Addison's disease has another name - bronze disease. By this is meant a violation of the functioning of the adrenal glands. In turn, this disrupts the hormonal balance, as a result, the synthesis of glucocorticoids decreases or completely disappears.

Addison-Birmer disease has a large number of symptoms, which mainly arise due to the defeat of most of the cortex. The cause of this disease can be different. In 8 out of 10 cases, Addison-Birmer disease develops due to an autoimmune process in the body.

But also sometimes the disease can be accompanied by tuberculosis, which has affected the adrenal glands. Pathology can be congenital and can be inherited. The autoimmune type of the disease is most common in the female half of the population.

The most common symptoms of Addison's disease are pain, disturbances in the functioning of the gastrointestinal tract and hypotension. Pathology can lead to metabolic disorders. Such a disease can also be treated with the help of traditional medicine, which will enhance the work of the adrenal glands, as well as help in the fight against microbes and inflammation.

General characteristics of the disease

Addison's disease, the photo of which clearly shows the affected area, can be with both primary and secondary insufficiency. As many people know, pathology affects the glands internal secretion responsible for the production of some of the most important hormones in the human body. These organs have 2 zones:

• crust;
• brain substance.

Each zone is responsible for synthesizing different types of hormones. The medulla produces norepinephrine and adrenaline. They are especially necessary for a person in stressful situation, these hormones will help to use all the reserves of the body.

Other hormones are also synthesized in the cortex.

• Corticosterone. It is necessary for the balance of water and salt metabolism in the body, and is also responsible for the regulation of electrolytes in blood cells.
• Deoxycorticosterone. Its synthesis is also required for water-salt metabolism, in addition, it affects the efficiency and duration of muscle use.
• Cortisol is responsible for the regulation of carbon metabolism, as well as for the production of energy resources.

The pituitary gland, a gland located in the region of the brain, has a great influence on the adrenal cortex. The pituitary gland produces a special hormone that stimulates the adrenal glands.

As mentioned above, Addison-Birmer disease has two types. Primary this is the disease itself, when the work of the adrenal glands is completely disrupted due to negative factors. The secondary implies a decrease in the amount of ACTH synthesized, which, in turn, impairs the functioning of the endocrine glands. In a situation where the pituitary gland produces an insufficient amount of hormones for a long period, dystrophic processes may begin to develop in the adrenal cortex.

Causes of the disease

The primary form of Addison-Birmer disease is quite rare. It is equally likely to be found in both men and women. In most cases, the diagnosis is made to people whose age is from 30 to 50.

There is also chronic form diseases. Such a development of pathology is possible with various negative processes. In almost all cases, namely in 80%, the cause of Addison-Birmer disease is an autoimmune condition of the body. In 1 out of 10 cases, the cause of the pathology is the defeat of the adrenal cortex by an infectious disease, for example, tuberculosis.

For the remaining 10% of patients, the causes may be of a different nature:

• this may be affected long-term use drugs, in particular, glucocorticoids;
• types of fungal infection;
• injury to the endocrine glands;
• amyloidosis;
• tumors of both benign and malignant nature;
• bacterial infections in weakened immune system person;
• pituitary dysfunction;
• genetic predisposition to the disease.

Other syndromes can also develop due to Addison's disease, such as an adrenal crisis, which occurs when the concentration of adrenal hormones is too low.

There are the most likely causes of the crisis:

• severe stressful condition;
• violations in the dosage when compiling a course of treatment with hormonal drugs;
• can aggravate the disease infection cortical substance of the adrenal glands;
• trauma to the adrenal glands;
• circulatory disorders, such as blood clots.

Symptoms of the disease

Symptoms of Addison's disease directly depend on the violation of the synthesis of certain types of hormones. Clinical manifestations of the disease may be different. The determining factors are the form of pathology and its duration.

