Traditional medicine

The key clinical syndrome of primary hyperaldosteronism is. Secondary hyperaldosteronism: symptoms, diagnosis, treatment

Hyperaldosteronism is a disorder of the adrenal glands caused by excessive secretion of aldosterone, one of the active mineralcorticoids. Its main symptom is increased blood pressure. The task of the adrenal glands is to produce a number of different hormones, including mineralcorticoids. With the help of the latter, the water-salt balance is regulated. Aldosterone plays a major role in this. Both excess and insufficient synthesis of the hormone leads to disturbances in the functioning of the body. Hyperaldosteronism is a syndrome that occurs when there is excessive production of aldosterone.

Hyperaldosteronism

Aldosterone is the most active of the mineralocorticoids and is produced in the zona glomerulosa of the adrenal cortex. and release into the blood is caused by a low concentration of sodium and a high concentration of potassium in the blood. Also, ACTH and, of course, the renin-angiotensin system can act as a stimulator of synthesis.

Aldosterone acts by the following mechanism:

the hormone binds to mineralocorticoid receptors in the renal tubules;
at the same time, the synthesis of sodium ion transporter proteins is stimulated and the latter is removed from the lumen of the tubule into the epithelial cell of the renal tubule;
the production of potassium ion transporter proteins increases. Potassium is excreted from the renal tubular cells into primary urine;
the water-salt balance is restored.

The picture looks different when, for one reason or another, the secretion of aldosterone unjustifiably increases. The hormone promotes increased sodium reabsorption, which leads to an increase in the content of sodium ions in the blood. This stimulates the production of antidiuretic hormone and causes water retention. At the same time, hydrogen, magnesium, and, most importantly, potassium ions are excreted in the urine, which automatically leads to the development of hypernatremia and hypokalemia.

Both deviations contribute to a sustained increase in blood pressure, in which excess mineralcorticoids cause direct damage to the myocardium, blood vessels and kidneys.

Hyperaldosteronism is a complex of symptoms resulting from excessive synthesis of aldosterone. Moreover, the synthesis is not caused by the action of conventional stimulants and is practically independent of the renin-angiotensin system.

Etiology and pathogenesis

Primary hyperaldosteronism is the cause of elevated blood pressure in 10–15% of cases. Usually it affects middle-aged people - 30-50 years old, mainly women - up to 70%.

There are primary and secondary hyperaldosteronism. In the first case, excessive production of aldosterone is caused by disturbances in the functioning of the adrenal cortex and does not depend on external factors. In the second case, hormone synthesis is stimulated by an external factor - arterial hypertension, heart failure, cirrhosis of the liver.

The causes of the syndrome are very diverse.

Primary hyperaldosteronism is caused by:

Conn's syndrome - aldosterone-producing adrenal adenoma, accounts for more than 65% of cases;
idiopathic hyperaldosteronism – is formed due to diffuse bilateral small-nodular hyperplasia of the adrenal cortex. It causes 30–40% of cases of the disease. Its etiology remains unclear. But unlike other forms of the disease, the zona glomerulosa remains sensitive to angiotensin II. ACTH regulates aldosterone synthesis;
adrenal hyperplasia – unilateral and bilateral;
glucocorticoid-dependent hyperaldosteronism is a hereditary disease caused by a gene defect;
aldosterone-producing carcinoma is a very rare case, no more than 100 such patients have been described;
pseudohyperaldosteronism - it is based on a congenital gene defect, which leads to inhibition of the production of angiotensin I and, ultimately, to a decrease in aldosterone;
Itsenko-Cushing syndrome – excess aldosterone is caused by increased secretion of ACTH;
congenital or drug-induced deficiency.

Causes of primary hyperaldosteronism

Secondary hyperaldosteronism is associated with the underlying pathology, which is the cause of its appearance:

excessive activity of the renin-angiotensin-aldosterone system - pregnancy, excessive potassium intake, sodium loss associated with diet, diarrhea, medication, decreased blood volume due to blood loss, and so on;
organic secondary hyperaldosteronism – arterial stenosis, tumor;
functional hyponatremia, hypovolemia, and so on;
disturbance of aldosterone metabolism in heart failure, kidney disease, etc.

A characteristic difference between secondary hyperaldosteronism and primary hyperaldosteronism is that it does not cause disturbances in the electrolyte balance, since it is a natural reaction to the excessive functioning of the renin-angiotensin-aldosterone system.

Causes of secondary hyperaldosteronism

Types and symptoms

Depending on the type of illness, the symptoms differ. The decisive factor here is the method of regulating the synthesis and secretion of aldosterone. Thus, in primary hyperaldosteronism, the hormone is produced uncontrollably due to a disorder in the adrenal cortex, while in the secondary form, production is stimulated by the RAAS. Accordingly, in the first case there is a violation of the water-salt balance, but in the second - not. This is precisely what explains the difference in the clinical picture.

