Adrenal insufficiency treatment with drugs. Hypocortisolism or adrenal insufficiency in women: symptoms and treatment of chronic hormone deficiency of paired endocrine organs

Adrenal insufficiency (AI, adrenal insufficiency, hypocortisolism)– an endocrine disease that is caused by weak production of hormones from the adrenal cortex due to damage or disruption of the hypothalamus and pituitary regulation.

The adrenal glands are paired glands that regulate metabolism, affecting most organs and their processes, thereby allowing them to cope with stress. The functional properties are almost the same for both women and men.

The disease is diagnosed quickly and simply and has virtually no hidden signs. It can occur in acute and chronic form.

ICD code

According to the international classification of diseases, it has an ICD code of 10. The list of these diseases includes the following categories of the endocrine system:

E 27.1 Primary adrenal insufficiency;
E 27.3 Drug failure;
E 27.4 Other species or unspecified categories.

Causes

The disease occurs as a result of:

idiopathic (autoimmune) atrophy of the adrenal cortex;
tuberculosis;
syphilis;
Itsenko-Cushing's disease;
human immunodeficiency virus (HIV);
scleroderma;
irradiation;
brain injuries.

Chronic adrenal insufficiency in children can be hereditary.

Stages

There are 3 main stages of the onset of this disease:

Addison's disease (primary type), the development factors of which are a decrease in the functioning of the immune system, tuberculosis, human immunodeficiency virus, venereal disease and fungal viruses. Due to weakened immunity, the layer of the adrenal cortex is rejected;
Secondary adrenal insufficiency develops with brain tumors, after surgery or head injuries, and heavy bleeding. Long-term therapy with glucocorticoids can also cause;
Tertiary view manifests itself in connection with radiation, operations, intoxication and anorexia nervosa.

The adrenal glands are one of the organs that are most supplied with blood in the body, as well as the thyroid gland. For this reason, when the tasks performed by the adrenal cortex are disrupted, metastasis occurs in the presence of oncology in the lungs.

Symptoms of adrenal insufficiency

Photos of symptoms of the disease
Acute adrenal insufficiency

Any type of disease has early manifestations; with NN, the following symptoms can be distinguished:

fatigue and drowsiness;
high level of sensitivity to sunlight;
reducing the body's resistance to infections;
loss of appetite;

Laboratory studies of the disease revealed a number of associated symptoms, such as:

change in skin color at the bends of the limbs, etc.;
modification of the color of the mucous membrane from golden to gray;
a sharp drop in blood pressure (BP);
increased heart rate and shortness of breath due to the development of tachycardia;
digestive tract disorder;
spasms in the gastrointestinal tract;
weight loss;
rapid muscle fatigue and difficulty moving.

The manifestation of the chronic stage in adolescents occurs with the following symptoms: brown pigmentation of scars, skin folds and gums. Also fatigue vomiting for no reason and increased salt intake, low blood sugar. With the development of the acute form of the disease, children experience diarrhea, vomiting and pain in the abdominal area. Also, when fluid is lost in large volumes, the skin acquires a bluish color and cold body temperature. You may experience fainting, drowsiness, and seizures.

Manifestation of the disease at various stages

Primary adrenal insufficiency reduces or increases hormonal levels, and inflammation of the endocrine gland and type 1 also appears. The disease develops in 85-90 percent of cases, due to the destruction of adrenal tissue.

In the second and third forms of adrenal insufficiency, symptoms appear to a much lesser extent.

Complications with adrenal insufficiency

A complication, also called an adrenal crisis, can occur when the functions of the adrenal cortex are disrupted; problems in the functioning of one of the organs or the whole organism may arise. Possible development into a comatose state.

The complication is characterized by:

severe decrease in blood pressure, possible fainting;
severe weakness, loss of space;
frequent vomiting and unstable stools;
dehydration of the body;
heart failure;
cramping and severe pigmentation.

Also, the acute form is often closely related to chronic manifestations. Exacerbation may occur due to the following factors:

previous viral infection during illness;
stopping taking medications prescribed for treatment;
suffered stress and other negative emotions;
sudden change in climate zone;
trauma or surgery in the abdominal cavity.

When sick, women often experience disruptions to their menstrual cycle, and men's potency.

Diagnosis of the disease.

Diagnosis first begins with asking the patient or adult relatives about the general condition, what are the manifestations and symptoms involved in the process of deterioration. They find out the reasons why this problem could arise and take into account the patient’s complaints.

Diagnostic methods.

The initial step in identifying pathology is to prescribe an ultrasound examination (ultrasound). Such an examination should show the following origins of the disease:

the presence of tuberculosis infection and excess of calcium salts indicates that the cause of insufficiency is tuberculosis;
the detection of an autoantibody to the 21-hydroxylase antigen is the reason for the diagnosis of an autoimmune manifestation of hypocortisolism.

The next stages of examination are diagnostics for magnetic resonance imaging (MRI) and computed tomography (CT). In the secondary stage, the same procedures are performed, including MRI of the brain.

If the doctor doubts the diagnostic results, he may conduct a stimulation test, in the form of hormones produced by the pituitary gland and hypothalamus. This will allow you to find out the content of a biologically active hormone in the blood; it is also called the “stress hormone” (cortisol).

A general blood test is required for donation. Which will show sodium ion concentration, high potassium content, increased lymphocyte count, decreased or increased eosinophil count, decreased white blood cell count.

Treatment

Modern medicine can provide treatments for this disease, and this depends on the cause and factors that projected the disease. Treatment methods are primarily aimed at eliminating adrenal insufficiency and restoring the hormone.

Treatment is carried out by taking medications for tuberculosis, fungal diseases, syphilis, getting rid of tumors, due to radiation therapy. If the treatment was carried out correctly, but did not have a sufficient effect, or contributed to other processes in the course of the disease, it is possible to support the patient with hormonal therapy for life.

Drug treatment

Prescribing and taking drugs of the glucocorticoid and mineralocorticoid groups begins. Most often, doctors prescribe "Cortisone" and "Cortef", for mild pathology. If the form is more severe, prednisolone is prescribed, in compounds in the form "Cortisone acetate" or "Cortef with mineralocorticoids".

Often, the onset and progression of a disease cannot occur without stress. If the patient has such manifestations, the dose of corticosteroids is increased 3-5 times. During pregnancy, higher doses are allowed only in the 2nd trimester.

Anabolic steroids are used to correct muscle mass, tone it and renew cell parts. The prescription occurs to any group of people, regardless of gender, often these are patients with a chronic course of the disease. Take 3 times a year at short intervals.

It is possible to use replacement therapy. It also provides for a gradual reduction in the use of drugs, i.e. used for subsequent cancellation. The main thing in replacement therapy is compensation of the clinical hormonal type, as well as its maintenance and preservation. In this case, the indicators will be considered:

baseline plasma cortisol level is more than 350 mmol/l;
Potassium within 4.0-4.5 mmol/l;
Sodium from 135 to 140 mmol/l;
Glucose 4.5-9.0 mmol/l per day.