The most common clinical manifestations of pathology are as follows:

• Addisonian pathology has its own name of bronze disease for a reason. Most clear sign This disease is a violation of pigmentation. The skin changes its color. The color of mucous membranes changes. It's all about too much pigmentation. With a lack of adrenal hormones, much more ACTH is produced, this is explained by the need to stimulate the work of the endocrine glands.
• One of the common clinical manifestations disease is chronic hypotension. This can lead to dizziness and fainting, increased sensitivity to low temperatures.
• With insufficient work of the endocrine glands, the whole organism weakens. If you have constant fatigue, fatigue, you should consult a medical specialist.
• With this pathology, there are often disturbances in the work of the gastrointestinal tract, this can manifest itself in the form of vomiting, constant nausea and diarrhea.

• The disease can affect the emotional component. depressive state is one of the clinical manifestations of Addison's disease.
• Patients noted in themselves hypersensitivity to irritants. The sense of smell, hearing is aggravated, the person feels the taste of food better. In most cases, patients prefer to eat salty foods.
• Pain in muscle tissues can also be a symptom of Addison's disease. This is explained by an increase in the concentration of potassium in blood vessels.
• As mentioned above, one of the clinical manifestations of the disease is an adrenal crisis, which occurs as a result of a sharp decrease in the level of hormones of the endocrine glands. The most popular symptoms of a crisis are pain in the abdomen, low arterial pressure, disturbed salt balance.

Diagnosis of the disease

First of all, patients pay attention to changes in the shade of the skin. This phenomenon signals an insufficient activity of adrenal hormones. When referring to a medical specialist in this situation, he determines the ability of the adrenal glands to increase the synthesis of hormones.

Diagnosis of Addison's disease occurs by the introduction of ACTH and measurements of the content of cortisol in the blood vessels before the administration of the drug and 30 minutes after vaccination. If the potential patient does not have problems with the functions of the adrenal glands, the level of cortisol will increase. If the concentration of the test substance has not changed, the person has disturbances in the functioning of the endocrine glands. In some cases, for more accurate diagnosis, measure the content of the hormone in urea.

Treatment of pathology

In the course of treatment Special attention should be given to the diet. It should be varied, it should contain the necessary amount of proteins, fats and carbohydrates to provide the body. Particular attention should be paid to vitamins B and C. They can be found in bran, wheat, fruits and vegetables. In addition, the patient is advised to drink more decoctions based on wild rose or black currant.

With Addison's disease, the sodium content in the body decreases, for this reason it is recommended to focus on salty foods. In addition, the pathology is characterized by an increased concentration of potassium in the blood vessels, it is recommended not to include foods that are rich in potassium in the diet. These include potatoes and nuts. Patients are advised to eat as often as possible. Before going to bed, medical experts recommend having dinner, this will reduce the chance of hypoglycemia in the morning.

Almost all folk recipes are aimed at stimulating the adrenal cortex. ethnoscience has a mild effect, side effects are practically absent. Application folk recipes will not only improve the functioning of the adrenal glands, but also have a positive effect on the state of the whole organism as a whole. Using this approach, you can normalize the work of the gastrointestinal tract, counteract inflammatory processes chronic nature. It is recommended to use several recipes in turn, this will avoid the addiction of the body.

Prevention and forecasting

If therapy was started in a timely manner and all the recommendations of a medical specialist were followed, the outcome of the disease will be favorable. The disease will not affect life expectancy in any way. In some cases Addison's disease accompanied by a complication - an adrenal crisis. In such a situation, you should immediately seek the advice of a medical specialist. The crisis may lead to lethal outcome. Addison's disease is accompanied by rapid fatigue, weight loss and loss of appetite.

Changes in the shade of the skin do not occur in all cases, deterioration in the work of the endocrine glands occurs gradually, so it is difficult for a person to detect this on their own. In such a situation, a critical condition develops abruptly and unexpectedly for the patient. Most often, the reason for some negative factor such as stress, infection or injury.

Since Addison's disease is often autoimmune in nature, there are practically no preventive measures. You should monitor your immune system, avoid eating alcoholic beverages, smoking. Medical experts recommend timely attention to the manifestations of infectious diseases, especially tuberculosis.

There are several types of pathologies associated with a lack of trace elements in the body. One of them is Addison Birmer's anemia. This is a malignant course of the disease, expressed as anemia in vitamin B12 deficiency and folic acid. The disease, which occurs in 30-50 cases per 10,000 of the population, is more susceptible to this ailment of women, and, at the age of over 50, the risk of developing the disease increases (perhaps this is due to menopause).

