Adrenal insufficiency (hypocorticism) is a deficiency of adrenal hormones. It is accompanied by a bronze coloration of the skin and mucous membranes, vomiting, diarrhea, weight loss, weakness, a tendency to faint, disorders of cardiac activity and water-salt metabolism.
Refers to the number of severe endocrine pathologies. It proceeds chronically, accompanied by acute crises that pose a danger to the life of the patient. It can be diagnosed in people of any age, including children of the first years of life. It is more common in women 20-55 years old.
Reasons for development
Primary adrenal insufficiency occurs when the adrenal gland does not work properly. Caused by the following reasons:
- Underdevelopment of organs. A rare hereditary disease that affects male patients.
- Immune to ACTH. Cortical receptors do not respond to adrenocorticotropic hormone, which normally stimulates the production of cortisol and aldosterone. It is observed in Allgrove’s syndrome, family glucocorticoid insufficiency.
Destruction of adrenal tissue:
- Autoimmune atrophy. It accounts for 85% of the total number of cases. Autoimmune bodies, for some unknown reason, attack the cells of the adrenal glands, recognizing them as foreign, “hostile”. Perhaps a combination with an autoimmune lesion of other organs (polyglandular syndrome).
- Infectious diseases. Tissue destruction occurs under the influence of bacteria, fungi and viruses. Tuberculosis has the greatest clinical significance (1-2% of cases). Rarely, organ destruction is observed in syphilis, histoplasmosis, blastomycosis, sepsis, and HIV-associated infections.
- A consequence of the treatment of other diseases. Adrenal insufficiency is formed after bilateral adrenalectomy for Cushing’s disease, while taking certain medications: steroid blockers, anticoagulants, barbiturates.
- Other reasons. Other causes of tissue destruction include hemorrhages in the adrenal glands, metastases from tumors of other organs, amyloidosis, hemochromatosis, and adrenoleukodystrophy.
In addition, primary adrenal insufficiency is formed when steroid production is impaired due to mitochondrial diseases or a genetically determined disorder of sterol metabolism.
Secondary and tertiary adrenal insufficiency occurs due to malfunction of the hypothalamic-pituitary structures, which are located in the brain, regulate the synthesis of hormones by the endocrine organs. Violation of regulation can be congenital or acquired. The acquired variant of the pathology is provoked by the following factors:
- traumatic brain injury;
- infections, the formation of infiltrates in sarcoidosis and syphilis;
- surgical interventions;
- pathology of cerebral vessels;
- abrupt withdrawal of glucocorticoids after prolonged use.
One of the most important functions of cortisol is the effect on glucose metabolism – with a lack of the hormone, hypoglycemia develops, which is accompanied by changes in heart rate and blood pressure, and disorders of the nervous system. Aldosterone has an effect on the functioning of the kidneys, which causes a decrease in the level of sodium in the blood. Because of this, blood pressure drops (up to collapse and shock), arrhythmias occur, myopathies form, and bowel function is disrupted.
In primary adrenal insufficiency, the levels of both hormones, cortisol and aldosterone, decrease. In other cases, cortisol falls, aldosterone remains normal, so the secondary and tertiary forms are easier. In addition, due to differences in the compensatory reactions of the body, these forms do not increase the production of melatonin, so the skin color does not change.
In clinical practice, the following classification of adrenal insufficiency is used:
- Primary – damage directly to the adrenal cortex.
- Secondary – damage to the pituitary gland with a decrease in the level of ACTH.
- Tertiary – damage to the hypothalamus with a violation of the production of substances that stimulate the production of ACTH.
With the flow:
- Acute. It occurs suddenly against the background of hemorrhagic shock, severe traumatic injuries, hemorrhages in both adrenal glands in patients with sepsis. May complicate end-stage chronic liver failure.
- Chronic. It develops slowly, accompanied by a gradual decrease or a stable low level of hormones.
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- Compensation. Treatment with hormones allows you to fully compensate for the lack of hormones and bring the body into a state close to the physiological norm. The patient feels well.
- Sub Compensation. It develops in stressful conditions or during acute illnesses. The body requires higher doses of hormones, and prescribed drugs do not fully meet the existing need. There are mild or moderately pronounced signs of adrenal insufficiency.
- Decompensation. The lack of hormones becomes so significant that there is a risk of a life-threatening condition – an Addisonian crisis.
Primary adrenal insufficiency
This type of disease is characterized by a detailed clinical picture with discoloration of the skin, digestive disorders and a complex of general manifestations that resemble exhaustion in chronic fatigue syndrome.
Skin color changes
The most noticeable symptom of primary hypocorticism is a bronze hue of the skin and mucous membranes. The intensity of hyperpigmentation is determined by the duration and severity of adrenal insufficiency. Open areas that are often exposed to the sun’s rays darken more: face, neck, hands, forearms. The skin may look slightly tanned, smoky, bronzed, dirty, or very dark.
At the same time, the color of the skin in the initially pigmented areas changes – in the area of the nipples, armpits, scrotum, perineum. Places of friction against clothing, natural skin folds (for example, along the flexor surface of the joints) are intensively stained. Visible mucous membranes – gums, palate, tongue, inner surface of the cheeks, vagina – acquire a bluish-black tint.
Patients with mild hyperpigmentation due to primary adrenal insufficiency are rare. A more common option is the formation of vitiligo – whitish spots devoid of pigment that stand out brightly on darkened skin.
Weight loss is typical, but the degree of weight loss varies greatly – from insignificant (by several kilograms) to pronounced (20 kilograms or more). Other signs include weakness, lethargy, irritability, unexplained fatigue, decreased exercise tolerance, memory impairment. Depression is often present.
