Endocrinologists in their practice often encounter pathologies in the work of the kidneys. Adrenal hyperplasia - what is it? The kidneys are a paired organ, just above the adrenal glands, which have a glandular shape. Hormones necessary for the human body are formed here (adrenaline, norepinephrine, glucocorticoids, as well as genital). Hyperplasia disrupts the proper production of hormones, and this is accompanied by various symptoms.
Adrenal hyperplasia - what is it?
With adrenal hyperplasia, the cellular tissue of the paired gland grows progressively, the organ significantly increases in size, although it retains its own shape. The adrenal gland consists of the medulla and the cortex.
Usually, hyperplasia affects the adrenal cortex, and a pathological process develops there. Various tumors are often diagnosed in the medulla.
The disease manifests itself at any age in women, men and children.
Congenital adrenal hyperplasia is most common in medicine. Usually it bears a hereditary character, but it can also occur due to a number of some unfavorable internal or external factors. As a concomitant pathology, it is detected in patients with Cushing's syndrome (in 40% of cases). This syndrome is usually diagnosed in the elderly and senile.
This serious pathology cannot be neglected. With hyperplasia, the production of necessary hormones is disrupted. In women, this often causes infertility. Several types of the disease are distinguished: nodular adrenal hyperplasia, nodular, diffuse, congenital and micronodular, as well as medial peduncle hyperplasia. More often this ailment is found in women.
Causes of the disease
The causes of symptoms with hyperplasia of the adrenal cortex in children, men or women will vary. Everything will depend on the type of the disease. Hyperplasia refers to one of the types, it can be hypertonic, salt-rubbing, viril.
With the hypertensive type, the active production of androgens and mineralocorticoids occurs, this process has a bad effect on the vessels of the kidneys and fundus, often leading to hypertension syndrome.
With the salt type of hyperplasia, androgen production is increased, while there is a shortage of other hormones produced by the adrenal cortex. There is hyperkalemia or hypoglycemia. The danger is that the body is dehydrated, weight is lost.
With the viril type of hyperplasia, the androgen secretion is super active. The external genitalia greatly increase in size, acne appears in large quantities, hair growth is increased, the muscles are too developed.
First of all, pathology begins to progress due to increased secretion of steroid hormones. The acquired form of the disease has a number of main reasons why it arose:
- metabolic disorder;
- severe stress;
- Cushing's disease;
Congenital adrenal hyperplasia is associated with the fact that even during pregnancy, during gestation, functional changes developed. Another assumption is the transmission of the disease at the genetic level. Factors provoking the manifestation of the symptoms of the disease: malfunction of the hypothalamus and pituitary gland, abnormalities at the genetic level, congenital autonomy of the cortex of the paired glands, inherited from the ancestors.
Nodular hyperplasia
Doctors divide the progression of adrenal hyperplasia into several groups according to the clinical picture, as well as the type of course.
Adrenal nodular hyperplasia. This form is hereditary. Most often diagnosed in old age, worsens over time. During the diagnosis, a characteristic sign is revealed - formed nodules on the adrenal cortex. They can be multiple (lobed structure) and single, up to 4-5 cm in size. Cushing's syndrome develops. Pathology occurs due to strong stimulation by adrenocorticotropic hormones synthesized in the pituitary gland. The main signs of nodular hyperplasia with Cushing's syndrome include:
- headache;
- "Flies" before the eyes;
- cramps
- dizziness;
- general malaise;
- weight gain (mainly stomach, neck, face);
- increased appetite;
- stretch marks on the skin;
- acne;
- excessive body hair (in women);
- poor regeneration;
- hyperpigmentation;
- high blood pressure;
- frequent fractures;
- depression.