Classification

For the first time, Addison Birmer's anemia develops with vitamin B12 deficiency, it was described in 1855 by Addison, later confirmed by Birmer, who studied the disease and gave a detailed clinical description. Subsequently, this condition was named after the names of its researchers. For a long time it was considered an incurable disease, proceeding hard and uncontrollably. Currently, the pathogenesis of the disease is quite clear, but the etiology to a greater extent remains only an assumption.

Addison-Birmer anemia is characterized by the presence of a specific triad of disorders in the body:

• Severe course of gastritis by atrophic type. There is a gradual decline in function glandular epithelium, the mucous membrane is infiltrated, replaced by cells unusual for this organ, production is sharply reduced or stops altogether.
• Inability to digest vitamin B12 and folic acid. Both components are necessary for building cells, with their help DNA is synthesized and the cell nucleus is formed correctly. With a lack of both of them, first of all, hematopoiesis and nervous tissue suffer.
• Development of megaloblastic hematopoiesis. This is the formation of a multitude that are not able to perform their functions normally. In this course, Addison-Birmer anemia is similar to malignant.

Causes

The main factor leading to the development of the disease, the cause of Addison Birmer's anemia, is atrophy of the gastric mucosa, as a result, the secretion (production) of pepsinogen stops. And the role of pepsinogen in the body is such that it provides transport and absorption of cyanocobalamin. However, atrophic gastritis does not always lead to the development of megablast anemia. Probably, several factors must coincide for the development of the disease.

The absorption of B12 and folic acid may be impaired due to extensive inflammation of the gastric mucosa and ileal calving or damage to their malignant tumors.

pernicious anemia to a large extent, is autoimmune disease, so in the blood serum of patients in 70 -75% of cases, antibodies to the internal cells of the stomach were detected. When conducting experiments on rats, it turned out that such cells lead to atrophy of the gastric glandular tissue. Similar antibodies are also present in gastric secretions. An autoimmune response is highly likely to be considered hereditary factor, since a certain amount of antibodies against parietal cells of the stomach, as well as cells endocrine system found in healthy relatives.

Additional but no less an important factor is a hereditary tendency to diseases of the pancreas.

Lifestyles leading to malfunctions in the immune system, for example, experimenting with hard foods, abrupt transition to vegetarianism, uncontrolled intake medical preparations, violation of dosages in taking medications. What is important, in the post-war famine years, the incidence of pernicious anemia did not increase, which means that quantitative and qualitative malnutrition can only be attributed to concomitant causes.

There are scattered facts on the description of cases of incidence of megaloblastic anemia. For example: in the northern regions, this pathology is more common; there are indications of an increase in morbidity in people whose work is associated with lead and the possibility slow poisoning carbon monoxide; after surgery to remove the stomach, when there is a total elimination of the secretory function, anemia can develop after 5-7 years; there is evidence of the development of megablast anemia as a result of toxic poisoning with chronic alcoholism.

Symptoms

Symptoms of Addison-Birmer anemia are as follows, erythrocyte cells of abnormal size are formed, with overgrown cytoplasm, and their nuclei contain small inclusions.

Due to a critical lack of vitamin B12, a defect occurs in the metabolism of folic acid, which is involved in DNA synthesis. As a result, cell division is also disrupted in the periphery. The same quantitative and qualitative deformations occur with platelets. The bone marrow changes color, acquires a rich scarlet color, it is dominated by megablast immature cells, which, by the type of development, resemble the malignant course of blood diseases.

Vitamin B12 is used by the body not only for hematopoiesis, but also to provide normal operation nervous system. With its deficiency, dystrophy is observed in nerve endings spinal column.

From the side digestive system atrophy of the mucous membrane of the palate, pharynx, esophagus, stomach and intestines is detected. Perhaps the formation of polyps, a slight increase in the liver. The clinical course of the disease of Addison-Birmer anemia manifests itself gradually: periodically occurs great weakness, attacks of dizziness intensify, there is noise in the ears.