If the cause of the disease is infection, antibiotic therapy is administered.

Determining improvement with medication

Improvement in condition can be determined by factors such as:

normalization of blood pressure;
decline in pigmentation;
weight gain within normal limits;
improvement of general condition;
eradication of problems with the gastrointestinal tract;
exclusion of anorexia;
restoration of muscle function and more.

Often the drugs offered by specialists are classified according to duration of action and quantity, as follows:

Short acting:
- Hydrocortisone – 20 mg;
- Cortisone – 25 mg;
- Prednisone – 5 mg;
- Prednisolone – 5 mg;
- Methylprednisolone – 4 mg.
Average duration:
- Triamcinolone – 4 mg;
- Paramethasone – 2 mg;
Long acting:
- Dexamethasone – 0.75 mg;
- Betamethasone – 0.6 mg.

For mild insufficiency, only Cortisone, if the degree of damage is higher, they also add Prednisolone, Fludrocortisone.

Fludrocortisone is an analogue of the mineralocorticoid aldosterone. It is always used regardless of the degree of deficiency. For example, if a child takes only glucocorticosteroids, weight gain will be very small. There will also be other problems: delayed psychomotor development, dehydration. The latter is most dangerous because the body can be filled with a lethal dose of potassium. Whether adult or child, treatment with corticosteroids alone may worsen the condition but also increase the chance of developing salt-wasting crises.

You can understand that the dose of Fludcortisone has been prescribed correctly by the following signs:

drop in sodium levels;
increase in potassium substances;
increase in renin activity.

An overdose of this drug will show its properties as follows: if during treatment swelling in the face and other areas appears, blood pressure rises, migraines and insomnia appear, and laboratory indicators show that there is a minimal amount of potassium in the blood and an increased level of sodium. Thus, you should reduce the amount consumed, but there is no need to cancel it at all.

Important! The dosage is increased only if the patient lives in hot climate zones, since sodium substances are excreted through sweat; during pregnancy, an increase is made from the second trimester.

If, in addition to the disease, an infectious disease with fever appears, if it is necessary to perform a traumatic operation, in order to avoid a subsequent Addisonian-type crisis, increase the dosage of the glucocorticoid drug by 3-5 times. Mineralocorticoids remain at the same dosage.

The effectiveness of treatment is checked every 30 days by testing the activity of hormones and electrolytes in the blood. Subsequent checks, when a person takes medications for a long time, take place every 2-3 months.

In case of diseases of the adrenal glands, it is possible to exceed the norm for taking glucocorticoids by 1-2 days in order to avoid the formation of an acute form of insufficiency.

Surgical operations

Surgical actions aimed at surgical intervention to restore the adrenal cortex are a complex process that can only be performed by highly qualified specialists. The recommended removal of the adrenal gland or its tissues or tumor is prescribed according to the doctor’s indications.

When removing a tumor, the quality factor (malignant and benign category) and the ability to produce hormones should be taken into account. Hormone levels are determined by taking blood and urine tests, and such types of examinations as:

blood test for chromogranin A, renin, aldosterone, cortisol, ACTH hormone, calcitonin, blood ions and parahormone (parathyroid);
urine test readings for metanephrines (adrenaline metabolite) and cortisol.

They are sent to the surgical table when the hormone production exceeds the established norm, or treatment of a malignant tumor is impossible, as prescribed by the doctor, based on the tests performed.

Alternative home treatments

You can start using folk remedies only after a diagnosis has been made. The use of herbs and mixtures is considered to be quite effective methods, but this should only be done with a doctor’s testimony, not on your own, as it is life-threatening.

The main goal of herbal treatment is to restore hormonal levels, both to suppress and to raise levels. The quantity, type and methods of use are prescribed by the attending physician. To treat disorders of the adrenal glands, it is recommended to take the following infusions:

Horsetail – promotes the production of hormones, cleanses the body of toxic substances, and promotes the functioning of water salt balance. Take with caution to people with diseases of the blood systems;
Geranium – containing radium molecules, promotes the production of hormones;
Currant leaves and vitamin C – have preventive properties, warn the immune system against various types of diseases;
The sequence – promotes the enrichment of minerals, restores the gastrointestinal tract, stimulates the adrenal glands.

There are herbal infusions that should be taken strictly according to the instructions and only under the supervision of a specialist; such folk remedies include licorice root. It has the properties of blocking hormone destruction.

Also, nettle ensures good metabolism and restores adrenal function, increases the number of red blood cells in the blood, reduces sugar, and mulberry leaves relieve inflammation in the kidneys.

Diet and prohibited foods to take when sick

If you have adrenal insufficiency syndrome, you should not eat foods high in sugar, as it can trigger the release of insulin, which is a big problem for the body.

Products that should be limited, or better yet completely excluded from the diet: chips, mayonnaise, sausages, coffee, instant noodles, alcoholic, carbonated and energy drinks and salt. It is also worth carefully monitoring the composition of products to ensure that they do not contain harmful additives and dyes.

Preference should be given to fruits and vegetables, especially with the onset of the sales season, as they contain many useful vitamins that the body needs.

To normalize the condition during the day, you should follow these instructions:

a good breakfast is the key to normal blood sugar levels; you can only eat before 8 o’clock;
an hour after breakfast you need a light snack, this also applies to the time before afternoon tea and lunch;
you should have lunch before 3 o’clock in the afternoon;
have dinner in small, light portions up to 6 o’clock; heavy and filling foods are consumed during the day;

Disease prevention

If a person has any suspicions, he should immediately go to the hospital, since timely diagnosis and treatment of this disease is in itself the basis for prevention.

Maintenance therapy should be intermittent. If the daily dose of the drug does not exceed 20 mg, it is best to take it in the morning. For large volumes, take as prescribed by the doctor or in the morning. To prevent withdrawal symptoms from affecting your body, it is best to gradually reduce your medication intake and only then stop taking it.

Also, people with such syndromes and problems of a similar nature need to register with an endocrinologist. If you are pregnant, limit access to tobacco places. Taking tobacco, alcohol, medications, and limiting these substances is in itself a prevention of adrenal insufficiency.

Forecast

Removing symptoms and effective rapid treatment is quite difficult and can result in death. In the case of the chronic stage, emergency care is not required, since this form very rarely leads to death; it is enough to contact the clinic in a timely manner for a detailed analysis and subsequent treatment.

For adrenal tumors, a favorable prognosis is possible based on the removal of a benign tumor. After 1-2 months, blood pressure recovers and metabolic processes return to normal.

In the presence of malignant tumors, it is difficult to give a good prognosis; it all depends on the timing of both the visit to the hospital and the treatment process.

It is unlikely that even one specialist will be able to give an accurate forecast, since it depends on many factors. Timely treatment and treatment as prescribed will lead to improvement of the condition. Ignoring adrenal insufficiency will naturally lead to death sooner or later.

