Disease of the adrenal glands, unspecified microbial code 10. Chronic adrenal insufficiency in adults. Complications of chronic adrenal insufficiency
Adrenal insufficiency (synonym: hypocorticism) - a decrease in the glucocorticoid and mineralocorticoid function of the adrenal glands - one of the most severe diseases of the endocrine system.
There are acute and chronic adrenal insufficiency. There are primary hypocorticism due to direct damage to the adrenal cortex, and secondary hypocorticism associated with a pituitary or hypothalamic defect, accompanied by a deficiency of ACTH.
ICD-10 code
- E27.1 Primary adrenal insufficiency.
- E27.3 Drug insufficiency of the adrenal cortex.
- E27.4 Other and unspecified adrenal insufficiency.
Causes of chronic adrenal insufficiency
The main cause of primary hypocorticism is autoimmune destruction of the adrenal cortex. The appearance of autoantibodies to antigens of adrenal cells is often accompanied by the production of other organ-specific autoantibodies, which leads to the development of polyendocrine syndrome - a combination of chronic adrenal insufficiency with autoimmune thyroiditis, diabetes mellitus, hypoparathyroidism, pernicious anemia, vitiligo, candidiasis. Tuberculosis is another cause of primary adrenal damage, although tuberculous etiology is less common in children than in adults. Sometimes primary hypocorticism can be due to congenital adrenal hypoplasia, a genetically determined disease with an X-linked recessive inheritance pattern (found only in boys).
The most common cause of secondary hypocorticism is destructive processes in the hypothalamic-pituitary system (tumor, trauma, infection).
Due to the lack of glucocorticoids, which provide activation of gluconeogenesis in a healthy body, glycogen stores in the muscles and liver decrease, and the glucose content in the blood and tissues decreases. Reduced glucose uptake into tissues leads to adynamia and muscle weakness. Mineralocorticoid deficiency causes an increase in sodium, chloride, and water excretion, leading to hyponatremia, hyperkalemia, dehydration, and decreased blood pressure. Adrenal androgen deficiency that accompanies adrenal insufficiency is manifested by growth retardation and sexual development. This reduces the intensity of anabolic processes in bone and muscle tissues. Clinical signs of chronic adrenal insufficiency appear when 90% of glandular cells are destroyed.
Symptoms of chronic adrenal insufficiency
Symptoms of chronic adrenal insufficiency are primarily due to glucocorticoid deficiency. Congenital forms of hypocorticism appear from the first months of life. With autoimmune adrenalitis, the onset of the disease occurs more often after 6-7 years. Characterized by lack of appetite, weight loss, lowering blood pressure, asthenia. Abdominal pain, nausea, and unexplained vomiting are often noted.
hyperpigmentation skin- pathognomonic clinical symptom of primary hypocorticism. Natural folds of the skin, places of contact with clothing are intensively stained. Hyperpigmentation is caused by excessive secretion of ACTH and melanocyte-stimulating hormone. With secondary hypocorticism, hyperpigmentation is absent.
In some cases, hypoglycemic conditions develop due to the absence of the contra-insular action of glucocorticoids.
With congenital chronic adrenal insufficiency, signs of the disease appear soon after birth. Characterized by rapid weight loss, hypoglycemia, anorexia, regurgitation. Typical pigmentation of the nipples, white line of the abdomen, external genital organs against the background of pale skin.
Classification of chronic adrenal insufficiency
Complications of chronic adrenal insufficiency
Without treatment, the symptoms of chronic adrenal insufficiency rapidly increase, and an adrenal insufficiency crisis develops, characterized by symptoms of acute adrenal insufficiency. There is a sharp weakness, a drop in blood pressure, vomiting, loose stools, abdominal pain. Clonic-tonic convulsions and meningeal symptoms are possible. Growing symptoms of dehydration, cardiovascular insufficiency. With untimely or inadequate treatment, a fatal outcome is possible.