The resulting symptoms of Addison Birmer's anemia can be divided into manifestations:

• from the nervous system: the regulation of movements is disturbed, paresthesia appears; there are pains in the intercostal space; sometimes there is damage to the visual and auditory nerve; .
• from the side digestive tract: glossitis, characterized by the syndrome of "varnished tongue" and painful sensations in the tongue; nausea, heaviness in the epigastric region, the formation of aversion to food, worsening taste sensations; an increase in the liver, less often - the spleen, the appearance of icterus of the sclera, mucous membranes;
• external manifestations: pale skin with a yellowish tint, a characteristic Addison-Birmer anemia syndrome is formed - the face of a wax doll; puffiness of the face, significant swelling; lethargy, drowsiness.
• from the side of the heart: the appearance, pain, dystrophic changes in the myocardium.

Diagnostics

Diagnosis of anemia consists of several stages.

Visual examination reveals: pale skin, icteric tint of the sclera, dark spots on the face, hands and body. A characteristic picture is given by inspection oral cavity, on initial stage disease, the tongue is painful, covered with small cracks. In the midst of the disease, the tongue becomes scarlet and puffy, looks like varnished. When a slight increase in the liver is detected and its protrusion beyond the edge of the rib. The spleen enlarges in a small number of patients. Conducting neurological tests reveals a change in sensitivity in the limbs.

When diagnosing Addison-Birmer anemia, a blood test plays a key role. A thorough study of peripheral blood is carried out, where a significant increase in erythrocyte blood volume is detected, while the number of reticulocytes is sharply reduced. The presence of hyperchromic erythrocytes is detected. The main sign of megablast anemia can be considered the presence of hypersegmental neutrophils (having five or more segments in the nucleus). In a relatively healthy person, such cells are found within 2%, in those suffering pernicious anemia, the number of hypersegmental neutrophils rises above 5%.

Equally important is research bone marrow with anemia. It reveals megaloblastic growth of cells - these are cells that have stopped in their development, preceding erythrocytes. They are abnormally enlarged, deformed, with a noticeable difference in the level of development of the nucleus and cytoplasm. In general, unproductive erythropoiesis - characteristic megablast anemia. The vast majority of immature and deformed erythrocyte cells (megablasts) are destroyed even in the bone marrow without entering the bloodstream. With the progression of the disease, the number of reticulocytes continues to fall, while their deformation occurs.

Instrumental diagnostics includes: a study of gastric juice, where, as a rule, a decrease in acidity or its complete absence is revealed. But there is a significant amount of mucus, similar in composition to the intestinal. An endoscopic examination is carried out, where extensive atrophy of the gastric mucosa, often called "pearl plaques", loss of secretory cells is clearly observed. Unfortunately, even during remission, pepsinogen synthesis is not restored.

often carried out histological examination tissues, since one of the causes that can cause Addison Birmer's anemia is malignant neoplasms.

These patients require further consultation. narrow specialists: neurologist, endocrinologist, cardiologist, immunologist.

A mandatory diagnostic method is the Schilling test. This method aims to differentiate folate deficiency anemia from B12-deficiency anemia in order to identify the root cause, and outline proper treatment. To do this, measure their concentration in blood serum. The norm of folic acid is 5-20ng / ml, the norm of B12 is 150-900ng / ml. Indicators below these limits indicate the presence of a deficiency of these components in the body. To conduct a test, the patient is injected with vitamin B12 intramuscularly, after a sufficient time, its concentration in the urine is determined, a small amount for B12-deficiency anemia, the maximum - for folic deficiency.

Folic acid deficiency in the body is more common in young age and does not have concomitant factors atrophy of the secretory function of the stomach and the presence neurological symptoms. It responds favorably to oral folic acid and responds better to treatment. When examining patients with B12 deficiency anemia, it is important to establish the underlying cause of the disease.

Treatment

Treatment of anemia Addison Birmer has its own characteristics. Choice medicinal product depends on the cause of the disease. Folic deficiency anemia occurs due to a violation of its absorption in the intestine. common cause This is chronic alcoholism, and it is especially dangerous during pregnancy.

The lack of folic acid is more favorable for the body in terms of treatment, since it does not affect the secretory function of the stomach, and oral administration therapeutic doses the drug gives a quick effect.