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Adrenal insufficiency is a serious disease of the endocrine system, a primary disorder of the adrenal cortex (also known as Addison's disease) or a secondary disorder in which ACTH secretion sharply decreases, and the functional ability of the production of hormones of the adrenal cortex is impaired. Disruption of any of the links leads to disorders of the hypothalamic-pituitary-adrenal system. This term can refer to various types of symptoms and complications of hypocortisolism. A characteristic process for the disease is destructive processes in the adrenal glands.

A lack or decrease in the production of the mineralocorticoid hormone provokes a decrease in the amount of sodium and water, which causes harm and dehydration of the body, and the mass of circulating blood decreases. This disease is often found in middle-aged and older women and men, and is quite rarely observed in children.

This kind of disease can be provoked by diseases that lead to the destruction of the adrenal cortex: syphilis, AIDS, tuberculosis, lymphogranulomatosis, amyloidosis, various types of adrenal tumors.

From the history of the disease it is known that it was Addison who first described the course of the disease in 1855, which is associated with adrenal ailment of tuberculosis origin - this explains the second name of the disease - Addison's disease.

Classification of adrenal insufficiency

Modern medicine distinguishes 3 types of adrenal insufficiency: primary, secondary, tertiary.

Primary type

Primary chronic adrenal insufficiency, by its characteristics, initially affects the adrenal glands themselves. This type of disease is one of the most common types of adrenal insufficiency. According to statistics, 90% of all cases of this disease occur.

Secondary and tertiary forms

As for secondary and tertiary chronic adrenal insufficiency, they are characterized by an acute lack of secretion of ACTH or corticoliberin, which is secreted by the hypothalamic-pituitary system. All this can cause disruption or complete loss of the adrenal cortex’s working abilities.

In medical practice, depending on the speed of development of the symptoms of the disease, doctors also divide acute and chronic adrenal insufficiency.

Causes of adrenal insufficiency

Doctors name the following diseases and factors as the causes of primary long-term adrenal insufficiency:

HIV infection, syphilis, fungal infections, tuberculosis, metastases of various tumors;
autoimmune destruction of the adrenal cortex, which can lead to damage and failure of other glands of the endocrine system;
iatrogenic factors - anticoagulant therapy, which can provoke hemorrhage from both sides into the adrenal glands;
surgical intervention to remove the adrenal glands due to Itsenko-Cushing's disease;
use of blockers of steroidogenesis in the adrenal glands (chloditan, spironolactone, aminoglutethimide).

The fundamental cause of primary chronic adrenal insufficiency is primarily autoimmune adrenaline. Studies of this type of disease showed that antibodies to various components of the adrenal cortex were detected in the blood of patients. In adrenal insufficiency, these antibodies affect the main enzymes - steroidogenesis and 21-hydroxylase. Localized in the endoplasmic reticulum of cells of the adrenal cortex, this fragment provokes the reaction of converting 17-hydroxyprogesterone into 11-deoxycortisol in the zona fasciculata, which supplies the synthesis of cortisol and the reaction of transformation of progesterone into 11-deoxycorticosterone in the zona glomerulosa, which ensures the synthesis of aldosterone.

In 60-80% of patients with primary chronic adrenal insufficiency, antibodies to 21-hydroxylase are detected. The level of antibodies in the blood will depend on the duration of the disease itself.

Doctors often combine primary chronic deficiency with various other autoimmune endocrine disorders. In medicine, this is called autoimmune polyglandular syndrome (APPS). The following types of autoimmune polyglandular syndrome are classified:

APGS type I - this disease is uncommon, it is characterized by an autoimmune type of inheritance and is expressed by mucocutaneous candidiasis (this occurs in childhood), but at later stages adrenal insufficiency manifests itself;
APGS type II is a disease that is observed mainly in adults, more often in women.

The underlying cause of primary chronic adrenal insufficiency approximately a century ago was tuberculosis. Nowadays, when almost everyone knows about this disease and treats even its most severe forms, there are only 7-8% of cases in which the cause of adrenal insufficiency is tuberculosis.

The cause of primary adrenal insufficiency can be a generic (genetic) disease such as adrenoleukodystrophy. This disease affects the adrenal cortex and white matter of the nervous system. The disease occurs rarely, occurring in only 1 in 20,000 children born. A variety of this disease is often encountered - this is the cerebral form, it is severe and appears only at an older age (6-12 years).

A fairly rare disease that results in chronic adrenal insufficiency is metastatic lesions of the adrenal glands. Often these are metastases of large cell lymphomas and bronchogenic lung cancer, which provoke adrenal insufficiency.

The rarest cause of adrenal problems is a fungal infection. Among them are the following: paracoccidomycosis, coccidomycosis, blastomycosis.

The main forms of adrenal insufficiency are secondary and tertiary chronic insufficiency. Typical disorders of secondary adrenal insufficiency arise equally as a result of underproduction of ACHT by the pituitary gland and disruption of the shape of the pituitary stalk. Secondary chronic adrenal insufficiency appears with fairly massive processes in the sella turcica (these can be various tumors and cysts in the sellar region), and with surgical hypophysectomy, and with irradiation of the hypothalamic-pituitary region.

Causes of secondary adrenal insufficiency

The causes of secondary adrenal insufficiency can also be:

ischemia of the hypothalamus and pituitary gland;
hemorrhage into the pituitary gland and other vascular diseases;
thrombosis of the cavernous sinus;
metabolic disorders;
any radiation therapy, surgical interventions that lead to injuries.

Causes of tertiary adrenal insufficiency

The cause of tertiary chronic adrenal insufficiency will be long-term use of impressive doses of glucocorticoids, which are used in the treatment of certain diseases. A lack of ACHT and CRH secretion leads to disorders of the adrenal cortex; atrophic processes may begin, which risk not being restored.

Symptoms of adrenal insufficiency

Symptoms of adrenal insufficiency can appear at different stages in different forms.

Thus, primary chronic failure does not appear suddenly and quickly, it is characterized by:

slow increase in skin pigmentation;
frequent fatigue (even with the slightest physical exertion);
loss of appetite, and as a result - loss of body weight.

The main symptoms of this disease are muscle and general weakness, pain and body aches.

Muscular and general insufficiency can occur periodically in the first stages during any stress and depression. It may disappear after rest (for example, after a night's sleep), but then return again and may develop into a permanent symptom - asthenia. With this outcome, mental asthenia often develops. It is the failure of electrolyte and carbohydrate metabolism that leads to such unfavorable endings.
Hyperpigmentation of the mucous membranes and skin will also be a pronounced symptom of adrenal insufficiency. Intense symptoms of the disease are a consequence of the long duration of the disease. To begin with, those parts of the body that are constantly exposed and most often exposed to sunlight, for example, hands, face, neck, can change color and darken.
Vitiligo (pigmentless spots) is often found in patients; all this occurs against the background of hyperpigmentation. They can vary in size from the smallest to the largest; their contours can be irregular in shape.
Gastrointestinal disorders, which are accompanied by loss of appetite, gag reflexes, nausea - these symptoms can occur in the early stages, gradually continuing to increase. Digestive upset is quite rare. The cause of this kind of symptoms may be low production of pepsin and hydrochloric acid, as well as hypersecretion of chloride into the intestinal area. The body gradually loses sodium - this occurs due to diarrhea and vomiting, as a result - acute adrenal insufficiency is guaranteed. Doctors have already noticed that with this outcome, patients often require salty foods.
A constant symptom is weight loss, which can vary from moderate (4-6 kg) to significant (15-30 kg), especially when a person has been overweight for a long time.
Patients with chronic adrenal insufficiency may often present with hypoglycemic conditions. Such cases occur suddenly both on an empty stomach and after a meal (especially after a meal rich in carbohydrates) after 2-3 hours. These types of attacks are accompanied by weakness and sweating.
One of the symptoms of adrenal insufficiency can also be hypotension, which often manifests itself at the first stages of the disease. This may cause fainting and dizziness. The cause of this symptom will be a decrease in sodium in the human body, as well as a decrease in plasma volume.