Diffuse hyperplasia
With this form of hyperplasia, the glands do not change their shape, the increase occurs gradually. But at the same time, nodules form on them. It is quite difficult to identify this type of disease. With the standard diagnostic method for ultrasound, there is no result. Changes in the organ are detected only on MRI or CT. Mixed forms often develop that occur with the following symptoms: hyperplasia of the adrenal cortex in women often leads to increased hair growth, panic attacks, hypertension, and obesity are manifested. Some have a glomerular lesion and develop bilateral hyperplasia.
Pathology idiopathic hyperaldosteronism is based on increased secretion of aldosterone. With glomerular hyperplasia, the following symptoms appear: arterial hypertension, cramps, muscle weakness, restriction of movement, stiffness up to paralysis, thirst, increased urine output, nightly urination. Violations are caused by an increase in blood potassium, acidosis. Increased blood pressure is observed continuously.
Nodular and congenital forms
The nodular form usually progresses in children and adolescents. It can occur due to impaired adrenal function or due to an overdose of glucocorticoids. The main signs of the nodular form: uneven deposition of fat, muscle atrophy, skin pigmentation, signs of diabetes, damage to the nervous system and cardiovascular, osteoporosis. If the symptoms of the disease are detected in children on time, hormonal treatment is performed, then the prognosis is favorable. Hyperplasia of the left adrenal gland - what is it? In this case, hyperplasia is independent of the action of hormones. When the tumor grows more than 3 cm, surgery is required. Laparoscopy or the Fedorov method with lumbotomy access to the peritoneum on the left side is used.
Congenital hyperplasia of the adrenal cortex (VCD) is diagnosed more often than other types. In newborns, it is detected with a frequency of 1 to 12 thousand. In this condition, the child shows metabolic and appearance disorders. There are three main forms (non-classical, simple classical, viril and salt-in.
The most common is salt form. It manifests itself in girls as a false hermophroditism, in boys the skin around the scrotum darkens, the penis enlarges. Patients are characterized by short stature and symptoms such as drowsiness, nausea, lethargy, low blood pressure, dehydration, and poor appetite. The disease must be treated from the first days of manifestation, otherwise a fatal outcome is possible.
With the classical form, there are no symptoms described above, but there is an increase in the synthesis of male sex hormones. If the disease is not treated, then in the future, women will experience problems in the sexual and mental sphere.
The non-classical form manifests itself in adult women and adolescents. The main complaints of infertility, menstrual irregularities, hirsutism and acne.
Adrenal hyperplasia: common symptoms
Very often in children, men and women, an erased form of the disease is observed, when there is no pronounced symptomatology, in such cases it is difficult to diagnose the ailment. The congenital form is determined in children already in the first or second year of life.
In general, adrenal hyperplasia (what it is, we have already explained) has the following main symptoms:
- unstable blood pressure (low or high);
- progressive rates of diabetes;
- weight gain;
- manifestations of osteoporosis;
- numbness of the muscles, their subsequent atrophy;
- women are characterized by a “moon-like face”;
- memory impairment;
- nervous breakdowns;
- pigmentation;
- stretch marks on the skin;
- disorders in the gastrointestinal tract;
- decreased immunity;
- nighttime urine discharge;
- intense thirst;
- psychoses
- infertility;
- amenorrhea;
- high growth not appropriate to age;
- bald patches in the temporal region;
- hirsutism.
In men and women, the clinical signs of the disease vary.
Treatment principles
The basis of the treatment, of course, is hormone therapy. Scientists have not yet decided how to most effectively influence adrenal hyperplasia. Treatment is often combined. For teens, drugs are prescribed that normalize the manifestation of secondary sexual characteristics, estrogen is shown to girls, androgens are shown to boys.
Used in the treatment of a combination of the following glucocorticoids:
- cortisone acetate ;
- hydrocortisone;
- dexamethasone;
- prednisone.
For a day, the patient receives 2 or 3 doses of hormones (in equal amounts). A 5% glucose solution is also prescribed. Girls, unlike boys, have to take the necessary drugs all their lives.
For each patient, the doctor must find a special approach, prescribe his treatment tactics (it all depends on his experience and knowledge). There is no single therapy in the treatment of this disease.
