Disease of Itenko-Cushing: symptoms, diagnosis and treatment
Ithenko-Cushing's disease is a pathological condition diagnosed in people of all ages.Its name was obtained by two researchers who studied it.For the first time Nikolai Itenko began talking about her in 1924, a neuropathologist from Odessa, who explained the development of such a state by malfunctions in the work of the hypothalamus.After 8 years, Harvey Cushing, a neurosurgeon from Canada, continued his research.In the course of his work, he suggested that the cause of the onset of symptoms of the disease becomes a tumor.At the moment it is known that both assumptions were true.Table of Contents: General Information Pathogenesis of Itenko-Cushing's Disease Symptoms of Itenko-Cushing's Disease
The disease is a neuroendocrine disorder in which the balance of the hypothalamic-pituitary-adrenal system and the disruption of the daily rhythm of corticotropin-the hormone( CRG) -adrenocorticotropic hormone( ACTH) hormone and hormones produced by the adrenal cortex, including cortisol .
Cortisol is a stress hormone, its excess accelerates the breakdown of proteins and fats, impairs the intake of calcium from the intestine, reduces immunity, accelerates the production of gastric juice, increases blood pressure and slows the production of the CRT in the hypothalamus( it is also called corticoliberin).CRT in turn reduces the formation of ACTH in the pituitary gland, while ACTH inhibits the function of the adrenal cortex, thereby reducing the production of cortisol.This is how the healthy hypothalamic-pituitary-adrenal system works.
With the illness of Itenko-Cushing, the mechanism established by nature fails.Modified pituitary cells begin accelerated secretion of ACTH, which leads to an increase in the synthesis of cortisol, but does not trigger the mechanism of "reaction to stress" - does not reduce the production of MCT.Those.There is a persistent and prolonged increase in the level of cortisol and other hormones of the adrenal glands in the blood.
In view of hypersecretion of corticosteroids, Itzenko-Cushing's disease is also called hypercorticism.
Please note! In medicine, they also operate with such a notion as the Itenko-Cushing syndrome.It is more extensive and covers all pathological conditions, united by a common symptomatology - increased production of adrenal cortisol.That is, in addition to the disease, the syndrome includes other conditions - corticoliberin-ectopic syndrome, adrenal cortex tumors, iatrogenic syndrome.
People at the age of 25-40 are at risk, although the disease can be diagnosed in adolescents during puberty and in children. According to statistics, women are exposed to it 4 to 8 times more often than men.There is a serious illness: with the failure of mineral and carbohydrate metabolism, the appearance of problems in the work of the nervous, cardiovascular, digestive and urinary systems.
Pathogenesis of Itenko-Cushing's disease
Scientists still have difficulty in identifying the exact causes of the Itenko-Cushing's disease.
At the same time, they note that the following factors provoke the pathology:
- Rapid restructuring of the body, which fixes failures in the pituitary and hypothalamus.Most often it is observed during pregnancy, childbirth, at the onset of menopause, during puberty.
- Serious craniocerebral trauma and severe bruises.
- The onset and development of cerebral infection( encephalitis, meningitis), resulting in a general intoxication of the body.
- Adenoma, or a tumor that emerged from the chromophobic and basophilic cells of the pituitary gland( the cause of the development of the disease in 85% of cases).
All these factors are capable of triggering a mechanism of development of severe symptomatology of pathology.
Symptoms of of Itenko-Cushing's disease
There are many signs that a doctor can diagnose a disease, as it affects many organs and systems.Skin, subcutaneous fat, muscular, nervous, endocrine, digestive and other systems suffer.
Skin signs of Itenko-Cushing's disease
Skin covers dry out, peel off, acquire a purple-marble shade.In areas where skin friction occurs, hyperpigmentation of appears.On the buttocks, abdomen, on the hips, on the chest, and under the arms appear brightly-expressed striae( violet, bright red stripes).
Gradually the skin becomes thinner, blood vessels appear on it.In some places, bruises are noticeable, which heal for a long time because of the fragility of the vessels.In patients, virilism and hirsutism are noted.The first phenomenon is characterized by increased growth of hair on the face and body, while on the head they quickly fall out.The second is the appearance of vegetation in women on the chest, abdomen, above the upper lip.There are also ulcers, ulcers, boils.
Changes in subcutaneous adipose tissue
In 95% of cases, obesity is noted in patients, and the accumulation of body fat is not uniform .The face is rounded, takes a lunate shape, in the zone of the 7th cervical vertebra, there is an adipose mound, there are folds in the abdomen and chest.
Bone symptoms of Itenko-Cushing's disease
The disease affects the bones of .This occurs in 95% of cases, because the hormones produced on one side negatively affect the process of calcium assimilation, and on the other - destroy the proteins that make up the basis of the bone.As a result, osteoporosis develops, affecting the shape and size of the skull, spine, hands and feet.Patients report severe pain syndrome, they have an increased risk of fractures, and growth in children slows down.
Symptoms of pathology from the respiratory system
Because of the increased production of hormones, the immunity of decreases.The organism is more often exposed to bronchitis, pneumonia.Possible development of tuberculosis.
Due to the destruction of muscle proteins, the limbs are thinned, the person grows thin and feels constant weakness, in some cases can not lift the weights or stand up on his own.
Signs of the pathology of the digestive system
Heartburn, gastric and intestinal ulcer are marked signs of Itenko-Cushing's disease.Under the influence of hormones, liver function also worsens.
Cardiovascular system disorders
In patients, first of all, blood pressure rises, and if he ignores it, heart failure develops. At the same time, the blood flow through the vessels slows down, and the organs and systems begin to lack oxygen.In the absence of qualified assistance, a fatal outcome may result.