Diagnosis of adrenal insufficiency

Before making a diagnosis, the patient needs to undergo a certain series of examinations and tests. But there is one “but”. Patients who have obvious symptoms of acute (crisis) adrenal insufficiency should be treated immediately, even without waiting for test results. In this case, it is impossible to stall for time under any circumstances, because a person’s life is at stake. If possible and given the available time, an ACTH stimulation test can be done fairly quickly, but there are cases when all examinations need to be postponed until the main symptoms are suppressed.

A random sample of a patient often reveals a low level of cortisol in the plasma. Even if the cortisol level is within the normal range, this is too low for a patient with an adrenal insufficiency crisis.

ACTH test

The ACTH test is more detailed and informative. The reaction and concentration of cortisol does not increase in response to exogenous administration of ACHT in primary chronic adrenal insufficiency. Primary or secondary adrenal insufficiency can be diagnosed by the ACTH level, that is, if the levels are low, then there is an ACTH deficiency; high levels will occur in Addison's disease.

Insulin hypoglycemia test

The insulin hypoglycemia test is the most accurate method for diagnosing secondary hypercortisolism in a patient, in which the degree of cortisol is determined during a hypoglycemic state provoked by the administration of short-acting insulin.

For a patient who has already received glucocorticoids, this type of test can be done first thing in the morning or at least 12 hours after the injection.

Other tests

If adrenal insufficiency is suspected, tests are carried out on the standard functioning of the thyroid gland, as well as the functions of the gonads, a blood test, it is necessary to determine the level of glucose and calcium in the plasma. Doctors may order a urine test and x-rays.

At the slightest suspicion of HIV infection, an analysis must also be carried out to confirm or refute the diagnosis.

Complications of adrenal insufficiency

Complications are accompanied by the progression of acute adrenal insufficiency, also known as crisis. With this outcome, the patient often experiences a decrease in glucocorticoids (adrenal cortex hormones). This kind of deterioration can occur with insufficient doses of hormones or a complete lack of treatment for the disease. Doctors classify the following symptoms of adrenal insufficiency crisis:

gastrointestinal symptoms and exacerbations;
cardiovascular symptoms;
neuropsychic form.

It is very important to consult a doctor in time if such symptoms are detected, because if the patient is not urgently hospitalized and the necessary treatment for adrenal insufficiency is not carried out, then there is every chance of the patient’s death.

Treatment of adrenal insufficiency

The choice of treatment method for adrenal insufficiency is determined by the following goals:

replacement of hormonal deficiency;
complete elimination of the cause of the disease.

Treatment of chronic adrenal insufficiency and elimination of the cause of this disease can be carried out with medication, radiation and surgery.

It is worth considering that in case of primary chronic adrenal insufficiency, glucocorticoid drugs (prednisolone, hydrocortisone), as well as mineralocorticoid drugs (fludrocortisone), are used. In secondary cases, only glucocorticoids are used.

The dosage of medications depends on the severity of the patient’s disease, as well as on the state of health.

The positive effects of therapy provoke a clear improvement in the patient’s well-being. After the patient's condition has stabilized, maintenance therapy should be continued.

Forecast and prevention of adrenal insufficiency

The prevalence of primary chronic adrenal insufficiency varies from forty to one hundred cases per year per one million people. The average age of patients ranges from 20 to 50 years, but deficiency is most common in patients aged 30 to 40 years.

People with symptoms of adrenal insufficiency without treatment will not be able to feel normal and lead a normal life. And with timely and correct treatment, they can live completely normally with the same duration and quality of life, you just need to choose the right dose of corticosteroids. The prognosis for adrenal insufficiency will be very favorable if replacement therapy is carried out in a qualified manner, and most importantly, in a timely manner. The prognosis may worsen if developing concomitant autoimmune diseases are present. Regarding prognosis for adrenoleukodystrophy, the outcome is poor and is determined by the rapid progression of the disease, primarily in the nervous system, and not by adrenal insufficiency.

As for the prevention of adrenal insufficiency, there is no such special therapy in medicine. If such a disease is familial (congenital), then medical genetic consultation is possible. First of all, it is important to recognize the disease in a timely manner. Further development of the first signs of acute hypocortisolism, as well as crisis, is easily prevented in patients with adrenal insufficiency during surgical interventions, childbirth or during pregnancy. In such cases, DOXA and glycocorticoids are prescribed to prevent the disease.

While pregnant, a woman should avoid the harmful effects of alcohol and tobacco products, which will be an excellent prevention of congenital underdevelopment of the adrenal glands, and subsequently adrenal insufficiency.

Adrenal insufficiency can be acute or chronic. Chronic adrenal insufficiency can be primary or secondary.

Primary adrenal insufficiency (Addison's disease) occurs as a result of destruction of the tissue of the adrenal gland itself. Primary adrenal insufficiency develops when less than 10-15% of the adrenal tissue is preserved and functioning.

Predisposing factors for primary adrenal insufficiency include:

infectious diseases (tuberculosis, fungal diseases of the adrenal glands);
adrenal amyloidosis;
HIV infection;
idiopathic atrophy of the adrenal cortex (an autoimmune process, in which the body’s immune control system is disrupted for an unknown reason and autoantibodies are formed that destroy the cells of its own adrenal glands).

Secondary insufficiency of the adrenal cortex occurs in diseases of the brain with damage to the pituitary gland or hypothalamus (brain tumors, traumatic brain injuries, after brain surgery, after various intoxications), which normally control the functioning of the adrenal glands.

Acute adrenal insufficiency, or Addisonian crisis, is an acute coma.

Acute adrenal insufficiency can develop:

Acute adrenal insufficiency can occur in a newborn due to hemorrhage in the adrenal glands during difficult and protracted labor, or as a result of exposure to various infections. This condition is called Waterhouse-Friderichsen syndrome. In adults and elderly people, hemorrhage into the adrenal glands can occur due to injuries to the abdomen and chest, during an overdose, during surgical interventions, sepsis, peritonitis, and burns.

With insufficiency of the adrenal cortex, the content of their hormones glucocorticoids and mineralocorticoids in the blood drops sharply. In this case, the body loses the ability to adapt to a stressful situation.