Diagnosis of chronic adrenal insufficiency
The main diagnostic criterion for hypocorticism is a decrease in the content of cortisol and aldosterone in the blood serum. In primary hypocorticism, low levels of cortisol and aldosterone are accompanied by an increase in plasma ACTH and renin levels.
Glucocorticoid deficiency leads to hypoglycemia. Mineralocorticoid deficiency is characterized by hyperkalemia and hyponatremia.
With erased forms of chronic adrenal insufficiency, a stimulation test with ACTH is performed: after blood sampling to determine the basal level of cortisol at 8 am, an ACTH drug of prolonged action tetracosactides or fast-acting syncorpine, corticotropin is administered. Then the level of cortisol is re-determined 12-24 hours after the administration of tetracosactide or 60 minutes after corticotropin. In healthy children, the content of cortisol after the introduction of ACTH is 4-6 times higher than the basal level. The lack of response to stimulation indicates a decrease in the reserves of the adrenal cortex.
Differential Diagnosis
Differential diagnosis of chronic adrenal insufficiency should be carried out with neurocirculatory dystonia of the hypotonic type, essential arterial hypotension. The combination of arterial hypotension with weight loss can be with gastric ulcer, anorexia nervosa, oncological pathology. In the presence of hyperpigmentation, differential diagnosis is carried out with dermatomyositis, scleroderma, pigment-papillary dystrophy of the skin, poisoning with salts of heavy metals.
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MINISTRY OF HEALTH AND SOCIAL DEVELOPMENT
RUSSIAN FEDERATION
On approval of the standard of medical care for patients
chronic adrenal insufficiency
In accordance with clause 5.2.11 of the Regulations on the Ministry of Health and Social Development of the Russian Federation, approved by Decree of the Government of the Russian Federation of June 30, 2004 N 321 (Collection of Legislation Russian Federation, 2004, N 28, Art. 2898), Art. 38 Fundamentals of the legislation of the Russian Federation on the protection of the health of citizens dated 07.22.93 N 5487-1 (Bulletin of the Congress of People's Deputies of the Russian Federation and the Supreme Council of the Russian Federation, 1993, N 33, Art. 1318; Collection of acts of the President of the Russian Federation and the Government of the Russian Federation, 1993, N 52, art. 5086; Collection of legislation of the Russian Federation, 1998, N 10, art. 1143; 1999, N 51, art. .4740; 2003, N 2, Art. 167; N 9, Art. 805; N 27 (part 1), Art. 2700; 2004, N 27, Art. 2711)
I order:
1. Approve the standard of care for patients with chronic adrenal insufficiency (Appendix).
Deputy Minister
V. Starodubov
Application. Standard of care for patients with chronic adrenal insufficiency
Application
to the order of the Ministry
health and
social development
Russian Federation
dated February 28, 2005 N 168
1. Patient model
Age category: Children Adults
Nosological form: chronic adrenal insufficiency
ICD-10 code: E27.1-27.4
Phase: chronic
Stage: all stages
Complication: all complications
Rendering condition: outpatient care
1.1. Diagnostics
Name | Delivery frequency | Average quantity |
|
Palpation in endocrinology | |||
Body weight measurement | |||
Height measurement | |||
Drawing up a pedigree | |||
Taking blood from a finger | |||
Research level 17- | |||
Examination of the level of adrenocorticotropic hormone in the blood | |||
The study of the level of follicle-stimulating hormone in the blood | |||
The study of the level of luteinizing hormone in the blood | |||
The study of the level of total estradiol in the blood | |||
The study of testosterone levels in the blood | |||
Urinary free cortisol testing | |||
Ultrasound examination of the adrenal glands | |||
Appointment (examination), consultation of a neurologist primary | |||
Reception (examination), consultation of a geneticist | |||