Primary

Primary hyperaldosteronism is characterized by:

– observed in 100% of patients, although recently an asymptomatic course of the disease has begun to be noted. Blood pressure is constantly elevated, especially diastolic, which quite quickly leads to left ventricular hypertrophy, and, therefore, to changes in the ECG. At the same time, 50% of patients have vascular lesions of the fundus, and 20% have visual impairment;
hypokalemia – 100% of patients. Lack of potassium leads to dysfunction of muscle and nervous tissue. This manifests itself as weakness and rapid muscle fatigue up to pseudoparalytic states and convulsions;
the analysis shows an increase in aldosterone levels and a low renin level in 100 cases out of 100. Moreover, the level of the hormone is not regulated;
hypochloremic alkalosis is observed - an increase in the pH level in the blood due to the accumulation of alkaline metabolic products;
and nocturnal polyuria - 85 and 72%, respectively, is caused by changes in the renal tubules caused by hypokalemia. The symptom is accompanied by a constant feeling of thirst;
in 65% of cases, hypernatremia is observed - an increase in the concentration of sodium ions with a decrease in potassium concentration - a natural phenomenon. However, the sensitivity of the renal tubules to sodium retention, which is caused by aldosterone, may be markedly reduced. In the absence of hypernatremia, suspicion of PHA is caused by urinary potassium excretion exceeding 40 mEq/day;
in 51% of cases, blood pressure causes persistent headache;
water-electrolyte imbalance can also cause psycho-emotional disorders - hypochondria, asthenic syndrome, and so on.

All the described symptoms are most characteristic of Conn's syndrome, the most common cause of RAH.

Other cases are much less common:

idiopathic hyperaldosteronism with similar symptoms allows you to regulate the production of aldosterone, since the zona glomerulosa remains sensitive to the action of angiotensin II;
Bilateral hyperplasia is characterized by sensitivity to glucocorticosteroids: when taking GCS, potassium metabolism is normalized and blood pressure decreases;
pseudohyperaldosteronism is accompanied by typical signs of PAH. However, there is no response to the drug.

Diagnosis of the disease is very difficult. Not only external symptoms are important, but the response to the administration of certain drugs. Thus, the administration of veroshpiron for 2 weeks normalizes potassium metabolism and reduces blood pressure. However, this effect is typical only for PHA. If it is absent, then the diagnosis was erroneous.

Symptoms and signs of primary hyperaldosteronism

Secondary

The clinical picture of HAV is strongly associated with the symptoms of the underlying disease. Secondary hyperaldosteronism is a kind of compensatory phenomenon and does not have its own characteristic symptoms. Its clear difference from PHA is the preservation of water-salt balance, which means the absence of high blood pressure, hypernatremia or hypokalemia.

Often secondary hyperaldosteronism is associated with the appearance of edema. Fluid retention and sodium accumulation causes increased secretion of aldosterone. In fact, in HAV, aldosterone synthesis is driven by hypernatremia.

Diagnostics

The similarity of symptoms and their ambiguity makes diagnosis of the disease very difficult and time-consuming. What is required is not just research, both laboratory and instrumental, but also a number of functional tests of different nature. Diagnostics is carried out in several stages.

Primary

Carried out in order to exclude or confirm PHA. To do this, the level of potassium in plasma is determined at least 2 times in all patients with high blood pressure. Primary hyperaldosteronism is characterized by a persistently low level of potassium in the blood - less than 2.7 mEq/L, regardless of the use of antihypertensive drugs. With normokalemic hyperaldosteronism, the potassium level against the background of increased aldosterone levels is above 3.5 mEq/L.

Diagnosis of PHA syndrome

At this stage, hormone levels are examined in order to establish the true cause of the disease.

Primary PHA is characterized by:

low renin activity is not a 100% indicator, since its insufficiency is, in principle, characteristic of 25% of hypertensive patients, especially the elderly;
high concentration of aldosterone in the blood or increased urinary excretion of hormone breakdown products. A sign characteristic of 70% of patients. It should be taken into account that the level of aldosterone decreases with hypervolemia, hypokalemia, and so on;
a sodium load stimulation test may provide the required response. The patient is injected with 2 liters of sodium chloride solution, which normally leads to a decrease in aldosterone concentration by 50%. With primary hyperaldosteronism, such a decrease does not occur, since hormone synthesis is insensitive to external factors. Carrying out the test requires great caution, since the sodium load noticeably worsens the patient’s well-being - weakness and heart rhythm disturbances appear.

Differential diagnosis of hyperaldosteronism

Determination of nosological form

At this stage, functional tests and biochemical tests of blood and urine are carried out:

An increase in the concentration of 18-hydroxycorticosterone is one of the most reliable signs of PHA. Again, with the exception of idiomatic, where 18-hydroxycorticosterone remains normal or slightly elevated.
High levels of cortisone breakdown products in the urine are also typical for PHA.
Functional tests are based on the body’s response to certain drugs and loads:
- orthostatic load - 4 hours of walking, in combination with a 3-day low-salt diet, does not stimulate renin activity in the blood - ARP, and the aldosterone level may even decrease. The same response follows when taking active saluretics. Basal ARP is measured in the fasting state after a night's sleep on a diet containing no more than 120 mEq/day of sodium;
- spironolactone test - 3-day administration of spironolactones (600 mg/day) does not stimulate renin activity and does not in any way affect the production of aldosterone;
- test with captopril - with aldosterone, the circadian rhythm of aldosterone is maintained both after walking and at rest. Absence of rhythm is an indicator of a malignant tumor;
- test with DOXA - 10 mg of the drug is administered every 12 hours for 3 days. With aldosterone and in most cases with idiopathic PHA, the drug has no effect on the synthesis of aldosterone.
Idiopathic PHA is more difficult to diagnose due to the preserved sensitivity of the renal tissue. With this disease, all the signs turn out to be mild, but at the same time, stimulating tests are less effective than in healthy people: the level of aldosterone is lower, the concentration of 18-hydroxycorticosterone is much lower, renin activity is reduced, but increases after walking.
In case of carcinoma, the reaction to the tests is completely absent.
Glucocorticoid hyperaldosteronism is detected with the following signs: ineffectiveness of antihypertensive therapy, increased excretion of 18-oxocortisol and 18-hydroxycortisol against the background of normal potassium levels in the blood, no change in aldosterone levels during orthostatic load. A trial of treatment with dexamethasone or prednisolone brings rapid and lasting results.
Familial forms of PHA can only be established using genetic diagnostics.

Instrumental methods

If PHA can be considered proven based on biochemical indicators, additional studies are prescribed to determine the localization of the pathology:

– allows you to identify aldosterone-producing adenoma with an accuracy of 62%. In addition, the method allows you to exclude tumors.
CRT – the accuracy of detecting adenoma is 100%.
Ultrasound – sensitivity is 92%. This is one of the safest methods.
Phlebography of the adrenal glands - here the concentration gradient of aldosterone and renin at different levels is studied. The method is accurate, but very complex.
adrenal glands - most informative for small and large nodular hyperplasia, as well as tumors and adenomas. The test is carried out against the background of blockade of the thyroid gland.

In the video about diagnosing hyperaldosteronism:

Treatment

The main treatment for PHA is surgery, usually removing the affected adrenal gland. But in certain cases this method is unacceptable.

Thus, for bilateral hyperplasia, surgery is indicated only if the drug treatment has completely failed.
In the idiopathic form of GPA, conservative treatment is prescribed.
In the case of carcinoma, surgery is combined with chemotherapy.
The glucocorticoid-dependent form does not require surgical intervention. The administration of dexomethasone completely normalizes blood pressure within 3–4 weeks.
Secondary hyperaldosteronism rarely requires its own treatment. Here it is necessary to eliminate the underlying disease.

Medication

For conservative treatment, the following drugs are used:

aminoglutethimide – 2-3 times a day. Treatment is carried out against the background of monitoring blood pressure, the level of cortisol in the urine - at least once a day, thyroid hormones, and so on;
spironolactone – 2 times a day, 50 mg. Treatment can be combined with the use of potassium-sparing diuretins - this way it is possible to prevent side effects;
spironolactone – 1–2 times a day 25–50 mg with amyloid and triamterene. For severe hypokalemia, potassium supplements are added. After normalization of the plasma potassium concentration, the dose is reduced.

Pseudohyperaldosteronism, as well as the glucocorticoid-dependent form, can be cured with small doses of dexamethasone.

Surgical intervention

The operation is quite complex and requires lengthy preparation – at least 4 weeks. Its goal is to reduce ADL, restore normal potassium levels in the blood and RAAS function.

For this purpose they prescribe:

aminoglutethimide – 250 mg 2-3 per day. The dose is increased if treatment is ineffective;
spironolactone – 50–100 mg 2–4 times a day. A combination of spironolactone and amiloride is used. If blood pressure does not decrease, antihypertensive drugs may be administered.

The most common procedure is unilateral adrenalectomy - removal of the adrenal gland. The operation is performed through entry into the abdominal cavity, without it and from the back. There are other methods - transarterial administration of alcohol, portalization of blood flow, but today they are not widespread.

In the postoperative period, replacement therapy is carried out: 25–50 mg of hydrocortisone is administered every 4–6 hours for 2–3 days. The dose is gradually reduced as signs of adrenal insufficiency decrease.

Clinical recommendations for this disease are general only. Treatment must be selected individually, taking into account the general condition of the patient and the characteristics of his body. Guidelines do not set standards because they do not guarantee results.

High blood pressure is a symptom of many diseases, which makes diagnosis extremely difficult. The complaints of patients in this case are non-specific, so the question of referral for research remains open. The recommendations help identify those groups of patients in whom PGA is more likely.

arterial hypertension stages 1 and 2;
high blood pressure, insensitive to drug treatment;
a combination of hypertension and hypokalemia, including drug-induced;
combination of hypertension and adrenal incidentaloma;
hypertension due to a family history - previous development of hypertension, a close relative with PHA, and so on.

As a primary study of patients in these groups, determination of the aldosterone-renin ratio is indicated. To clarify PGA, it is recommended to carry out functional tests.

A CT scan is ordered to rule out cancer.

Patients with early development of the disease - under 20 years of age, and who have relatives with PHA, are prescribed genetic testing in order to establish glucocorticoid-dependent PHA.

Forecasts

According to statistics, surgical intervention provides 50–60% of complete recovery for adrenal adenoma. Carcinoma has a poor prognosis.