Signs characteristic of adrenal insufficiency

Primary adrenal insufficiency (Addison's disease) is relatively rare and occurs at any age and in both sexes.

Primary adrenal insufficiency usually begins gradually. At the beginning, complaints of weakness and fatigue appear, especially in the evening. Sometimes such weakness occurs only after physical exertion or stressful situations. Appetite worsens, patients often suffer from colds. Poor tolerance to solar radiation appears, accompanied by a persistent tan.

As Addison's disease progresses, muscle weakness becomes more pronounced. It is difficult for the patient to carry out any movements. Even the voice becomes quiet. Body weight decreases. Almost all patients develop persistent hyperpigmentation (increased skin coloration), especially in places where clothing rubs, in open areas of the body exposed to tanning, and increased coloration of the nipples, lips, and cheeks. There is a persistent decrease in blood pressure and increased heart rate. Disorders of the gastrointestinal tract appear: nausea, vomiting, constipation, followed by diarrhea. The amount of glucose in the blood decreases. Kidney function is impaired, often manifested by night urination. From the central nervous system, disturbances of attention, memory, and depressive states occur. In women, due to a lack of androgens, hair falls out on the pubis and armpits.

Patients with secondary adrenal insufficiency have most of the same signs and symptoms as patients with Addison's disease, but they are characterized by the absence of hyperpigmentation.

Addisonian crisis is characterized by: cardiovascular failure, drop in blood pressure, gastrointestinal disorders (uncontrollable vomiting, repeated loose stools), and neuropsychiatric disorders.

Forecast

The prognosis of patients with Addison's disease depends mainly on the prevention and treatment of Addisonian crises. In case of infection, injury, including surgery, gastrointestinal disorders or other types of stress, the dose of the hormone taken must be immediately increased. Treatment should be aimed at quickly increasing the level of glucocorticoids in the blood and replenishing sodium and water deficiency.

What can your doctor do?

Diagnosing the early stages of adrenal insufficiency is often difficult. However, mild gastrointestinal disturbances with weight loss, loss of appetite and suspected increased pigmentation justify special provocative tests to rule out adrenal insufficiency, especially before starting hormone replacement therapy. All patients with Addison's disease should receive specific hormone replacement therapy: glucocorticoids and mineralocorticoids.

During the treatment of patients with Addison's disease, body weight, serum potassium levels and blood pressure should be periodically recorded.

Glucocorticoid replacement therapy in patients with secondary adrenal insufficiency does not differ from that in patients with Addison's disease. Mineralocorticoid replacement therapy is usually not necessary. In all other respects, the treatment of patients with secondary adrenal insufficiency is based on the same principles.

Adequate replacement therapy with corticosteroid hormones under constant monitoring is the most effective method of preventing Addisonian crisis.

What can you do?

If the above symptoms appear, you should immediately consult a doctor. If this disease is not treated, Addisonian crisis may occur at any time, which is difficult to treat and can lead to death.

RCHR (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical protocols of the Ministry of Health of the Republic of Kazakhstan - 2013

Primary adrenal insufficiency (E27.1)

Endocrinology

general information

Short description

Approved by the Minutes of the meeting
Expert Commission on Health Development of the Ministry of Health of the Republic of Kazakhstan
No. 23 dated December 12, 2013

Chronic adrenal insufficiency(adrenal insufficiency, hypocortisolism) is a clinical syndrome caused by insufficient secretion of hormones from the adrenal cortex as a result of disruption of the functioning of one or more parts of the hypothalamic-pituitary-adrenal system.

I. INTRODUCTORY PART

Protocol name: Chronic adrenal insufficiency in adults
Protocol code:

ICD-10 code: E 27.1

Abbreviations used in the protocol:
CHI - chronic adrenal insufficiency
LH - luteinizing hormone
FSH - follicle stimulating hormone
TSH - thyroid stimulating hormone
STH - somatotropic hormone
ACTH - adrenocorticotropic hormone
BC - Koch bacillus
CT - computed tomography
MRI - magnetic resonance imaging
GPs - general practitioners

Date of protocol development: 2013

Protocol users: endocrinologists, therapists, GPs in clinics and hospitals.

Classification

Clinical qualifications
In clinical practice there are acute and chronic adrenal insufficiency (CAI).

In accordance with localization The pathological process is distinguished:
primary congenital indigestion - a decrease in the production of adrenal hormones as a result of a destructive process in the adrenal glands themselves;
secondary CNN - decreased or absent ACTH secretion by the pituitary gland;
tertiary CNN - inability of the hypothalamus to produce corticotropin-releasing hormone.

Primary chronic adrenal insufficiency
Causes of primary CNN are:
- autoimmune destruction of the adrenal cortex (85% of all cases), which is often combined with damage to other endocrine glands;
- tuberculosis, tumor metastases, adrenoleukodystrophy, HIV infection, fungal infections, syphilis, amyloidosis;
- iatrogenic factors - removal of the adrenal glands due to Itsenko-Cushing's disease, bilateral hemorrhage in the adrenal glands during anticoagulant therapy, use of blockers of steroidogenesis in the adrenal glands (aminoglutethimide, chloditan, ketoconazole, barbiturates, spironolactone).

Secondary and tertiary chronic adrenal insufficiency
Secondary and tertiary CNN - central forms of adrenal insufficiency. Secondary CNN develops as a result of a decrease in the production of ACTH by the pituitary gland, tertiary - with damage to the hypothalamus and a violation of the integrity of the pituitary stalk. Secondary and tertiary CNN usually develop simultaneously with a deficiency of other pituitary tropic hormones (luteinizing hormone (LH), follicle-stimulating hormone (FSH), thyroid-stimulating hormone (TSH), somatotropic hormone (GH)). Isolated ACTH deficiency is extremely rare. The causes of secondary and tertiary CIU are presented in table. 2.

Most often, secondary CNN within the framework of hypopituitarism occurs with volumetric processes in the area of the sella turcica (tumors of the pituitary gland and various formations of the sellar and parasellar regions), irradiation of the hypothalamic-pituitary region, hypophysitis and surgical hypophysectomy.

The most common cause of tertiary CIU is long-term use of suppressive doses of glucocorticoids in the treatment of various systemic diseases. Long-term insufficiency of CRH and ACTH secretion leads not only to functional, but also morphological changes in the adrenal cortex: atrophy of the zona fasciculata and reticularis of the cortex develops. The zona glomerulosa and therefore mineralocorticoid secretion are less affected.