The study of the level of free thyroxine in serum (T-4) blood | |||
The study of the level of thyroid-stimulating hormone in the blood | |||
The study of the level of renin in the blood | |||
Computed tomography of the head with contrasting brain structures | |||
X-ray of the hand | |||
Reception (examination, consultation) of a phthisiatrician |
2. 1. Treatment at the rate of 12 months
Name | Delivery frequency | Average quantity |
|
Collection of anamnesis and complaints in endocrinology | |||
Visual examination in endocrinology | |||
Palpation in endocrinology | |||
Body weight measurement | |||
Height measurement | |||
Measurement of blood pressure in peripheral arteries | |||
Heart rate measurement | |||
Visual examination in gynecology | |||
Palpation in gynecology | |||
Ultrasound examination of the uterus and appendages | |||
Ultrasound examination of the scrotum (testicles, appendages) | |||
Taking blood from a peripheral vein | |||
Study of the level of potassium in the blood | |||
Study of the level of sodium in the blood | |||
Blood cortisol test | |||
Study of the level of renin activity in the blood | |||
Computed tomography of the head | |||
Nuclear magnetic resonance imaging of the central nervous system and brain | |||
Self care training | |||
X-ray of the hand | |||
Prescribing drug therapy for diseases of the endocrine glands |
Pharmacote- | ATH* group | International non-proprietary name | Assignment Frequency | |||
________________ |
||||||
Hormones and drugs that affect the endocrine system | ||||||
Non-sex hormones, synthetic substances and antihormones | ||||||
Hydrocortisone (tablets) | ||||||
Hydrocortisone (solution) | in crisis* 100 mg | in a crisis |
||||
________________ |
||||||
Prednisolone | ||||||
Dexamethasone | ||||||
fludrocortisone | ||||||
Adrenal insufficiency (hypocorticism) - a decrease in the glucocorticoid and mineralocorticoid function of the adrenal glands - is one of the most severe diseases of the endocrine system.
There are acute and chronic adrenal insufficiency.
There are primary hypocorticism due to direct damage to the adrenal cortex, and secondary hypocorticism associated with a pituitary or hypothalamic defect, accompanied by a deficiency of ACTH.
Codes according to ICD-10 E27.1. Primary adrenal insufficiency. E27.3. Drug insufficiency of the adrenal cortex. E27.4. Other and unspecified insufficiency of the adrenal cortex.
Chronic adrenal insufficiency Classification Primary insufficiency of the adrenal cortex (Addison's disease). Congenital. Congenital hypoplasia of the adrenal cortex. Hypoaldosteronism. Adrenoleukodystrophy. Familial isolated GC deficiency. Allgrove syndrome. Acquired. Autoimmune adrenalitis. Infectious adrenalitis (tuberculosis, syphilis, mycoses). Amyloidosis. Metastases of malignant tumors. Secondary insufficiency of the adrenal cortex. Congenital.
Chapter 48 Diseases of the adrenal glands 673 Isolated insufficiency of corticotropin. Hypopituitarism. Acquired. Destructive damage to the pituitary gland (tumors, hemorrhage, infections, autoimmune hypophysitis). Tertiary insufficiency of the adrenal cortex. Congenital. Isolated deficiency of corticoliberin. Multiple insufficiency of the hypothalamus. Acquired. Destructive damage to the hypothalamus. Violation of the reception of steroid hormones. Pseudohypoaldosteronism. Iatrogenic insufficiency of the adrenal cortex.
Etiology and pathogenesis
The main cause of primary hypocorticism is autoimmune destruction of the adrenal cortex. Another cause of the primary lesion of the adrenal glands is tuberculosis. Sometimes primary hypocorticism can be due to congenital adrenal hypoplasia, a genetically determined disease with an X-linked recessive inheritance pattern (found only in boys).
The most common cause of secondary hypocorticism is destructive processes in the hypothalamic-pituitary system (tumor, trauma, infection).