When adenoma is combined with diffuse and diffuse nodular hyperplasia, complete recovery cannot be achieved. To maintain remission, patients require ongoing therapy with spironolactone or steroidogenesis inhibitors.

The same applies to patients with bilateral adrenal hyperplasia.

Hyperaldosteronism is a common name for a number of diseases that cause the same clinical picture. Secondary hyperaldosteronism, as a rule, disappears along with the underlying disease. The prognosis for the cure of PHA is not so encouraging.

Contents of the article

Primary hyperaldosteronism (Conn's syndrome)- excessive secretion of aldosterone by the adrenal cortex, regardless of its external stimulation. Manifestations of primary hyperaldosteronism were first described by J. Conn (1956).

Etiology and pathogenesis of primary hyperaldosteronism

Primary hyperaldosteronism can be caused by adenoma, carcinoma and bilateral hyperplasia of the adrenal cortex. The most common type is adenoma of the adrenal cortex, which usually occurs in women aged 30 to 50 years. Primary hyperaldosteronism is considered the cause of 1% of cases of arterial hypertension. Excessive secretion of aldosterone leads to increased sodium reabsorption in the distal tubules of the kidneys. As a result of water retention, the extracellular volume of fluid increases. In this regard, sodium reabsorption in the proximal tubules decreases, which leads to some stabilization of the state of sodium metabolism in the body. The main manifestations of primary hyperaldosteronism are associated with an increase in the volume of extracellular fluid - arterial hypertension and a decrease in plasma renin activity.
Aldosterone increases the secretion of potassium and hydrogen in the distal tubules, which can increase even when sodium metabolism is stabilized.

Clinic of primary hyperaldosteronism

The main clinical manifestation is arterial hypertension, which is sometimes accompanied by orthostatic hypotension. Patients often complain of headaches, tinnitus, blurred vision, and cerebrovascular accidents may occur. Disorders of electrolyte metabolism are typical - hypokalemia, hypernatremia and metabolic alkalosis. It is hypokalemia that causes other important manifestations of this syndrome - muscle weakness, polyuria, especially at night, polydipsia and paresthesia. With severe hypokalemia, periodic paralysis of the limbs and even tetany can develop. Concomitant orthostatic hypotension is not accompanied by reflex tachycardia. With arterial hypertension and hypokalemia, dystrophic changes in the myocardium develop, arrhythmias appear, in particular extrasystole, and the U wave on the ECG increases. Edema of the extremities is uncommon. With a long course of the disease, damage to the kidneys and heart develops.

Diagnosis and differential diagnosis of primary hyperaldosteronism

Primary hyperaldosteronism should be suspected in patients with diastolic hypertension without edema and low plasma renin levels, which do not increase under the influence of various stimuli, in particular with an increase in dietary sodium. Urinary aldosterone excretion is increased and does not decrease with sodium loading. Characterized by persistent hypokalemia. It should be remembered that hypokalemia in patients with arterial hypertension can develop quickly when treated with diuretics (thiazides, furosemide), therefore the level of potassium in the blood should be determined before starting treatment. If diuretic treatment has already been started, it should be stopped and the patient should be prescribed potassium chloride orally for 1-2 weeks. It should be borne in mind that plasma renin levels are low, in approximately 1/4 of hypertensive patients without hyperaldosteronism. However, in this case, it increases under the influence of various stimuli that reduce plasma volume. If there are laboratory signs of hyperaldosteronism, computed tomography of the adrenal glands is performed to clarify the possible location of the adenoma.

Arterial hypertension, close to malignant, can occur with hypokalemia and hyperaldosteronism. However, unlike primary hyperaldosteronism, the plasma renin level is increased. Primary adrenal hyperplasia with aldosteronism is accompanied, in contrast to adrenal adenoma, by less pronounced hypokalemia, lower aldosterone secretion and a higher level of plasma renin activity. A reliable method for their differential diagnosis is computed tomography of the adrenal glands.
Adrenal cortical adenomas that secrete deoxycorticosterone, in contrast to aldosterone, are characterized by normal plasma aldosterone levels, although plasma renin activity is reduced. Increased mineralocorticoid secretion may be associated with a hereditary defect of certain enzymes. Deficiency of 11-(3- and 17-a-hydroxylases leads to impaired secretion of hydrocortisone with an increase in the release of ACTH and a secondary increase in the production of deoxycorticosterone. With a deficiency of 17-a-hydroxylase, the biosynthesis of androgens and estrogens is disrupted by both the adrenal glands and the gonads. As a result, the biosynthesis of androgens and estrogens is impaired. development of secondary sexual characteristics. In these conditions, arterial hypertension and hypokalemia can be corrected by the administration of glucocorticoids. To clarify the diagnosis, the level of precursors of hydrocortisone biosynthesis is determined both in the blood and in the urine. In some patients with an increase in mineralocorticoid function and ACTH levels, the administration of glucocorticoids improves the condition. absence of hydroxylase defect.

Secondary hyperaldosteronism develops in response to activation of the renin-angiotensin system. This condition occurs during normal pregnancy, arterial hypertension with a tendency to a malignant course, especially renovascular hypertension, edema syndrome, liver cirrhosis, nephrotic syndrome, congestive heart failure. In these situations, increased aldosterone secretion is due to arterial hypovolemia and hypotension.