Reasons leading to the development of secondary and tertiaryadrenal insufficiency

Secondary CNN
(destruction or absence of ACTH-secreting cells)

Tertiary CNN
(CRH deficiency)

I. Tumors of the sellar and parasellar region (pituitary adenomas, craniopharyngioma, meningioma, glioma, mucocele)
II. Pituitary ischemia
1. Postpartum (Sheehan syndrome)
2. Systemic diseases (temporal arteritis, sickle cell anemia)
III. Hemorrhage into the pituitary gland
IV. Iatrogenic (after irradiation of the pituitary gland, surgery)
V. Empty sella syndrome
VI. Autoimmune lymphocytic hypophysitis
VII. Intracranial aneurysm of the internal carotid artery
VIII. Thrombosis of the cavernous sinus
IX. Infectious diseases (tuberculosis, syphilis, malaria, meningitis)
X. Infiltrative lesions (hemochromatosis, histiocytosis X)
XI. Metabolic disorders (chronic renal failure)
X II. Idiopathic or genetic (insufficient production of ACTH, synthesis of abnormal forms of ACTH)

I Lesions of the hypothalamus or other parts of the central nervous system:
1. Traumatic, incl. postoperative
2. Irradiation of the hypothalamic region
3. Tumor (primary, metastatic, lymphoma)
4. Violations of the integrity of the pituitary stalk
5. Anorexia nervosa
6. Inflammatory (sarcoidosis, histiocytosis X)
7. Infiltrative (lipid deposition diseases)
8. Toxic
9. Nutritional (fasting, obesity)
10. Idiopathic or genetic (congenital or familial)
II. Long-term use of suppressive doses of glucocorticoids for various somatic diseases

By severity Both primary and secondary adrenal insufficiency are divided into forms: mild, moderate and severe.

According to the severity of clinical manifestations CNN is divided into explicit and latent. During the treatment of CNN, the following may be identified: phases: decompensation, subcompensation and compensation.

Diagnostics

II. METHODS, APPROACHES AND PROCEDURES FOR DIAGNOSIS AND TREATMENT

List of basic and additional diagnostic measures

Basic
Outpatient:
- general blood analysis
- general urine analysis

In the hospital:
- determination of blood levels of renin, ACTH, cortisol, aldosterone, circadian rhythm of cortisol,
- determination of blood electrolytes.

Outpatient:
- ECG,
- X-ray of the chest organs.

In the hospital:
- CT scan of the adrenal glands,
- MRI of the brain with contrast.

Additional:
- culture of sputum for BC.

Diagnostic criteria HNN

Complaints and anamnesis:
- gradual loss of body weight,
- weakness,
- low blood pressure,
- darkening of the skin and mucous membranes with primary congenital incontinence,
- attacks of hunger,
- darkening of the eyes in the morning on an empty stomach.

Physical examination
In general, CUI is characterized by an insidious onset and slow progression of fatigue, muscle weakness, loss of appetite, weight loss, hypotension, and sometimes hypoglycemia.
The main difference primary congenital indigestion secondary is the presence of hyperpigmentation of the skin and mucous membranes.

Main symptoms of chronic adrenal insufficiency

For secondary and tertiary congenital indigestion symptoms of mineralocorticoid deficiency are less pronounced or even absent, and there are almost always clinical signs of deficiency of other pituitary tropic hormones - hypogonadism, hypothyroidism, growth hormone deficiency.
Nelson's syndrome develops in patients at different intervals after total adrenalectomy. The clinical picture of Nelson's syndrome is characterized by a labile course of CNN, progressive hyperpigmentation of the skin, an ACTH-producing pituitary tumor, ophthalmological and neurological disorders.

With severe decompensation of CNN as a result of stress, acute infection, or surgical intervention, the development of acute adrenal insufficiency - Addisonian crisis. Addisonian crisis often develops gradually over several days, less often acutely - over several hours. Symptoms: progressive decrease in blood pressure, increased general weakness, hyperpigmentation, vomiting, abdominal pain, dehydration.

Asthenia. General and muscle weakness can initially only occur periodically - during stress. In the early stages, they increase towards the end of the day and disappear after a night's rest; later they increase and become permanent, acquiring the character of adynamia. Along with adynamia, mental asthenia develops up to the development of psychosis.

Hyperpigmentation of the skin and mucous membranes- a frequent and early sign of primary CNN. Hyperpigmentation has the appearance of diffuse brown or bronze darkening on both open and closed parts of the body, especially in places of friction with clothing, on the palmar lines, in the area of postoperative scars, on the oral mucosa, in the area of the nipple areola, anus, and external genitalia. Some patients develop dark freckles, and sometimes areas of depigmentation form - vitiligo.
As an early sign, patients may notice unusual preservation of the tan after sun exposure.

Gastrointestinal disorders- increasing loss of appetite, nausea, vomiting. Some patients feel a constant need for salty food.

Weight loss is a constant symptom of adrenal insufficiency and is associated with decreased appetite, impaired absorption in the intestines, and dehydration.

Hypotension- one of the characteristic symptoms of adrenal insufficiency, often occurs in the early stages of the disease. Systolic blood pressure 90-80 mm Hg. Art., diastolic - below 60 mm Hg. Art. Dizziness and fainting.
However, in patients with concomitant arterial hypertension, blood pressure may be normal or elevated.

Hypoglycemic conditions in patients with chronic adrenal insufficiency, they can occur both on an empty stomach and 2-3 hours after eating a meal rich in carbohydrates. Attacks are accompanied by weakness, hunger, and sweating.

Central nervous system dysfunction occur in more than half of patients and are manifested by a decrease in mental activity and memory, apathy, and irritability.

It is often noted nocturia against the background of decreased glomerular filtration and renal blood flow.

Cessation of secretion adrenal androgens in women it can determine a decrease in libido and plays a role in the reduction and complete disappearance of axillary and pubic hair.

In a small proportion of patients with long-term presence of hypocortisolism, the presence of calcification of ear cartilage(probably due to the resulting hypercalcemia).

Laboratory research
Criteria for primary CNN:
- increased levels of ATG and renin in the blood plasma,
- decrease in the levels of aldosterone and cortisol in the blood plasma,
- disturbance of the rhythm of cortisol secretion, hyperkalemia, hyponatremia, hypochloremia.

Criteria for secondary congenital indigestion:
- increased levels of ACTH, renin, aldosterone, cortisol in the blood plasma.
- Disturbance in the rhythm of cortisol secretion, hyperkalemia, hyponatremia, hypochloremia.

Instrumental studies
- ECG signs (due to hyperkalemia): low voltage, high pointed T wave, slow conduction, prolongation of the ST interval and QRT complex.
- X-ray of the chest organs: with primary congenital insufficiency - signs of pulmonary tuberculosis are possible
- CT scan of the adrenal glands: reduction in size
- MRI of the brain with contrast: in case of secondary and tertiary CNN - organic brain lesions are possible
- Sputum culture for BC: for pulmonary tuberculosis - positive result

Specialist consultations
According to indications - phthisiatrician, neurosurgeon, neurologist.

Differential diagnosis

Differential diagnosis of primary and secondary CNN

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Treatment

Goal of treatment: elimination of clinical and laboratory signs of hypocortisolism.

Treatment tactics

Non-drug treatment: gentle regime, food enriched with table salt and ascorbic acid.

Drug treatment
Treatment of CNN is aimed at replacing hormonal deficiency and, if possible, eliminating the pathological process that caused damage to the adrenal glands.