Folic acid is available as separate drug in the form of tablets or solution for injection, and as part of complex vitamins. Side effects when taking folic acid in the treatment of anemia are rare, but possible allergic reactions on intramuscular injection drug.

In the case of vitamin B12 deficiency in the body, the use of tablet forms is not justified, since the presence of atrophic gastritis reduces similar treatment to zero. In the treatment of this type of anemia, intramuscular or subcutaneous injections cyanocobalamin. Intravenous administration drug is dangerous. Cyanocobalamin is a pinkish liquid, in 1 ml ampoules, sometimes its use can cause allergic rash. The drug is administered daily at a dose of up to 500 mg for 6 weeks, folic acid is additionally administered at a dose of up to 100 mg.

With mild or medium degree severity of anemia, treatment may be delayed until after complete diagnosis and identifying the causes of shortages. With severe neurological disorders and significant changes in the blood picture, treatment is started immediately.

Often, external signs anemia of Addison-Birmer disappear in the first days of treatment. Pain in the tongue and mouth subsides, appetite appears, weakness disappears, vision and hearing are restored. After a few days, reticulocytosis is restored, the number of megablasts in the bone marrow is sharply reduced. Recovery of hematopoiesis usually occurs after 1-2 months. Heavy neurological disorders may retain their symptoms for several months, or may not completely disappear at all.

With severe atrophy of the gastric mucosa and with B12-deficiency anemia, preparations containing vitamin B12 have to be taken throughout life. The patient should be aware that the failure of maintenance therapy entails a relapse of megaloblastic anemia. As a rule, those who have been ill with Addison-Birmer anemia are registered in the dispensary and are under regular observation. Maintenance doses are administered in short courses as prescribed by a doctor and under constant monitoring of the blood picture.

Preparations for prevention

In order to prevent anemia, folic acid is prescribed only to certain groups of citizens. For example, pregnant women to prevent pathologies spinal cord in the fetus, nursing mother, for proper development child. Elderly people with some forms of anemia, as well as patients who are in a coma. In all other cases, the amount that comes with food in the normal diet is sufficient.

Vitamin B12 for prevention purposes is prescribed only in case of its possible deficiency, for example, with strict vegetarianism or total removal of the stomach. Cyanocobalamin has a wide reputation as a general tonic, which is completely unproven, but it is often prescribed for general exhaustion, fatigue and increased fatigue as a tonic. Given the need for B vitamins in the regulation of the nervous system, it is possible to use cyanocobolamin to treat inflammation. trigeminal nerve and other neuropathies.

Treatment of Addison Birmer's anemia with any vitamin preparations must be strictly targeted. And only if there is a suspicion of a deficiency of several vitamin components You can take multivitamin complexes.

• Symptoms of anemia (Addison-Birmer's disease)

Symptoms of anemia (Addison-Birmer's disease)

Clinic

Addison-Birmer anemia most often affects women aged 50-60 years. The disease begins slowly and gradually. Patients complain of weakness, fatigue, dizziness, headache, palpitations and shortness of breath on movement. In some patients in clinical picture dyspeptic symptoms dominate (belching, nausea, burning at the tip of the tongue, diarrhea), less often disorders of the nervous system (paresthesia, cold extremities, unsteady gait).

Objectively, pale skin (with a lemon tint), slight yellowness of the sclera, puffiness of the face, sometimes swelling of the legs and feet, and almost naturally - pain in the sternum when tapped.

The nutrition of patients was preserved due to a decrease in fat metabolism. The temperature, usually subfebrile, rises to 38-39°C during a relapse.

Characterized by changes in the digestive system. The edges and tip of the tongue are usually bright red with the presence of cracks and aphthous changes (glossitis). Later, the papillae of the tongue atrophy, in connection with which it becomes smooth, "varnished". Due to atrophy of the gastric mucosa, achilia develops and, in connection with it, dyspeptic phenomena (less often diarrhea). In half of the patients there is an increase in the liver, and in the fifth part - an increase in the spleen.