Hyperaldosteronism is a fairly frequently diagnosed pathological condition that develops against the background of increased secretion of an adrenal hormone such as aldosterone. The pathology most often occurs in adults, but can also affect children.

Provoking factors will differ depending on the form of the disease, ranging from burdened heredity and ending with the course of ailments of an endocrinological or other nature.

Clinical signs for the primary and secondary forms of the disease will be different. The basis of the symptoms are impaired functioning of the heart, muscle weakness, seizures and development.

Only an endocrinologist can make a correct diagnosis and differentiate between the different types of disease, based on data from a wide range of instrumental and laboratory examinations.

Treatment tactics can be either conservative or surgical, which is directly dictated by the type of such pathology. In any case, the lack of therapy is fraught with life-threatening complications.

Etiology

Hyperaldosteronism is a complex of syndromes with different mechanisms of occurrence, but similar in symptoms, that develop due to increased secretion of aldosterone.

Since there is primary and secondary hyperaldosteronism, it is natural that the predisposing factors will be somewhat different.

The first type of disease in extremely rare cases occurs against the background of a genetic predisposition. The familial form can be inherited in an autosomal dominant manner - this means that to diagnose such a disease in a child, it is enough for him to inherit the mutant gene from one of the parents.

The defective segment is the enzyme 18-hydroxylase, which for unknown reasons goes beyond the control of the renin-angiotensin system and is corrected by glucocorticoids.

Rare provocateurs of primary hyperaldosteronism include cancer of the adrenal glands.

However, in the vast majority of situations, this variant of the course of the disease is caused by the formation of aldosteroma - this is a neoplasm, which, in fact, is an aldosterone-producing adenoma of the adrenal cortex. Such a tumor is diagnosed in approximately 70% of cases of the primary form of pathology.

Secondary hyperaldosteronism is characterized by the occurrence of another disease in the human body, which means that dysfunction of the endocrine system in such situations acts as a complication.

The following pathological conditions can lead to the development of a secondary type of disease:

Barter syndrome;
dysplasia and stenosis of the arteries in the kidneys;
formation of reninoma in the kidneys;

In addition, the following can lead to secondary hyperaldosteronism:

sodium deficiency, which is very often provoked by strict diets or excessive diarrhea;
a decrease in the volume of circulating blood - this is often observed against the background of heavy blood loss and dehydration;
excess potassium;
uncontrolled use of certain medications, in particular diuretics or laxatives.

It is worth noting that the main risk group is female representatives in the age category from 30 to 50 years. However, this does not mean that the disease does not occur in other categories of patients.

Classification

Endocrinologists distinguish the following main types of such pathology:

primary hyperaldosteronism- considered one of the most common variations of the disease;
secondary hyperaldosteronism- is a complication of diseases that negatively affect the heart, liver and kidneys;
pseudohyperaldosteronism- is a consequence of impaired perception of aldosterone by the distal renal tubules.

At the same time, primary hyperaldosteronism has its own classification, which includes:

Conn's syndrome;
idiopathic hyperaldosteronism - develops only against the background of diffuse nodular hyperplasia of the adrenal cortex, which is bilateral. Diagnosed in approximately every third patient who seeks qualified help when symptoms arise;
unilateral or bilateral adrenal hyperplasia;
glucocorticoid-dependent hyperaldosteronism;
aldosterone-producing carcinoma - in total, no more than 100 patients with a similar diagnosis have been registered;
pseudohyperaldosteronism - is a consequence of impaired perception of aldosterone by the distal renal tubules;
congenital insufficiency of the adrenal cortex or caused by drug overdose.

As a separate form, it is worth highlighting extra-adrenal hyperaldosteronism - it is the most rare. Among the provoking factors, the main place is occupied by diseases of the endocrine system, for example, the ovaries and thyroid gland, as well as the gastrointestinal tract, in particular the intestines.

Symptoms

As mentioned above, the symptomatic picture will differ depending on the type of disease. Thus, with primary hyperaldosteronism, the expression of the following symptoms is observed:

increased blood tone - a symptom observed in absolutely all patients, but recently clinicians have noted an asymptomatic course of the disease. Blood pressure is constantly elevated, and this can lead to hypertrophy of the left ventricle of the heart. Against the background of this manifestation, half of the patients experience vascular damage to the fundus, and 20% have a decrease in visual acuity;
muscle weakness - similar to the previous sign, typical for 100% of patients. In turn, it becomes the cause of decreased performance, the development of a pseudoparalytic state and convulsions;
change in the shade of urine - it becomes cloudy due to the presence of a large amount of protein in it. Composes the clinical picture for 85% of people;
an increase in the daily volume of urine excreted - occurs in 72% of patients;
constant thirst;
persistent headaches;
development ;
causeless anxiety.

It is worth considering that the above symptoms refer to the most common form of primary hyperaldosteronism - Conn's syndrome.