Treatment of chronic adrenal insufficiency

Etiotropic	Pathogenetic	Symptomatic
MEDICATION (therapy of tuberculosis, sepsis, hypophysitis, fungal diseases, etc.)	GLUCOCORTICOIDS (hydrocortisone, cortisone acetate, prednisolone)	Diet enriched with table salt
RADIAL (tumors of the pituitary gland, hypothalamus)	MINERALOCORTICOIDS (fludrocortisone, deoxycorticosterone acetate)	VITAMIN THERAPY
NEUROSURGICAL (removal of a tumor, aneurysm, etc. of the brain)	ANABOLIC STEROID (nandrolone, estrenol trimethylsilyl ether)

Replacement therapy with synthetic hormones is vital and cannot be canceled under any circumstances.

In clinical practice, hydrocortisone (cortisol), cortisone acetate and their semisynthetic derivatives are used. The latter, in turn, are divided into non-fluorinated (prednisone, prednisolone, methylprednisolone) and fluorinated (triamcinolone, dexamethasone and betamethasone).

When taken orally, glucocorticoids are rapidly and almost completely absorbed in the upper jejunum. Eating does not affect the degree of absorption of hormones, although the rate of this process slows down somewhat.

The peculiarities of the use of injectable forms are determined both by the properties of the glucocorticoid itself and the ester associated with it. For example, succinates, hemisuccinates and phosphates are water-soluble and, when administered parenterally, have a rapid but relatively short-term effect. In contrast, acetates and acetonides are finely crystalline suspensions and are insoluble in water. Their action develops slowly, over several hours, but lasts a long time. Water-soluble glucocorticoid esters can be used intravenously, fine-crystalline suspensions cannot.

Depending on the duration of action, all glucocorticoids are divided into 3 groups: short-acting, medium-acting and long-acting.

Dosage equivalence of glucocorticoid drugs

Duration of action	Drug name	Equivalent dosage (mg)
Short acting	Hydrocortisone	20
	Cortisone	25
	Prednisone	5
	Prednisolone	5
	Methylprednisolone	4
Average duration of action	Triamcinolone	4
Average duration of action	Paramethasone	2
Long-lasting	Dexamethasone	0,75
Long-lasting	Betamethasone	0,6

Hydrocortisone and cortisone preparations, in addition to glucorticoid, also have mineralocorticoid activity, although weaker than true mineralocorticoids. Non-fluorinated semisynthetic glucocorticoids also have mineralocorticoid effects (the severity of which, in turn, is inferior to the effects of natural glucocorticoids). Fluorinated drugs do not have mineralocorticoid activity.

Comparative characteristics of gluco- and mineralocorticoid drugs

Drug name	Biological half-life, min	Protein binding, %	Glucocorticoid activity	Mineral-corticoid activity
HYDROCORTISONE	80	80	1	1
CORTISONE	30	75	0.8	0.6
PREDNISONE	60	72	3.5	0.4
PREDNISOONE	200	73.5	4	0.6
METHYL- PREDNISOONE	160	60	5	0.5
DEXAMETHASONE	240	61.5	30	0
ALDOSTERONE	50	67	0.3	750

The glucocorticoid activity of semisynthetic drugs is higher than that of hydrocortisone and cortisone, which is explained by lower protein binding compared to natural glucocorticoids. A feature of fluoridated drugs is their slower metabolism in the body, which entails an increase in the duration of action of the drugs.

HYDROCORTISONE is the only glucocorticoid that satisfies all the requirements for drugs used for continuous replacement therapy of CNN. Hydrocortisone is almost 4 times weaker than prednisolone in glucocorticoid activity, but exceeds it in the severity of mineralocorticoid action. In cases of congenital insufficiency, as well as in acute adrenal insufficiency and other emergency conditions, hydrocortisone drugs are the drugs of choice.

Basic principles of CNN replacement therapy
1. In patients with CIU, glucocorticosteroids are used for life;

2. Physiological doses of glucocorticoids are used for replacement therapy of CNN. The drugs are administered taking into account the rhythm of glucocorticoid secretion (2/3 of the daily dose in the morning and 1/3 in the evening).

3. The main drugs for glucocorticoid replacement therapy for chronic indigestion are hydrocortisone, cortisone, prednisolone, and for the correction of mineralocorticoid deficiency - fludrocortisone.

4. Triamsinolone, dexamethasone, beclamethasone and other synthetic analogues forCNNs do not apply, since these drugs are practically devoid of mineralocorticoid activity and cannot provide significant assistance in the correction of water-electrolyte disorders and hemodynamic disorders. In addition, they have a number of complications (signs of overdose quickly develop, damage to various organs and systems appears, such as Cushing’s syndrome).

5. Adequacy assessment Replacement therapy with gluco- and mineralocorticoids is carried out according to clinical and laboratory parameters: general condition, appetite, physical activity, body weight dynamics, blood pressure, heart rate, level of electrolytes in the blood, fasting blood glucose. In primary congenital insufficiency, the objective criterion for compensation of mineralocorticoid deficiency is the normalization of plasma renin content and the normalization of glucocorticoid ACTH concentration.

Determination of blood cortisol and free urine cortisol while taking glucocorticoid drugs to select their adequate dose is not informative and is impractical to carry out.

Features of replacement therapy for chronic congenital insufficiency
Doses of drugs depend on the severity of the disease, the degree of compensation, and the state in which the patient’s body is (stress, rest). At mild form of CNN Hydrocortisone is prescribed at a dose of 15 mg per day or cortisone acetate at a dose of 12.5-25 mg per day in one or two doses. If the drug is taken once a day, then in the morning after breakfast.

In case of illness moderate to severe Combination therapy is usually prescribed - a combination of hydrocortisone or cortisone acetate, prednisolone and mineralocorticoids. For moderate severity of the disease, hydrocortisone 15-20 mg in the morning, 5-10 mg in the afternoon + fludrocortisone 0.1 mg in the morning is usually prescribed; prednisolone 5-7.5 mg and 0.1 mg fludrocortisone after breakfast, hydrocortisone 10 mg after lunch + 5 mg after dinner or cortisone acetate 25 mg after lunch + 12.5 mg after dinner.

At severe decompensation It is advisable to transfer patients to intramuscular injections of hydrocortisone - at least 3-4 injections per day at a dose of 75-100 mg, followed by a gradual reduction in the dose and transfer of the patient to taking oral medications. Any stress, including febrile illness, trauma, surgery, can trigger Addisonian crisis. Therefore, it is necessary to pre-administer additional doses of glucocorticoids (the dose of glucocorticoids and mineralocorticoids is increased by 2-5 times compared to the maintenance dose). For mild or moderate infectious diseases, it is enough to increase the dose of glucocorticoids by 2-3 times. If the disease occurs with vomiting, as well as when symptoms of adrenal crisis appear, the patient should be hospitalized for intensive measures. In case of severe infectious diseases or surgical operations under general anesthesia, intravenous administration of hydrocortisone (100 mg every 8 hours) or hydrocortisone acetate 50 mg IM every 4-6 hours is usually required. Impact doses of glucocorticoids are reduced quickly - 1 or 2 days after the elimination of the stressful situation.