Changes in the cardiovascular system are manifested by tachycardia, hypotension, enlargement of the heart, deafness of tones, systolic murmur over the apex and over pulmonary artery, "the noise of the top" over the jugular veins, and in severe cases- circulatory failure. As a result of dystrophic changes in the myocardium, the ECG determines the low voltage of the teeth and the lengthening of the ventricular complex; T waves in all leads decrease or become negative.

Changes in the nervous system occur in approximately 50% of cases and are characterized by damage to the posterior and lateral columns of the spinal cord (funicular myelosis), manifested by paresthesia, decreased tendon reflexes, violation of deep and pain sensitivity, and in severe cases - paraplegia and dysfunction of the pelvic organs.

On the part of the blood - a high color index (up to 1.2-1.3). This is due to the fact that the number of erythrocytes decreases to a greater extent than the hemoglobin content. A qualitative analysis of a blood smear reveals pronounced macroanisocytosis with the presence of megalocytes and even single megaloblasts, as well as a sharp poikilocytosis. Often there are erythrocytes with the remains of nuclei - in the form of Cabot rings and Jolly bodies. On the part of white blood - leukopenia with hypersegmentation of neutrophil nuclei (up to 6-8 segments instead of 3). Permanent sign Birmer's anemia is also thrombocytopenia. The amount of bilirubin in the blood is usually increased due to increased hemolysis of megaloblasts and megalocytes, the osmotic resistance of which is reduced.

ANEMIA-B12-DEFICIENCY (ANEMIA ADDison - BIRMER)- formation of megaloblasts in the bone marrow, intra-bone marrow destruction of erythrocytes. changes in the nervous system in the form of funicular myelosis.

Etiology and pathogenesis

One of highlights The biological action of vitamin B12 is the activation of folic acid, vitamin B12 promotes the formation of folate derivatives of folic acid, which are directly necessary for bone marrow hematopoiesis. With a deficiency of vitamin B12 and folate, DNA synthesis is disrupted, which in turn leads to a violation of cell division, an increase in their size and qualitative inferiority. The cells of the erythroblastic germ are most significantly affected: instead of erythroblasts, large cells of embryonic hematopoiesis, megaloblasts, are found in the bone marrow, they are not able to “mature” to a full-fledged erythrocyte, that is, they cannot carry hemoglobin and oxygen. Average term the life of megalocytes is approximately 3 times less than that of "normal" erythrocytes. With a lack of the second coenzyme of vitamin B12 - an internal factor - there is another mechanism for the development of anemia - there is a violation of fat metabolism with the accumulation of methylmalonic acid, which is toxic to the nervous system. As a result, funicular myelosis occurs - a violation of hematopoiesis in the bone marrow and the development of anemia. B12 deficiency anemia also develops as a result of malabsorption of vitamin B gastrointestinal tract due to atrophic gastritis or as a result of congenital insufficiency of the glandular apparatus of the stomach, while in gastric juice there is no gastromucoprotein, which is directly involved in the breakdown and absorption of B12 and its coenzymes.

Clinic

The disease begins imperceptibly, weakness gradually progresses, palpitations, dizziness and shortness of breath appear, especially with physical activity, sudden movements, working capacity decreases, appetite worsens, nausea is possible. Often the first complaint with which patients go to the doctor is a burning sensation of the tongue, its cause is characteristic of this disease atrophic glossitis. As a result of dystrophic changes in the nervous system, skin anesthesia and paresthesia occur, in severe cases, gait disturbance (spastic paraparesis) is often noted, and functional disorders may occur. Bladder and rectum, sleep is disturbed, emotional instability, depression appear. When examining the patient, pay attention to pallor skin and mucous membranes (usually with a yellowish tint due to increased disintegration of megalocytes and the formation of bilirubin from released hemoglobin), puffiness of the face; a bright red, shiny, smooth tongue (due to severe atrophy of the papillae) is very characteristic - a “polished” tongue. Atrophic gastritis is very characteristic. Often, when tapping on flat and some tubular bones, pain is noted - a sign of bone marrow hyperplasia. Common symptom B12 deficiency anemia is subfebrile temperature.