Due to the lack of GCs, which provide activation of gluconeogenesis in a healthy body, glycogen stores in the muscles and liver decrease, and the glucose content in the blood and tissues decreases. Reduced glucose uptake into tissues leads to adynamia and muscle weakness. The lack of mineralocorticoids causes an increase in the excretion of sodium, chlorides and water, which leads to hyponatremia, hyperkalemia, dehydration and a decrease in blood pressure. Adrenal androgen deficiency that accompanies adrenal insufficiency is manifested by growth retardation and sexual development. This reduces the intensity of anabolic processes in bone and muscle tissues.
Clinical signs of chronic adrenal insufficiency appear when 90% of glandular cells are destroyed.
Clinical picture
Symptoms of the disease are primarily due to the lack of HA. Congenital forms of hypocorticism appear from the first months of life. With autoimmune adrenalitis, the onset of the disease occurs more often after 6-7 years. Lack of appetite, loss of body weight, decrease in blood pressure, asthenia are characteristic. Abdominal pain, nausea, and unexplained vomiting are often noted.
674 Chapter 48 Diseases of the Adrenal Gland
Hyperpigmentation of the skin is a pathognomonic clinical symptom of primary hypocorticism. Natural folds of the skin, places of contact with clothing are intensively stained. Hyperpigmentation is caused by excessive secretion of ACTH and melanocyte-stimulating hormone. With secondary hypocorticism, hyperpigmentation is absent.
Without treatment, the symptoms of the disease rapidly increase, and an adrenal insufficiency crisis develops, characterized by symptoms of acute adrenal insufficiency. There is a sharp weakness, a drop in blood pressure, vomiting, loose stools, abdominal pain. Clonic-tonic convulsions and meningeal symptoms are possible. Growing symptoms of dehydration, cardiovascular insufficiency.
Diagnostics
The main diagnostic criterion for hypocorticism is a decrease in the content of cortisol and aldosterone in the blood serum. In primary hypocorticism, low levels of cortisol and aldosterone are accompanied by an increase in plasma ACTH and renin levels.
GA deficiency leads to hypoglycemia. Mineralocorticoid deficiency is characterized by hyperkalemia and hyponatremia.
With erased forms of the disease, a stimulation test with ACTH is performed. In healthy children, the content of cortisol after the introduction of ACTH is 4-6 times higher than the basal level. The lack of response to stimulation indicates a decrease in the reserves of the adrenal cortex.
Differential Diagnosis
Differential diagnosis should be carried out with neurocirculatory dystonia of the hypotonic type, essential arterial hypotension. The combination of arterial hypotension with weight loss can be with gastric ulcer, anorexia nervosa, oncological pathology. In the presence of hyperpigmentation, differential diagnosis is carried out with dermatomyositis, scleroderma, pigment-papillary dystrophy of the skin, poisoning with salts of heavy metals.
Treatment
Medical treatment
Treatment of the crisis is aimed at eliminating electrolyte disturbances and hypoglycemia. Infusion therapy includes 0.9% sodium chloride solution and 5% glucose solution *. The total volume of liquid is calculated based on the physiological need, taking into account losses.
At the same time, parenteral hydrocortisone therapy is started, followed by transfer to a permanent substitute.
Chapter 48 Diseases of the Adrenal Gland 675
therapy with tablet preparations (cortef*, cortineff*). The adequacy of treatment is assessed by the parameters of physical and sexual development, blood pressure, electrolyte levels in blood plasma, ECG.
Forecast
With adequate replacement therapy, the prognosis for life is favorable.
Acute adrenal insufficiency
Acute adrenal insufficiency is an emergency condition that occurs as a result of a sharp decrease in the production of hormones by the adrenal cortex, clinically manifested by severe adynamia, vascular collapse, and gradual blackout of consciousness.