Symptoms of secondary type hyperaldosteronism are presented:

an increase in blood pressure, especially diastolic, which over time leads to the appearance of chronic renal failure, kidney dysfunction and damage to the walls of blood vessels;
neuroretinopathy leading to optic nerve atrophy and complete blindness;
hemorrhages in the fundus of the eye;
severe swelling.

Some patients do not show signs of arterial hypertension, and in rare cases, there is an asymptomatic course of such a pathology.

In children, hyperaldosteronism often manifests itself before the age of 5 and is expressed in:

bright manifestation;
increasing arterial hypertension;
retardation in physical development;
psycho-emotional disorders.

Diagnostics

The implementation of a whole range of diagnostic measures is aimed not only at establishing the correct diagnosis, but also at differentiating various forms of the disease in women and men.

First of all, the endocrinologist must:

get acquainted with the medical history of not only the patient, but also his close relatives - to detect pathologies that may cause secondary hyperaldosteronism or confirm the hereditary nature of the disease;
collect and study a person’s life history;
carefully examine the patient - a physical examination is aimed at assessing the condition of the skin and measuring blood pressure. This should also include an ophthalmological examination of the fundus;
interview the patient in detail - to draw up a complete symptomatic picture of the course of hyperaldosteronism, which can actually indicate the type of its course.

Laboratory diagnosis of hyperaldosteronism involves:

biochemical blood test;
general clinical study of urine;
measuring the daily volume of urine excreted;
PCR tests - to diagnose the familial form of the disease;
tests with spironolactone and hypothiazide load;
"march" test;
serological tests.

The following instrumental examinations are of greatest value:

In addition to the basic diagnosis, the patient should be examined by an ophthalmologist, nephrologist and cardiologist.

Treatment

The tactics of treating the disease are dictated by its type, however, there are several treatment methods inherent in all forms of hyperaldosteronism. These include:

maintaining a gentle diet aimed at reducing the consumption of table salt and enriching the menu with foods enriched with potassium;
taking potassium-sparing diuretics;
injection of potassium preparations.

Treatment of hyperaldosteronism caused by the formation of aldosteroma or cancer of the adrenal glands is only surgical. The operation involves excision of the affected segment, which first requires restoration of the water and electrolyte balance.

Bilateral hypoplasia of the adrenal cortex is eliminated in a conservative way. Through the use of ACE inhibitors and calcium channel antagonists.

The hyperplastic form of hyperaldosteronism is treated with complete bilateral adrenalectomy.

Patients with secondary hyperaldosteronism are advised to eliminate the underlying disease and mandatory intake of glucocorticoids.

Possible complications

Due to the rapid progression of clinical signs, hyperaldosteronism quite often leads to the following complications:

chronic renal failure;
complete loss of vision;
nephrogenic diabetes insipidus;
hearts;
coronary heart disease;
destruction of the walls of blood vessels;
paresthesia;
malignant arterial hypertension.

Prevention and prognosis

To ensure that a man, woman and child does not have problems with the formation of such a disease, it is necessary to adhere to the following general clinical recommendations:

maintaining a healthy lifestyle;
proper and nutritious nutrition;
consultation with geneticists - this is necessary for couples who decide to have children to find out the likelihood of giving birth to a baby with hyperaldosteronism;
constant clinical observation - indicated for patients with hypertension;
taking only those medications prescribed by the clinician - with strict adherence to the daily dosage and duration of treatment;
undergoing a full preventive examination in a medical institution - for early detection of ailments that can lead to the appearance of secondary hyperaldosteronism.

As for the prognosis of the disease, it is dictated by the severity of the underlying disease and the degree of damage to internal organs, as well as timely diagnosis and adequate therapy.

Radical surgical intervention and adequate drug treatment guarantee a complete recovery. The outcome of adrenal cancer is often unfavorable.

Is everything in the article correct from a medical point of view?

Answer only if you have proven medical knowledge

Hyperaldosteronism is a syndrome caused by hypersecretion of aldosterone (mineralocorticoid hormone of the adrenal cortex), accompanied by arterial hypertension and severe electrolyte disturbances. It is customary to distinguish primary and .

Primary hyperaldosteronism is a consequence of primary excess production of aldosterone directly in the glomerular layer of the adrenal cortex.

In secondary hyperaldosteronism, stimulation of the production of excess aldosterone occurs due to the influence of pathological factors located outside the adrenal glands. In addition, there is a group of diseases that are characterized by similar symptoms that are not accompanied by increased levels of aldosterone (syndromes that mimic hyperaldosteronism).

Primary hyperaldosteronism, first described by Conn in 1956, is in most cases the result of an autonomous solitary aldosterone-producing adrenal adenoma ( Conn's syndrome), less commonly - macronodular or micronodular bilateral hyperplasia (idiopathic hyperaldosteronism) or adrenal cancer. In most cases, a unilateral adrenal adenoma is detected, usually small in size (up to 3 cm in diameter), occurring with equal frequency on both sides.

Etiology and pathogenesis

The disease occurs more often in women (2 times more often than in men), usually between the ages of 30 and 50 years. Since the main symptom of hyperaldosteronism is arterial hypertension, it is of fundamental importance that primary hyperaldosteronism is detected in approximately 1% of the general population of patients with arterial hypertension. The cause of the disease is unknown. It should be remembered that hyperaldosteronism, caused by hyperplasia of the zona glomerulosa of the adrenal cortex, is characterized by maintaining sensitivity to stimulation by angiotensin II.