Diagnostics

In the peripheral blood, a sharp decrease in the number of erythrocytes is determined (up to 0.8 X 1012), the color index remains high - 1.2-1.5. Red blood cells are not the same in size (anisocytosis), large erythrocytes - macrocytes predominate, many erythrocytes have a shape in the form of an oval, racket, crescent and other (poikilocytosis).

In the bone marrow punctate, the number of cells of the red germ is sharply increased, 3-4 times more than the cells of the leukocyte germ (normal - the opposite ratio). In the blood plasma, there is an increase in the content of free bilirubin and iron (up to 30-45 mmol / l).

Treatment

Vitamin B12 is prescribed. Treatment begins with the introduction of 100-300 micrograms of vitamin subcutaneously or intramuscularly 1 time per day. On the 2nd-3rd day of therapy, erythropoiesis is completely normalized, and on the 5th-6th day, newly formed full-fledged erythrocytes begin to enter the bloodstream in the required amount, and the patients feel gradually normal. After the restoration of the blood picture, they switch to maintenance therapy - the introduction of vitamin B12 at a dose of 50-100 μg, which is carried out throughout the patient's life. In case of disorders of the nervous system, at the first stage, apply neurotropic drugs.

Forecast

At adequate therapy favorable. Without treatment, the disease progresses and can lead to the death of the patient.

- a violation of the red germ of hematopoiesis, due to a lack of cyanocobalamin (vitamin B12) in the body. With B12-deficiency anemia, circulatory-hypoxic (pallor, tachycardia, shortness of breath), gastroenterological (glossitis, stomatitis, hepatomegaly, gastroenterocolitis) and neurological syndromes(disturbance of sensitivity, polyneuritis, ataxia). Pernicious anemia is confirmed by results laboratory research(clinical and biochemical analysis blood, bone marrow punctate). Treatment for pernicious anemia includes balanced diet, intramuscular injection of cyanocobalamin.

ICD-10

D51.0 Vitamin B12 deficiency anemia due to intrinsic factor deficiency

General information

Pernicious anemia is a type of megaloblastic deficiency anemia that develops with insufficient endogenous intake or absorption of vitamin B12 in the body. "Pernicious" in Latin means "dangerous, disastrous"; in the domestic tradition, such anemia used to be called "malignant anemia". In modern hematology, pernicious anemia is also synonymous with B12-deficiency anemia, Addison-Birmer disease. The disease occurs more often in people older than 40-50 years, somewhat more often in women. The prevalence of pernicious anemia is 1%; however, about 10% of older people over the age of 70 suffer from vitamin B12 deficiency.

Causes of pernicious anemia

The daily human need for vitamin B12 is 1-5 micrograms. It is satisfied by the intake of vitamin with food (meat, fermented milk products). In the stomach, under the action of enzymes, vitamin B12 is separated from dietary protein, but for absorption and absorption into the blood, it must combine with a glycoprotein (Castle factor) or other binding factors. The absorption of cyanocobalamin into the bloodstream occurs in the middle and lower parts of the ileum. The subsequent transport of vitamin B12 to tissues and hematopoietic cells is carried out by blood plasma proteins - transcobalamins 1, 2, 3.

The development of B12-deficiency anemia can be associated with two groups of factors: alimentary and endogenous. Nutritional causes are due to insufficient intake of vitamin B12 with food. This can occur with fasting, vegetarianism, and diets that exclude animal protein.

Under endogenous causes is meant a violation of the absorption of cyanocobalamin due to a deficiency of the internal factor of Castle with its sufficient intake from the outside. Such a mechanism for the development of pernicious anemia occurs in atrophic gastritis, a condition after gastrectomy, the formation of antibodies to internal factor Castle or parietal cells of the stomach, congenital absence of the factor.

Violation of the absorption of cyanocobalamin in the intestine can be observed with enteritis, chronic pancreatitis, celiac disease, Crohn's disease, diverticula small intestine, tumors of the jejunum (carcinoma, lymphoma). Increased consumption of cyanocobalamin may be associated with helminthiases, in particular, diphyllobothriasis. There are genetic forms of pernicious anemia.