Etiology
Adrenal or Addisonian crises develop more often in patients with a primary lesion of the adrenal cortex. The development of acute adrenal insufficiency may be the first manifestation of chronic adrenal insufficiency. Acute adrenal insufficiency may be the result of thrombosis or embolism of the adrenal vessels (Waterhouse-Frideriksen syndrome). Hemorrhagic infarction in this syndrome occurs against the background of meningococcal, pneumococcal or streptococcal bacteremia. In newborns, the most common cause of adrenal apoplexy is birth trauma, and infectious-toxic factors are in second place.
Pathogenesis
In acute adrenal insufficiency, due to the lack of synthesis of gluco- and mineralocorticoids, there is a loss of sodium and chloride ions, a decrease in their absorption in the intestine, which leads to dehydration and a secondary transition of water from the extracellular space to the cell. In connection with a sharp dehydration, the BCC decreases and shock develops. The concentration of potassium in the blood serum, in the interstitial fluid and in the cells increases and leads to a violation of myocardial contractility.
In the absence of GC, hypoglycemia develops, glycogen stores in the liver and muscles decrease. A decrease in the filtration and reabsorption functions of the kidneys is characteristic.
With Waterhouse-Frideriksen syndrome, bacterial shock develops, leading to acute vascular spasm, necrosis and hemorrhage in the cortical and medulla of the adrenal glands. Lesions of the adrenal glands can be focal and diffuse, necrotic and hemorrhagic.
676 Chapter 48 Diseases of the Adrenal Gland
Clinical picture
It is characterized by a rapidly progressive decrease in blood pressure. Muffled heart sounds, pulse of weak filling, tachycardia, acrocyanosis, "marbling", congestive bluish-purple spots on the skin are noted. Decreased diuresis to anuria. Cramping abdominal pain, vomiting, diarrhea, weakness, muscle hypotension. In the terminal stage, convulsions and coma are possible.
The course of acute adrenal insufficiency can be from several hours to several days, depending on the etiology.
Diagnostics
Diagnosis is based on the clinical picture, since acute adrenal insufficiency develops extremely quickly. Determination of electrolytes in blood plasma is desirable: hyperkalemia and hyponatremia, hypochloremia are typical. For optimal infusion therapy, it is necessary to determine the hematocrit, acid-base state, the concentration of glucose and hemoglobin in the blood.
Differential Diagnosis
Differential diagnosis is carried out with coma of various origins, acute surgical diseases of the abdominal organs.
Treatment
Medical treatment
Treatment is carried out in the intensive care unit. To correct hypoglycemia and salt loss, sodium chloride 0.9% and 5% glucose solution are administered - for children under 1 year old in a ratio of 1: 1, for children older than a year - sodium chloride solution containing 5% glucose. At the same time, water-soluble hydrocortisone is injected intravenously at a dose of 10-15 mg/kg of body weight per day. You can simultaneously enter half daily dose, then distribute half the dose evenly throughout the day. At the same time, careful monitoring of blood pressure and the concentration of sodium ions in the blood plasma is necessary.
Primary adrenal insufficiency develops when 85-90% of the adrenal tissue is affected.
In 98% of cases, the cause of primary hypocorticism is idiopathic (autoimmune) atrophy of the adrenal cortex. At the same time, for unknown reasons, autoimmune antibodies to the 21-hydroxylase enzyme are formed in the body, destroying healthy tissues and cells of the adrenal glands. Also, 60% of patients with primary idiopathic form of adrenal insufficiency have autoimmune lesions of other organs, more often autoimmune thyroiditis. Tuberculosis of the adrenal glands occurs in 1-2% of patients and in most cases is combined with pulmonary tuberculosis.
A rare genetic disease - adrenoleukodystrophy causes primary adrenal insufficiency in 1-2% of cases. As a result of a genetic defect of the X chromosome, there is a lack of an enzyme that breaks down fatty acids. preferential accumulation fatty acids in the tissues of the nervous system and the adrenal cortex causes their degenerative changes.
Extremely rarely, coagulopathy, tumor metastases to the adrenal glands (usually from the lung or mammary gland), bilateral adrenal infarction, HIV-associated infections, and bilateral removal of the adrenal gland lead to the development of primary adrenal insufficiency.