There is a decrease in libido. Patients suffer from orthostatic hypotension – sudden dizziness when moving to an upright position. Frequent fainting is found with strong excitement and stress. Patients with previously normal blood pressure develop hypotension; in people with hypertension, blood pressure sometimes drops to normal levels.
Gastrointestinal manifestations are observed in almost all patients. There may be a decrease in appetite, nausea, vomiting, pain in the epigastric region, constipation or diarrhea. Due to metabolic disorders, an addiction to salty foods develops. Some patients not only oversalt food, but also use salt in its pure form.
Other types of hypocorticism
For secondary and tertiary adrenal insufficiency, general non-specific symptoms and attacks of hypoglycemia that occur several hours after a meal are typical. Arterial hypotension and disorders of the digestive system are absent. Skin color does not change. An addiction to salty foods does not develop.
In the absence of treatment, an incorrect regimen of therapy, irregular intake of medications, a life-threatening complication, the Addisonian crisis, can occur. The condition occurs suddenly, accompanied by a sharp weakness, a rapid drop in blood pressure, followed by the development of collapse and loss of consciousness.
Repeated vomiting, persistent diarrhea, and convulsions are observed. Rapidly formed and progresses acute heart failure. The smell of acetone comes from the mouth. There are three types of Addisonian crisis:
- Cardiovascular. Symptoms from the heart and blood vessels predominate: pallor and cooling of the skin, acrocyanosis, lowering blood pressure, tachycardia, weakening of the pulse, collaptoid state.
- Gastrointestinal. Reminds me of the clinical picture of an acute abdomen or food poisoning. The patient is concerned about pain and cramps in the abdomen. Bloating, discharge of liquid feces mixed with blood, indomitable vomiting are noted.
- Neuropsychic. At the initial stage, the patient complains of a severe headache. Then there are convulsions, impaired consciousness, stupor, lethargy, delirium. An objective examination reveals focal and meningeal symptoms.
Diagnosis of hypocorticism is carried out by an endocrinologist. If a tuberculous origin of adrenal insufficiency is suspected, patients are referred to a phthisiatrician. With the possible tumor nature of the pathology, an oncologist is involved in the examination. To make a diagnosis, anamnesis data, the results of an objective examination and additional studies are used:
- Hormone tests. The level of cortisol in the blood and urine is reduced. With primary hypocorticism, an increased level of ACTH is detected in the blood, with a secondary form of the disease – a reduced content of ACTH. In mild cases, the indicators may be within the normal range, therefore, with negative results of baseline studies, stimulation tests are performed.
- Blood tests. As part of a biochemical study, the level of sugar and electrolytes is determined. With adrenal insufficiency, an increase in the content of potassium, a decrease in the concentration of sodium and glucose are found. In the general analysis, hypo- or normochromic anemia, leukopenia, eosinophilia and relative leukocytosis are determined.
- Antibody test. Allows detection of circulating antibodies to 21-hydroxylase. It is rarely used in Russian clinical practice. Of indirect importance is the determination of antibodies to other endocrine organs, primarily to thyroid tissue, indicating a polyglandular syndrome.
- Other techniques. To rule out tuberculosis, an MRI or CT scan of the adrenal glands is prescribed. If a lesion of the hypothalamus and pituitary gland is suspected, radiography of the sella turcica, CT and MRI of the brain, an ophthalmological examination with determination of visual fields, and an examination of the fundus are performed.
The tactics of treatment is determined by the cause of adrenal insufficiency:
- Infectious diseases. Taking into account the etiology, long-term course treatment with anti-tuberculosis drugs is carried out, antifungal agents or drugs that are effective for syphilis are prescribed.
- Action of drugs. The drug therapy that provoked hypocorticism is corrected. Taking drugs replaces or reduces the dose.
- Tumors. Neoplasms of the pituitary and hypothalamus are removed surgically or radiation therapy is performed. Often both methods are combined.
In some cases, it is not possible to influence the cause of the disease. Even after treatment of the underlying pathology, with irreversible changes in the adrenal glands or the hypothalamic-pituitary system, patients require subsequent hormone replacement drug therapy.
Patients with primary adrenal insufficiency are shown to take glucocorticoids and mineralocorticoids continuously. In mild cases, hydrocortisone or cortisone is used. For moderate to severe hypocorticism, hydrocortisone, cortisone acetate, or prednisolone is given in combination with decocorticosterone acetate or decocorticosterone trimethylacetate.
The drugs act for 6-12 hours. Reception should not be postponed, this is fraught with the occurrence of an Addisonian crisis. It is better to schedule the hydrocortisone in the evening so that it does not occur before bedtime, since this medicine provokes insomnia. The dose of medicines is increased by 2-3 times, and then gradually reduced in the following cases:
- acute respiratory infections, food poisoning, other conditions accompanied by a significant rise in temperature;
- severe stress, situations of strong excitement;
- feeling of weakness, decreased ability to work, loss of strength.
In secondary and tertiary hypocorticism, treatment is carried out only with glucocorticoids; mineralocorticoids are not required. Patients with all variants of adrenal insufficiency are prescribed a diet rich in proteins, fats, carbohydrates and sodium salts, but with a restriction of potassium salts. With the development of an Addisonian crisis, glucocorticoids are administered intravenously in combination with infusions of an isotonic solution and a glucose solution.
In the treatment of adrenal insufficiency, only hormonal drugs are effective. There are no analogues in traditional medicine. No folk remedy can normalize hormone levels. Any attempts to be treated using folk recipes create an immediate threat to the life of the patient.