Vitamin B12 absorption is impaired in patients undergoing small bowel resection with gastrointestinal anastomosis. Pernicious anemia may be associated with chronic alcoholism, the use of certain medicines(colchicine, neomycin, oral contraceptives, etc.). Since the liver contains a sufficient reserve of cyanocobalamin (2.0-5.0 mg), pernicious anemia develops, as a rule, only 4-6 years after the violation of the intake or absorption of vitamin B12.

In conditions of vitamin B12 deficiency, there is a deficiency of its coenzyme forms - methylcobalamin (participates in the normal course of erythropoiesis processes) and 5-deoxyadenosylcobalamin (participates in metabolic processes occurring in the central nervous system and peripheral nervous system). A lack of methylcobalamin disrupts the synthesis essential amino acids And nucleic acids, which leads to a disorder in the formation and maturation of red blood cells (megaloblastic type of hematopoiesis). They take the form of megaloblasts and megalocytes, which do not perform an oxygen transport function and are rapidly destroyed. In this regard, the number of erythrocytes in the peripheral blood is significantly reduced, which leads to the development of anemic syndrome.

On the other hand, with a deficiency of the coenzyme 5-deoxyadenosylcobalamin, the metabolism fatty acids, resulting in the accumulation of toxic methylmalonic and propionic acids, which have a direct damaging effect on the neurons of the brain and spinal cord. In addition, myelin synthesis is disrupted, which is accompanied by degeneration of the myelin layer of nerve fibers - this is due to damage to the nervous system in pernicious anemia.

Symptoms of pernicious anemia

The severity of pernicious anemia is determined by the severity of circulatory-hypoxic (anemic), gastroenterological, neurological and hematological syndromes. Signs of an anemic syndrome are nonspecific and are a reflection of a violation of the oxygen transport function of erythrocytes. They are represented by weakness, decreased endurance, tachycardia and palpitations, dizziness and shortness of breath when moving, low-grade fever. On auscultation of the heart, a whirlpool or systolic (anemic) murmur may be heard. Outwardly, there is pallor of the skin with a subicteric shade, puffiness of the face. A long "experience" of pernicious anemia can lead to the development of myocardial dystrophy and heart failure.

Gastroenterological manifestations of B12 deficiency anemia are decreased appetite, stool instability, hepatomegaly ( fatty degeneration liver). The classic symptom found in pernicious anemia is a raspberry-colored “varnished” tongue. The phenomena of angular stomatitis and glossitis, burning and pain in language. During gastroscopy, atrophic changes in the gastric mucosa are detected, which are confirmed by endoscopic biopsy. Gastric secretion is sharply reduced.

Neurological manifestations of pernicious anemia are caused by damage to neurons and pathways. Patients indicate numbness and stiffness of the limbs, muscle weakness, gait disturbance. Possible incontinence of urine and feces, the occurrence of persistent paraparesis lower extremities. An examination by a neurologist reveals a violation of sensitivity (pain, tactile, vibration), increased tendon reflexes, symptoms of Romberg and Babinsky, signs of peripheral polyneuropathy and funicular myelosis. B12 deficiency anemia can develop mental disorders- insomnia, depression, psychosis, hallucinations, dementia.

Diagnosis of pernicious anemia

In addition to a hematologist, a gastroenterologist and a neurologist should be involved in the diagnosis of pernicious anemia. Vitamin B12 deficiency (less than 100 pg / ml at a rate of 160-950 pg / ml) is established during biochemical research blood; it is possible to detect antibodies to the parietal cells of the stomach and to the internal factor of the Castle. For general analysis blood typical pancytopenia (leukopenia, anemia, thrombocytopenia). Microscopy of a peripheral blood smear reveals megalocytes, Jolly and Cabot bodies. Examination of feces (coprogram, analysis for eggs of worms) can reveal steatorrhea, fragments or eggs of a wide tapeworm in diphyllobothriasis.

The Schilling test allows you to determine the malabsorption of cyanocobalamin (by urinary excretion of radioactively labeled vitamin B12 taken orally). Bone marrow puncture and myelogram results reflect an increase in the number of megaloblasts characteristic of pernicious anemia.

To determine the causes of impaired absorption of vitamin B12 in the gastrointestinal tract, EGD, radiography of the stomach,