Severe suppurative diseases, syphilis, fungal infections and amyloidosis of the adrenal glands, malignant tumors, heart defects, the use of certain drugs (anticoagulants, steroidgenesis blockers, ketoconazole, chloditan, spironolactone, barbiturates) predispose to the development of atrophy of the adrenal cortex.
Secondary adrenal insufficiency is caused by destructive or tumor processes in the hypothalamic-pituitary region, leading to impaired corticotropic function, as a result of:
tumors of the hypothalamus and pituitary gland: craniopharyngiomas, adenomas, etc.;
vascular diseases: hemorrhages in the hypothalamus or pituitary gland, aneurysm of the carotid artery;
granulomatous processes in the hypothalamus or pituitary gland: syphilis, sarcoidosis, granulomatous or autoimmune hypophysitis;
destructive traumatic interventions: radiation therapy of the hypothalamus and pituitary gland, surgery, long-term treatment with glucocorticoids.
Primary hypocorticism is accompanied by a decrease in the secretion of hormones of the adrenal cortex (cortisol and aldoatherone), which leads to metabolic disorders and the balance of water and salts in the body. With a deficiency of aldosterone, progressive dehydration develops due to sodium loss and potassium retention (hyperkalemia) in the body. Fluid and electrolyte disorders cause disorders of the digestive and cardiovascular systems.
A decrease in cortisol levels reduces glycogen synthesis, leading to the development of hypoglycemia. Under conditions of cortisol deficiency, the pituitary gland begins to increase the production of ACTH and melanocyte-stimulating hormone, which causes increased pigmentation of the skin and mucous membranes. Various physiological stresses (injuries, infections, decompensation of concomitant diseases) cause the progression of primary adrenal insufficiency.
Secondary hypocorticism is characterized only by a deficiency of cortisol (as a result of a lack of ACTH) and the preservation of aldosterone production. Therefore, secondary adrenal insufficiency, compared with primary, proceeds relatively easily.
Congenital dysfunction of the adrenal cortex (adrenogenital syndrome) includes a group of hereditary fermentopathies. At the heart of each of the fermentopathies is a genetically determined defect in the enzyme involved in steroidogenesis. Defects in five enzymes involved in the synthesis of glucocorticoids and mineralocorticoids have been described, with the formation of one or another variant of the disease. All forms of congenital adrenal dysfunction are inherited in an autosomal recessive manner.
E25.8. Other adrenogenital disorders.
E25.9. Adrenogenital disorder, unspecified.
ETIOLOGY AND PATHOGENESIS. In 90% of cases, mutations in the CYP21 gene encoding this enzyme are observed. With a partial deficiency of this enzyme, a simple (virile) form of the disease develops. 21-hydroxylase is involved in the synthesis of cortisol and aldosterone and is not involved in the synthesis of sex steroids. Violation of the synthesis of cortisol stimulates the production of ACTH, which leads to hyperplasia of the adrenal cortex. At the same time, 17-OH-progesterone, a precursor of cortisol, accumulates. Excess 17-OH-progesterone is converted to androgens. Adrenal androgens lead to virilization of the external genitalia in a female fetus - a girl is born with false female hermaphroditism. In boys, hyperandrogenemia determines the premature appearance of secondary sexual characteristics (precocious puberty syndrome - PPR).
With a significant deficiency of 21-hydroxylase, the hyperplastic adrenal cortex does not synthesize cortisol and aldosterone in the required quantities, while against the background of hyperandrogenemia, salt loss syndrome develops, or a salt-losing form of the disease.
The non-classical form of 21-hydroxylase deficiency manifests itself in pre- and pubertal age in the form of adrenarche, moderate hirsutism, and menstrual irregularities in girls.