Symptoms of nervous system disorders
The bone fragility provokes pressure on the nerves that pass in close proximity to them.As a consequence, a person has severe pain localized in the extremities and prevents him from fully walking, lifting loads of .At the same time, deterioration of memory, intellect is diagnosed, depression appears, increased irritability or, conversely, apathy, indifference to everything.In the absence of skilled care, the patient is born with thoughts of suicide.
Changes in the urinary system
Reduced immunity and increased susceptibility to pathogens lead to the development of pyelonephritis .Elimination of calcium from bones and malfunctions during its assimilation contribute to the development of urolithiasis.Hypertension provokes the development of nephrosclerosis, which, in turn, only strengthens it, and so on in a circle.
Symptoms of the Itenko-Cushing's disease from the side of the sexual and endocrine system
In a third of patients, as a result of all changes, diabetes mellitus is observed, disruptions in the metabolism of proteins, fats, carbohydrates are diagnosed, and the water-salt balance of is disturbed.All patients note a decrease in sexual desire.Women stop menstruating, and the sex organs decrease in size.It is difficult for them to conceive a child, and if conception does occur, endure it.
Diagnostics and types
When diagnosing a disease, an anamnesis is collected.Based on the findings, the physician classifies its form.
Illness of Itenko-Cushing happens:
- of mild severity, when the symptomatology is not pronounced, there are no problems with the bone system;
- of moderate degree, when all the signs are there, but no complications are observed;
- severe - in this case there are fractures, diabetes, mental disorders, nephrosclerosis.
Important! With the flow, the disease is progressive and torpid.In the first case, the development of symptoms proceeds rapidly, and the patients quickly become disabled.In the second - the symptomatology is manifested slowly( can be stretched for several years).
To confirm the diagnosis, blood and urine are taken for analysis to identify hormones and the results of their disintegration in them.
The following test methods allow us to verify the correctness of the diagnosis:
- Radiography of the bone system, in which the skull, extremities, spine are studied.
- MRI and computed tomography of the brain, which can detect the presence of pituitary neoplasm and assess the degree of destruction of the osseous system.
- Ultrasound, MRI, computed tomography, angiography of the adrenal glands, which allow to assess their size and degree of functioning.
Please note! In order to dispel all doubts about the correctness of the diagnosis, doctors conduct tests with dexamethasone and methopyron.
An experienced endocrinologist should advise and prescribe the treatment.
Treatment of Isenko-Cushing's disease
There are two ways to combat the disease.The first involves the elimination of the causes of its appearance and the normalization of the hypothalamus, pituitary and adrenal glands.The second goal is to eliminate the malfunctioning of all affected systems and organs.
Depending on the severity of the Itenko-Cushing disease, physicians prescribe:
- Radiation therapy - proton or gamma at moderate to severe severity;
- Medication, when dopamine receptor agonists are prescribed, antitumor drugs and preparations that slow down the synthesis of steroids.
- In severe cases, the use of surgical treatment is justified, when one-sided or bilateral adrenal removal is prescribed, the introduction of an agent destroying their structure, removal of the pituitary neoplasm.
For the normalization of the work of organs and systems, symptomatic treatment is provided.In this case:
- in diabetes mellitus appoint a strict diet with mandatory intake of drugs to reduce blood sugar;
- with arterial hypertension prescribed diuretics and drugs to reduce blood pressure;
- for detection of heart failure - digitalis preparations and cardiac glycosides;
- for osteoporosis - vitamin complexes with vitamin D, preparations for restoring the protein structure of the bone and improving the processes of calcium assimilation;
- for strengthening immunity in Isenko-Cushing's disease, the doctor prescribes funds that stimulate the growth and maturation of lymphocytes.
Please note! The effectiveness of the diet in the treatment of Itenko-Cushing's disease is weak, so it is often underestimated.Meanwhile, it is the new eating habits that allow patients to improve their overall health and prevent the development of consequences.For the prevention of osteoporosis and urolithiasis, doctors advise them to eat eggs, dairy products, and when obesity - to give up salt and fatty foods, as well as increase the amount of drunk water per day.
In addition, doctors always advise to exclude fatty, smoked, salty, sour, to abandon bad habits - drinking alcoholic beverages, smoking.
Because of serious hormonal failures and problems in the work of individual organs and systems, doctors rarely give accurate predictions.Ignoring the symptoms of the disease and lack of qualified treatment can result in irreversible effects in the body or fatal.At the same time, timely access to a specialist allows to stop the development of an ailment and to keep working capacity.
Important! 50% of patients with this disease die in the absence of treatment for 5 years.
In adults, the predictions depend on the degree of development of the Itenko-Cushing's disease and the presence of severe symptoms.In children, the situation is worse because of accelerated growth and increased metabolism. At best, the development of the child is inhibited, and at worst - the curvature of the spine, which entails cosmetic defects.
The person can increase the chances of a favorable outcome of treatment if all the prescriptions of the doctor are followed, as well as undergoing a regular examination for the detection of complications.
Please note! The sooner a diagnosis is made, the more likely that the disease will be cured.
The most terrible complications in the Itenko-Cushing disease are symptoms that have become independent diseases.We are talking about:
- diabetes mellitus, which affects every fifth patient;
- osteoporosis, from which most of them suffer;
- heart failure, which causes irreversible processes in the myocardium;
- weakened immunity, as a result of which a person often suffers from respiratory, skin diseases;
- stomach ulcer;
The fact that the Itzenko-Cushing's disease itself is extremely rare is exacerbating the situation, so is not always immediately diagnosed.Thus, while doctors treat her symptoms, recognizing them for independent ailments, she is progressing.To prevent this from happening, if any similar symptoms occur, you need to seek advice from the endocrinologist.
Chumachenko Olga, medical reviewer