The dose of replacement therapy during pregnancy remains the same, with a slight increase in dose required after three months of pregnancy. During childbirth, hormones are administered under the same conditions as during planned operations.

With glucocorticoid replacement therapy, it is possible to develop signs of overdose drugs: rapid weight gain, muscle weakness, increased blood pressure, headaches, fluid retention (edema), decreased potassium levels and increased sodium levels in plasma. In this case, the dose of administered drugs should be reduced. Elimination of symptoms of hypercortisolism occurs slowly - within 4-8 weeks.

At combination of CNN and peptic ulcer stomach and/or duodenum, preference is given to mineralocorticoids. In cases of insufficient therapeutic effect, glucorticoids are additionally prescribed, starting with small doses under the cover of antacid drugs with the obligatory simultaneous administration of anabolic steroids. In rare cases, with severe ulcerative lesions, intramuscular administration of prolonged forms (depot forms) of glucocorticoids (methylperdnisolone) is used in combination with misoprostol drugs - synthetic analogues of prostaglandin E1. The latter have a cytoprotective effect associated with increased mucus formation and increased bicarbonate secretion by the gastric mucosa, thereby increasing its resistance to harmful factors.

At combination of congenital insufficiency and diabetes mellitus It is preferable to prescribe mineralocorticoids, which have virtually no effect on carbohydrate metabolism.

If the effectiveness is insufficient, glucocorticoids are used.

In cases of combination CHF and arterial hypertension First of all, glucocorticoids with minimal mineralocorticoid effect are prescribed. Preference is given to prednisolone, which is prescribed under the control of blood pressure levels, general condition, study of electrolytes and blood sugar.

Other types of treatment: according to indications, treatment of pulmonary tuberculosis and associated diseases

Surgical intervention:
According to indications - operations for space-occupying processes in the brain

Preventive actions
Teaching a patient suffering from any form of chronic adrenal insufficiency to regularly take adequate doses of gluco- and mineralocotrichoid drugs, as well as to independently double their dosages under any stress (intercurrent illnesses, operations, emotional stress, etc.).

Work ability
Patients with chronic adrenal insufficiency are advised to transfer to light work with a standardized working day, excluding night shifts, business trips, and heavy physical activity. During the period of decompensation of hypocortisolism, patients are considered disabled. In severe cases of adrenal insufficiency, transfer to disability is indicated.

Further management: in patients with any form of chronic adrenal insufficiency, before expected stress, for example, before childbirth, major and minor surgical interventions, hydrocortisone is administered intramuscularly 25-50 mg 2-4 times a day, deoxycorticosterone acetate - 5 mg per day. On the day of surgery, the dose of the drug is increased 2-3 times. During surgery, 100-150 mg of hydrocortisone is administered intravenously and 50 mg of hydrocortisone intramuscularly every 4-6 hours for 1-2 days. Parenteral administration of hydrocortisone is continued after surgery for 2-3 days. Then they are gradually transferred to replacement therapy with prednisolone, hydrocortisone or cortisone and fludrocortisone per os. After eliminating stress, the patient is transferred to the previous dose.

Indicators of treatment effectiveness and safety of diagnostic and treatment methods described in the protocol: elimination of clinical and laboratory signs of hypocortisolism.

Hospitalization

Indications for hospitalization indicating the type of hospitalization

Emergency
- acute adrenal insufficiency - emergency

Planned
- clarification of the diagnosis of chronic congenital disorder
- selection of adequate doses of replacement therapy
- decompensation of chronic adrenal insufficiency

Information

Sources and literature

Minutes of meetings of the Expert Commission on Health Development of the Ministry of Health of the Republic of Kazakhstan, 2013
1. 1. Endocrinology according to Williams. Diseases of the adrenal cortex and endocrine arterial hypertension. Translation from English Edited by acad. RAS and RAMS Dedov I.I., Moscow, 2010. 2. Algorithms for the diagnosis and treatment of diseases of the endocrine system. / Edited by I.I. Dedova, Moscow, 1995. 3. Balabolkin M.I. “Endocrinology”, Moscow, “Universum Publishing”, 1998, pp. 492-520. 4. Bereznyakov I.G. “Glucocorticosteroids in clinical practice”, Pharmacist, 1998. 5. Jones R. “Adrenal insufficiency” / Secrets of endocrinology, Moscow, ZAO BINOM Publishing House, 1998, pp. 217-224. 6. Zelinsky B.A. “Addison’s disease”, Kyiv, “Health”, 1988. 7. Zefirova G.S., Baysugurov M.Sh. “Diagnostics of hypocortisolism”, Moscow, 1988. 8. Knappe G. “Hormones of the adrenal cortex and ACTH” / Hormone therapy edited by H. Schambach, G. Knappe, V. Karol, Moscow, Medicine, 1988, pp. 68-100. 9. Marova E.I. Chronic adrenal insufficiency / Clinical endocrinology, edited by prof. N.T. Starkova, Moscow, Medicine, 1991, pp. 312-323. 10. Melnichenko G.A., Fadeev V.V. “Laboratory diagnosis of adrenal insufficiency”, Problems of endocrinology, 1997, T.43, No. 5, pp. 39-47. 11. Melnichenko G.A., Fadeev V.V., Buziashvili I.I. “Etiological aspects of primary chronic adrenal insufficiency”, Problems of endocrinology, 1998, T.44, No. 4, pp. 46-55. 12. Nasonov E.L. “General characteristics and mechanisms of action of glucocorticosteroids”, Russian Medical Journal, Volume 7, No. 8 (90), 1999, pp. 364-370. 13. Fadeev V.V. "Primary chronic adrenal insufficiency (etiology, clinical picture, replacement therapy)." Author's abstract. Ph.D. honey. Sci. Moscow, 1999. 14. Williams G., Dlyukhi R. “Diseases of the adrenal cortex” / Internal diseases edited by T.R. Harrison, book 9, Moscow, “Medicine”, 1997, pp. 134-177. 15. Stern N., Tak M. “Diseases of the adrenal cortex” / Endocrinology, edited by N. Lavin, Moscow, Praktika, 1999, pp. 173-221.

Information

III. ORGANIZATIONAL ASPECTS OF PROTOCOL IMPLEMENTATION

List of protocol developers:
1. Bazarbekova R.B. - Doctor of Medical Sciences, Professor, Head. Department of Endocrinology, RGKP "Almaty State Institute for Advanced Training of Physicians"
2. Dosanova A.K. - Candidate of Medical Sciences, Assistant of the Department of Endocrinology, Almaty State Institute for Advanced Training of Physicians.

Reviewer: Doctor of Medical Sciences, Professor of the Department of Endocrinology of KazNMU named after S.D. Asfendiyarova Nurbekova A.A.

Indication of no conflict of interest: absent.

Indication of the conditions for reviewing the protocol:
This protocol is subject to revision every three years, or when new proven data becomes available.

Attached files

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