CLINICAL PICTURE. In the classical viril form of the disease, the external genital organs of the girl are formed according to the heterosexual type - the clitoris is hypertrophied, the labia majora resemble the scrotum, the vagina and urethra are represented by the urogenital sinus. In newborn boys, no obvious abnormalities can be identified. From 2-4 years old, other symptoms of androgenization appear in children of both sexes: axillary and pubic hair develops, skeletal muscles develop, voice coarsens, figure masculinizes, juvenile acne appears on the face and torso. In girls, the mammary glands do not grow, menstruation does not appear. At the same time, the differentiation of the skeleton is accelerated, and the growth zones close prematurely, which leads to short stature.
In the salt-wasting form of 21-hydroxylase insufficiency, in addition to the symptoms described above, children from the first days of life have signs of adrenal insufficiency. Regurgitation first appears, then vomiting, loose stools are possible. The child rapidly loses body weight , dehydration symptoms develop , microcirculation disorders , blood pressure decreases , tachycardia begins , cardiac arrest due to hyperkalemia is possible .
The non-classical form of the disease is characterized by the early appearance of secondary hair growth, accelerated growth and differentiation of the skeleton. In girls of puberty, moderate signs of hirsutism, menstrual irregularities, and the formation of secondary polycystic ovaries are possible.
DIAGNOSTICS.
LABORATORY DIAGNOSIS. All children with an abnormal structure of the external genital organs, including boys with bilateral abdominal cryptorchidism, are shown to determine the sex chromatin and study the karyotype.
From the first days of life, an increased content of 17-OH-progesterone is found in the patient's blood serum. When conducting a screening test in newborns on the 2nd-5th days of life, 17-OH-progesterone was increased several times.
Salt loss syndrome is characterized by hyperkalemia, hyponatremia, and hypochloremia.
INSTRUMENTAL METHODS. The bone age according to the radiograph of the wrist joints is ahead of the passport one.
With ultrasound of the pelvic organs in girls, the uterus and ovaries are found.
DIFFERENTIAL DIAGNOSIS. In children of the first year of life, differential diagnosis is carried out with various forms of false male hermaphroditism and true hermaphroditism. The reference point in the diagnosis is karyotyping (karyotype 46XX with a bisexual structure of the external genital organs) and the determination of 17-OH-progesterone in the blood serum. The salt-wasting form of congenital adrenal dysfunction should be differentiated from pyloric stenosis, in which case hyperkalemia and a high level of 17-OH-progesterone are important in congenital adrenal dysfunction.
In older children with symptoms of hyperandrogenemia, androgen-producing adrenal tumors or androgen-producing tumors of the gonads should be remembered.
MEDICAL TREATMENT. The viril form requires constant replacement therapy with prednisone. The dose of the drug is selected individually and distributed evenly throughout the day.
Treatment of the salt-losing form in a crisis of adrenal insufficiency is carried out by drip administration of a solution of sodium chloride and glucose, as well as parenteral administration of hydrocortisone (10-15 mg / kg per day). The daily amount of hydrocortisone is distributed evenly. The drug of choice is water-soluble hydrocortisone (Solucortef). When the condition is stabilized, hydrocortisone injections are gradually replaced with hydrocortisone tablets, if necessary, a mineralocorticoid - fludrocortisone (2.5-10.0 μg / day) is added. Criteria for the effectiveness of treatment: normalization of the child's growth rate, normal performance blood pressure, electrolytes in blood serum. The optimal dose of GCS is determined by the level of 17-OH-progesterone in the blood serum, mineralocorticoids - by the content of renin in the blood plasma.
SURGERY. Girls at 4-6 years of age undergo surgical correction of the external genitalia.
FORECAST. The prognosis for life with timely diagnosis and proper treatment is favorable. It should be remembered about the risk of developing acute adrenal insufficiency in injuries, intercurrent diseases, stressful situations, and surgical interventions. To prevent crises of adrenal insufficiency, the dose of GCS should be increased by 3-5 times. In emergencies, timely parenteral administration of hydrocortisone is important.