In addition, familial hyperaldosteronism is distinguished, suppressed by glucocorticoids and with preserved sensitivity to pituitary ACTH (familial hyperaldosteronism type I), which develops due to the formation of a defective enzyme during crossing over of the 11-β-hydroxylase and aldosterone synthetase genes located on the 8th chromosome. As a result of this breakdown, both genes become sensitive to ACTH and aldosterone synthesis is initiated not only in the zona glomerulosa, but also in the zona fasciculata of the adrenal cortex, which is accompanied by an increase in the production of aldosterone and 11-deoxycorticortisol metabolites (18-oxocortisol and 18-hydroxycortisol).

The pathogenesis of primary hyperaldosteronism is associated with excessive accumulation of sodium in the blood serum and increased excretion of potassium in the urine. As a result, intracellular hypokalemia and partial replacement of potassium ions in the cell with hydrogen ions from the extracellular fluid are observed, which is accompanied by stimulation of the excretion of chlorine in the urine and causes the development of hypochloremic alkalosis. Persistent hypokalemia leads to damage to the renal tubules, which lose the ability to concentrate urine, and clinically this is accompanied by hyposthenuria and secondary polydipsia. At the same time, hypokalemia leads to a decrease in sensitivity to ADH (antidiuretic hormone - vasopressin), which aggravates polyuria and polydipsia.

At the same time, hypernatremia causes water retention with the development of hypervolemia and arterial hypertension. The important fact is that, despite the retention of sodium and fluid, with primary hyperaldosteronism edema does not develop (the escape phenomenon), which is explained by an increase in cardiac output, arterial hypertension and hypertensive diuresis.

The long-term presence of hyperaldosteronism is accompanied by complications caused by arterial hypertension (myocardial infarction, stroke) and specific myocardial hypertrophy. As mentioned above, constant hypersecretion of aldosterone leads to progressive hypokalemia, which determines the development of hypokalemic myopathy, which leads to the appearance of degenerative changes in the muscles.

Symptoms

Most patients have arterial diastolic hypertension, accompanied by headaches (arterial hypertension syndrome) and not amenable to treatment with antihypertensive drugs in average therapeutic doses; hypertensive crises can be provoked by thiazide or loop diuretics and accompanied by cardiac or cerebral symptoms.

An increase in blood pressure in combination with hypokalemia causes electrocardiographic abnormalities: flattening or inversion of the T wave appears, a decrease in the S-T segment, the Q-T interval lengthens, a pronounced U wave (wave) appears. Cardiac arrhythmias and extrasystole and signs of left ventricular hypertrophy are recorded. In primary hyperaldosteronism, there is no edema, while in secondary hyperaldosteronism, edema syndrome is the pathogenetic basis of the disease.

Hypokalemia, a characteristic symptom of hyperaldosteronism, predetermines the development of muscle weakness (myopathic syndrome), fatigue and decreased performance. Muscle weakness increases sharply with physical activity or suddenly (for no reason). At the same time, the severity of weakness at the time of the attack limits the possibilities of movement or minimal physical work. Paresthesia and local convulsions are possible.

As a result of impaired ability of the kidneys to concentrate urine, polyuria with hyposthenuria develops, often accompanied by secondary polydipsia. A characteristic symptom is with a predominance of night diuresis over daytime diuresis.

Depending on the degree of manifestation of the above symptoms, various options for the course of the disease are possible before diagnosis is made:

crisis variant - accompanied by hypertensive crises with pronounced neuromuscular symptoms (adynamia, paresthesia, convulsions);
a constant form of arterial hypertension with constant muscle weakness, the degree of which is inferior to the crisis form;
option without significant arterial hypertension with a predominance of transient neuromuscular disorders at the time of crisis.

Diagnostics

Diagnosis of primary hyperaldosteronism includes two mandatory stages: proof of hyperaldosteronism and diagnosis of the nosological form of the disease.

The following indicators serve as evidence of primary hyperaldosteronism:

serum potassium level
renin level is reduced (plasma renin activity);
blood aldosterone levels are increased;
daily excretion of aldosterone metabolites in urine (aldosterone-18-glucoronite) is increased.

The listed studies can be used when examining patients with arterial hypotension as screening techniques to identify the target group and conduct a special examination. In difficult cases, pharmacodynamic tests can be used:

test with an isotonic sodium chloride solution: the patient in a horizontal position is injected with 2 liters of 0.9% sodium chloride solution slowly (for at least 4 hours) and after the end of the test, the level of aldosterone is determined, which does not decrease with primary hyperaldosteronism;
test with spironolactone: for 3 days the patient receives 400 mg/day of spironolactone orally. An increase in potassium levels of more than 1 mmol/l confirms hyperaldosteronism;
test with furosemide: the patient is prescribed 0.08 g of furosemide orally. After 3 hours, there is a decrease in plasma renin activity and an increase in aldosterone levels with hyperaldosteronism;
test with 9α-fluorocortisol: for 3 days the patient receives 400 mcg/day orally of 9α-fluorocortisol (Cortinef) and the level of aldosterone is examined before and after the test. With bilateral hyperplasia of the glomerular layer of the adrenal cortex, a decrease in aldosterone levels is observed, and with aldosteroma, there is no decrease in aldosterone levels:
dexamethasone test: used to differentiate glucocorticoid-suppressed hyperaldosteronism, administration of 0.5 - 1.0 mg 2 times a day for a week leads to a decrease in the manifestations of the disease;
orthostatic test (allows you to differentiate primary hyperaldosteronism from unilateral aldosteroma and bilateral adrenal hyperplasia): after 3-4 hours of the patient staying in an upright position (standing, walking), the level of aldosterone and plasma renin activity are assessed. With autonomous aldosterome, plasma renin activity does not change (it remains low), and aldosterone levels decrease or change slightly (normally, plasma renin activity and aldosterone increase by 30% above basal values).

Indirect signs of hyperaldosteronism:

hypernatremia;
hyperkaliuria, hypokalemia;
polyuria, iso- and hyposthenuria;
metabolic alkalosis and increased bicarbonate levels in the blood serum (the result of loss of hydrogen ions in the urine and impaired bicarbonate reabsorption), as well as alkaline urine;
with severe hypokalemia, the level of magnesium in the blood serum also decreases.

Criteria for the diagnosis of primary hyperaldosteronism include:

diastolic hypertension in the absence of edema;
reduced secretion of renin (low plasma renin activity) without a tendency to adequately increase under conditions of volume reduction (orthostasis, sodium restriction);
hypersecretion of aldosterone, which is not sufficiently reduced under conditions of increased volume (salt load).

As mentioned above, the cause of primary hyperaldosteronism can be established by performing certain functional tests (orthostatic test, test with 9α-fluorocortisol). In addition, in familial hyperaldosteronism, suppressed by glucocorticoids and with preserved sensitivity to pituitary ACTH (familial hyperaldosteronism type I) and bilateral adrenal hyperplasia, there is an increase in the levels of the precursor in aldosterone synthesis - 18-hydroxycorticosterone > 50 - 100 ng/dl and increased excretion from urine 18-hydroxycortisol > 60 mg/day and 18-hydroxycortisol > 15 mg/day. These changes are most pronounced in familial hyperaldosteronism, suppressed by glucocorticosteroids.

After verification of hyperaldosteronism, additional examination is carried out aimed at clarifying the nosological form of primary hyperaldosteronism and topical diagnosis. The first step is to visualize the adrenal gland area. The preferred methods are CG, MRI and PET. Detected bilateral symmetrical pathology or unilateral space-occupying formation in the adrenal gland allows us to establish the cause of primary hyperaldosteronism. It should be remembered that imaging of the adrenal glands is only relevant in relation to the metabolic abnormalities identified.

In recent years, the list of possible evidence of primary hyperaldosteronism has been supplemented by the possibility of isolated blood sampling from the inferior hollow foam and adrenal veins with the study of aldosterone levels in samples. An increase in aldosterone levels by 3 times is considered characteristic of aldosteroma, less than 3 times is a sign of bilateral hyperplasia of the zona glomerulosa of the adrenal cortex.

Differential diagnosis is carried out with all conditions accompanying hyperaldosteronism. The principles of differential diagnosis are based on examination and exclusion of various forms of hyperaldosteronism.

Syndromes that mimic primary hyperaldosteronism include a number of diseases characterized by arterial hypertension and myopathic syndrome caused by hypochloremic alkalosis and low renin levels (pseudohyperaldosteronism), are rare and are caused by various enzymopathies. In this case, there is a deficiency of enzymes involved in the synthesis of glucocorticosteroids (11-β-hydroxylase, 11-β-hydroxysteroid dehydrogenase, 5α-reductase, P450c11, P450c17).

In most cases, syndromes that mimic primary hyperaldosteronism appear in childhood and are characterized by persistent arterial hypertension, as well as other laboratory signs of hyperaldosteronism.

Treatment

Treatment of primary hyperaldosteronism is carried out taking into account the cause that caused it.

When aldosteroma is detected, the only treatment option is surgical treatment (adrenalectomy). Preoperative preparation is carried out for 4 - 8 weeks with spironolactone at a dose of 200 - 400 mg / day. With unilateral adrenalectomy, glucocorticosteroid replacement therapy is not indicated in the vast majority of cases. After removal of the adenoma, cure of hypertension is observed in 55-60% of patients. However, hypertension may persist in approximately 30% of operated patients.

If bilateral adrenal hyperplasia is suspected, surgical intervention is indicated only in cases where severe hypokalemia accompanied by clinical symptoms cannot be controlled medically with spironolactone. Bilateral adrenalectomy, as a rule, does not improve the course of hypertension associated with idiopathic hyperplasia of the zona glomerulosa of the adrenal glands, therefore, in such cases, complex antihypertensive therapy with the obligatory use of maximum doses of spironolactone is recommended.

For familial glucocorticoid-suppressed hyperaldosteronism, suppressive therapy with dexamethasone is used at a dose of 0.5-1.0 mg/day.