deterioration of the skin condition - hyperhidrosis, increased dryness, marble shade, thinned layer of the surface epithelium, loss of elasticity (appearance of stretch marks) and regenerative functions (slowly healing wounds).
4. Decreased libido.
5. Male-type hair in women, failure and absence of menstruation.
6. Development of osteoporosis. In the initial stage, it is distinguished by joint pain. In the future, it can manifest itself as spontaneous fractures of the limbs and ribs.
7. Due to the negative hormonal effects on the myocardium, there are problems with the work of the cardiovascular system. - cardiomyopathy, angina pectoris, hypertension, heart failure.
8. Often, hypercortisolism goes hand in hand with steroid diabetes.
9. The nervous system reacts to hormonal imbalance with lethargy, depression, euphoria, steroid psychosis.
Cushing's syndrome: treatment
The disease is diagnosed by the results of biochemical blood and urine tests. Further, differential diagnosis is carried out to identify the causes - MRI of the pituitary gland, abdominal cavity, layer-by-layer radiography, biochemical study of hormones.
When establishing the causes that caused Cushing's disease, an appropriate treatment method is selected, aimed at eliminating the cause and restoring hormonal balance.
Medical option - the appointment of drugs that reduce the production of cortisol.
Radiation therapy - used to influence the pituitary adenoma.
Surgery - is performed to excise neoplasms of the pituitary gland and adrenal cortex, in severe cases, the adrenal glands are removed and hormone replacement therapy is prescribed for life. Efficiency - 70-80%, rapid improvement in the patient's condition.
Often, in the treatment of this disease, complex measures are taken that combine all available methods of treatment.
It is known that the hormones produced by the adrenal cortex perform very important functions in the body. With insufficient or excessive secretion of cortical hormones, various diseases develop. Itsenko-Cushing's syndrome is a complex of symptoms arising from excessive synthesis of cortical hormones in the adrenal glands. There are several types of this disease, and they all have similar symptoms. The reasons for this state of affairs are many. It can be a tumor, a violation of hormonal regulation and other disruptions in the body. Subclinical hypercortisolism can be completely asymptomatic.
Itsenko-Cushing syndrome, or hypercortisolism syndrome, occurs due to the fact that the hypothalamus secretes a lot of corticoliberins - substances that stimulate the pituitary gland to increase the production of adrenocorticotropic hormone (ACTH), and it, in turn, causes excessive synthesis of hormones of the adrenal cortex.
What types of adrenal hyperfunction are there?
In the photo - the examination process during kidney diseaseThere are primary and secondary hypercortisolism:
- The causes of primary hyperfunction are mainly tumors of the adrenal glands - corticosteromas. They are mainly hormonally active, that is, they produce hormones. A lot of glucocorticoids and a certain amount of androgens and mineralocorticoids are released into the blood. Also, the cause of this type of disorder can be tumors located in other organs that synthesize ACTH-like compounds.
- Secondary hypercortisolism occurs due to disturbances in the work of the hypothalamic-pituitary-adrenal system. Short description the mechanism for the development of this state is found above. Another cause of secondary hypercortisolism may be pituitary adenoma, which stimulates increased production of ACTH, which in turn leads to a large release of cortical hormones into the blood.
According to the forms of the course, the syndrome of hyperfunction of the adrenal glands is divided into 3 types:
- The subclinical form occurs in the presence of so-called inactive tumors of the adrenal gland. In 10 patients out of 100, a corticosteroma is determined, which secretes cortisol in small amounts, which does not cause clinical manifestations. But according to some signs, it can be concluded that there is a syndrome of hypercortisolism. The patient may experience an increase in blood pressure, disorders in the reproductive system, diabetes mellitus.
- Iatrogenic hypercortisolism is also called medicinal or exogenous. It occurs in patients who have been treated with synthetic corticosteroids for a long time. What is it and in what cases is it applied? Long-term corticosteroids are indicated for inflammatory diseases such as rheumatism, various kidney pathologies, blood diseases, and chronic connective tissue diseases. Also, this group of drugs is prescribed to people who have undergone organ transplantation. Exogenous hypercortisolism occurs in 70% of cases.
- Functional hypercortisolism occurs in people with obesity, diabetes, liver disease, hypothalamic syndrome, and depression. Also, this condition is diagnosed with pubertal and youthful dispituitarism, pregnancy and alcoholism.
Clinical manifestations of the disease
The main symptoms of hypercortisolism:
- Constant fatigue
- Sleep disturbance up to insomnia
- progressive weakness
- Decreased appetite
- Painful sensations in the abdomen
- Change in body weight
- Nausea and vomiting
- Constipation followed by diarrhea
- Increasing muscle weakness
- Decreased muscle tone of the skeleton and internal organs
- Hyperpigmentation of the skin and mucous membranes - melanosis.