Congenital severe adrenal cortical hyperplasia is treated by surgeons. There are cases that the genitals cannot be differentiated as female or male. For girls, plastic is recommended with an irregular shape of the labia. Already in the first year of life, such an operation is indicated, sometimes (in serious condition) it is performed later. They also perform operations with tumors.
If the body lacks salt, then the patient should increase the daily dose by 1-3 grams. Mineralocorticoids are also prescribed in such cases.
With diffuse hyperplasia, drug therapy does not have a large effect. Adrenalectomy is required in such cases. Treatment with a combination of aldosteroma with diffuse hyperplasia is also ineffective. The forecast will be unfavorable. The subject of choice is drug therapy for bilateral glomerular hyperplasia (idiopathic hyperaldosteronism). Operation in this case is an extreme measure.
The basis for the treatment of IHA is spironolactone; it is an antagonist of aldosterone. Also effective is the medicine eplerenone. Spironolactone is often taken in combination with calcium antagonists. If arterial hypertension is present in the symptoms, ACE inhibitors are connected to the treatment.
In the case of virilization, supportive medication is required on an ongoing basis. If the type of adrenal hyperplasia is virilizing, cortisol is necessary. It can be used as an intramuscular injection. The dose is selected by the attending physician. For older children, the use of prednisone is possible.
If proper treatment is not available, then hyperplasia can lead to the development of Cohn's disease or Cushing's disease. After a correct diagnosis is made, treatment should be started immediately.
Diagnosis of the disease
Adrenal hyperplasia - what is it? How to diagnose a disease? Nodular and diffuse hyperplasia is detected during instrumental and laboratory studies. Diagnostics includes: patient interrogation, physical examination, clinical examination, X-ray examination or magnetic resonance imaging, radionuclide scanning. According to the results, the size and shape of both adrenal glands are estimated. A radioimmunological laboratory test is being conducted. The content of cortisol in the urine and blood is determined. To assess the hormonal status, an enzyme-linked immunosorbent assay is performed. Sometimes it is necessary to carry out aspiration puncture.
Based on the results of the diagnosis, the doctor makes a diagnosis and prescribes the most suitable treatment method.
Hyperplasia in adults: what to do?
Usually, hyperfunction of the adrenal cortex is diagnosed in infancy, but adults also show signs of the disease. Hyperplasia of the adrenal glands in women is characterized by increased pubic hair growth, low timbre of the voice, acne, pain in the back of the head, night cramps, undeveloped breasts, high blood pressure, adrenogenital syndrome.
If you find yourself with these symptoms, do not try to make a diagnosis yourself. In these cases, folk remedies will not help either. Urgently contact the doctors. Hormonal treatment is needed. Usually, women with a similar diagnosis are not recommended to plan a pregnancy. In many cases, it is even forbidden to give birth. Sometimes the best solution to the problem is the complete elimination of the diseased kidney, as well as all its appendages.
Prevention
What is adrenal hyperplasia - this is especially necessary for those couples who have planned the birth of a child, and in the family they (genetically) have cases of congenital hyperplasia. It is necessary to turn to geneticists - qualified, knowledgeable specialists. For the prevention of the disease, it will be necessary to make perinatal diagnosis (at a time when the baby is still developing and is in the womb). In the first trimester of pregnancy, a chorionic biopsy is performed. In the second half, an analysis of amniotic fluid is required, which will determine the level of hormones.
A possible option is IVF. In this case, a preliminary genetic analysis of germ cells can be done before they are fertilized.
During gestation, women with a diagnosis of hyperplasia should be under the constant supervision of a doctor, regularly take all the required tests. It is necessary to exclude from life all possible toxic substances and exposure to radiation.
For the prevention of adrenal hyperplasia, an important role is played by screening testing conducted in a newborn baby. For analysis, it is necessary to take blood from the capillary (the heel of the baby is chosen as the place for puncture).