- depression
- Dry skin and its increased peeling
- Increasing the amount of hair on the face and body
- Rachiocampsis
- Spontaneous bone fractures
- Osteoporotic changes in the bones
- Fluid retention in the body
- Edema
- Cardiovascular disorders
- neurological disorders.
What therapeutic measures are assigned to patients?
An integrated approach is used to treat hypercortisolism. The main goal of treatment is to stop the clinical manifestations of the syndrome and restore the physiological rhythm of hormone synthesis. Prescribe medications that can suppress the excess synthesis of cortisol and corticotropin. In the case of adrenal hyperplasia or pituitary adenoma, surgery or radiation therapy is indicated.
Drug treatment with inhibitors of the production of corticotropin and corticoliberin is prescribed when central hypercorticism is detected. Usually, patients are prescribed Peritol, a serotonin inhibitor, for a course of 4 weeks. It reduces the production of corticoliberin in the hypothalamus. Also dopamine agonists such as Abergin and Bromergon (Parlodel) for 6 to 10 months.
They reduce the release of corticotropin. In addition, inhibitors of the formation of corticosteroids in the cortical substance of the adrenal glands are prescribed. These are drugs Mamomit and Metapiron. Do not think that such a violation as hyperfunction of the adrenal glands is treated quickly. It takes time for all processes to return to normal. Usually treatment is delayed for 6 months or more.
Symptomatic therapy is aimed at correcting the metabolism of carbohydrates, electrolytes and proteins. Prescribe drugs that lower blood pressure and normalize the work of the heart and blood vessels. Osteoporosis is a frequent complication of adrenal hyperfunction, so prevention of bone fractures is important in the treatment of this syndrome. To normalize protein metabolism, doctors prescribe anabolic steroids, for example, Nerobolil and Retabolil. I regulate carbohydrate metabolism by correcting nutrition if there is a violation of carbohydrate tolerance, as well as by prescribing insulin or sulfonylurea derivatives in case of diabetes mellitus.
Since the syndrome of hyperfunction of the adrenal cortex is accompanied by hypoxia, patients should not take biguanides, and correction of electrolyte metabolism is carried out with potassium preparations. With high blood pressure, antihypertensive therapy is prescribed, and in case of violations of the cardiovascular system, cardiac glycosides and diuretics are prescribed. To prevent osteoporosis, it is recommended to take drugs that accelerate the absorption of calcium in the intestines: vitamin D derivatives. To fix calcium in the bones, Calcitonin and Calcitrin are prescribed. To prevent fractures and other unpleasant complications of osteoporosis, therapy should be carried out for at least 1 year.
A prolonged increase in the blood level of adrenal hormones, glucocorticoids, leads to the development of hypercorticism. This syndrome is characterized by a violation of all types of metabolism, failures in the work of various body systems. The causes of hormonal imbalance are volumetric formations of the pituitary gland, adrenal glands and other organs, as well as the intake medicines containing glucocorticoids. To eliminate the manifestations of the disease, medications are prescribed, and the tumor is affected by radiation methods or removed surgically.
Adrenal hormones and their role
The adrenal cortex produces steroid hormones - glucocorticoids, mineralocorticoids and androgens. Their production is controlled by the pituitary adrenocorticotropic hormone (ACTH, or corticotropin). Its secretion is regulated by the releasing hormone corticoliberin and hypothalamic vasopressin. Cholesterol is a common source for the synthesis of steroids.
The most active glucocorticoid is cortisol. An increase in its concentration leads to the suppression of corticotropin production according to the feedback principle. Thus, hormonal balance is maintained. The production of the main representative of the group of mineralocorticoids, aldosterone, depends on ACTH to a lesser extent. The main regulatory mechanism for its production is the renin-angiotensin system, which responds to changes in circulating blood volume. Androgens are produced in greater quantities in the gonads.
Glucocorticoids affect all types of metabolism. They contribute to an increase in blood glucose, protein breakdown and the redistribution of adipose tissue. Hormones have pronounced anti-inflammatory and anti-stress effects, retain sodium in the body and increase blood pressure.
Symptoms of hypercortisolism
Symptoms of hypercortisolism
A pathological increase in the level of glucocorticoids in the blood leads to the occurrence of hypercorticism syndrome. Patients note the appearance of complaints associated with a change in their appearance and disruption of the cardiovascular, reproductive, musculoskeletal and nervous systems. Symptoms of the disease are also caused by high concentrations of aldosterone and adrenal androgens.
In children, as a result of hypercortisolism, collagen synthesis is disrupted and the sensitivity of target organs to the action of somatotropic hormone decreases. All these changes lead to growth retardation. Unlike adults, it is possible to restore the structure of bone tissue after the pathology has been cured.
Manifestations of hypercortisolism are shown in the table.
| Symptoms associated with excess glucocorticoids | Symptoms Associated with Excess Mineralocorticoids | Symptoms associated with excess sex hormones |
| Obesity with the deposition of fat masses in the body and face, atrophy of the muscles of the limbs | Increase in blood pressure resistant to therapy | Acne, seborrhea, acne |
| Thinning of the skin, the appearance of purple stretch marks | muscle weakness | Menstrual disorders in women |
| Carbohydrate metabolism disorders - prediabetes and diabetes mellitus | Dishormonal myocardial dystrophy with the development of arrhythmias and heart failure | Excess hair growth on the face, chest, abdomen, buttocks in women |
| Development of osteoporosis | Peripheral edema | Infertility |
| Secondary immunodeficiency with the occurrence of various infections and fungal infections | Profuse and frequent urination | Decreased libido |
| Inflammatory processes in the kidneys, urolithiasis | Numbness, tingling and leg cramps | With an excess of estrogens in men - an increase in the timbre of the voice, a decrease in facial hair growth, an increase in the mammary glands |
| Mental disorders - emotional instability, depression, behavior change | Headache | erectile disfunction |
Diagnosis of pathology
Regulation of the synthesis and secretion of glucocorticoids - direct (+) and reverse (-) connections
A syndrome associated with an increase in the level of glucocorticoids can be suspected by the characteristic appearance the patient and the complaints he makes.
Since the main manifestations of hypercortisolism are associated with an excess of cortisol, its level in daily urine or saliva is examined to make a diagnosis. At the same time determine the concentration of corticotropin in the blood. To clarify the form of pathology, functional tests are carried out - small and large dexamethasone tests.
Types of hypercortisolism and their treatment
Excessive synthesis of glucocorticoids occurs under the influence of corticotropin and corticoliberin or independently of them. In this regard, ACTH-dependent and ACTH-independent forms of pathology are distinguished. The first group includes:
- Central hypercortisolism.
- ACTH-ectopic syndrome.
Types of hypercortisolism independent of corticotropin are:
- Peripheral.
- Exogenous.
- Functional.
The central form is Itsenko-Cushing's disease
The cause of the pathology is a pituitary tumor. Microadenomas are characterized by sizes up to 1 centimeter. With formations of a larger size, they speak of macroadenomas. They produce an excess amount of ACTH, which stimulates the production of glucocorticoids. violated Feedback between hormones. There is an increase in the level of corticotropin and cortisol, proliferation of adrenal tissue - hyperplasia.
For treatment, irradiation of the pituitary gland with a proton beam is used. Sometimes combined methods are used - telegammatherapy and removal of one adrenal gland. Large tumors are operated on using an intranasal transsphenoidal or open approach. Additionally, unilateral or bilateral removal of the altered adrenal glands is done.
Of the drugs in the period of preparation for surgery, inhibitors of steroidogenesis are prescribed, which block the formation of adrenal hormones. These include ketoconazole (Nizoral), aminoglutethimide (Mamomit, Orimeten). And also use drugs that reduce blood pressure, normalize blood glucose levels.
ACTH-ectopic secretion
In this case, malignant tumors of various organs produce an excessive amount of substances similar in structure to corticotropin or corticoliberin. They interact with ACTH receptors in the adrenal glands and enhance the synthesis and secretion of their hormones.
Hormonally active volumetric formations are more common in the bronchi, thymus, uterus, pancreas, and ovaries. They are characteristic fast development symptoms of hypercortisolism. After establishing the localization of the tumor, it is removed. If there are contraindications to surgery, inhibitors of steroidogenesis and drugs that correct concomitant disorders are used.
Peripheral hypercortisolism
The formation of the adrenal cortex, which produce an excess amount of hormones, leads to the development of pathology. By their nature, they can be malignant or benign. Corticosteromas, adenocarcinomas are more common, tissue hyperplasia is less common. In laboratory tests, an increase in the concentration of cortisol and a decrease in the level of corticotropin are determined. Tumors are removed surgically. Preoperative preparation is similar to that which is carried out with Itsenko-Cushing's disease.
Exogenous form
In this case, hormonal imbalance is a consequence of taking drugs containing glucocorticoids, which are prescribed for various diseases. Their excessive intake leads to the suppression of the synthesis of ACTH and the production of adrenal hormones. People who take these drugs in high doses for a long time develop all the symptoms characteristic of hypercortisolism. Diagnosis can be made by examination and by establishing characteristic changes in metabolism.
To prevent exogenous hypercorticism, they try to prescribe the lowest possible doses of glucocorticoids. With the development of concomitant conditions of the syndrome - diabetes mellitus, arterial hypertension, osteoporosis, infectious diseases, they are treated.
Functional hypercortisolism
This type of pathology occurs in some patients with diabetes mellitus, liver disease, hypothalamic syndrome, and chronic alcoholism. There is a violation of daily fluctuations in the concentration of corticotropin and cortisol. Perhaps the development of typical signs of hypercortisolism without changes in the structure of the adrenal cortex and pituitary gland. Treatment involves the correction of lifestyle, therapy of the underlying disease and metabolic disorders, the rejection of bad habits.
