Aldosteronism is a clinical syndrome associated with increased production of the adrenal hormone aldosterone in the body. There are primary and secondary aldosteronism. Primary aldosteronism (Conn's syndrome) occurs with a tumor of the adrenal gland. It is manifested by an increase in blood pressure, a change in mineral metabolism (the content in the blood sharply decreases), muscle weakness, seizures, and an increase in the excretion of aldosterone in the urine. Secondary aldosteronism is associated with increased production of aldosterone by normal adrenal glands due to excessive stimuli that regulate its secretion. It is observed in heart failure, some forms of chronic nephritis and cirrhosis of the liver.

Violations of mineral metabolism in secondary aldosteronism are accompanied by the development of edema. With kidney damage, aldosteronism increases. Treatment of primary aldosteronism is surgical: removal of the adrenal tumor leads to recovery. In secondary aldosteronism, along with the treatment of the disease that caused aldosteronism, aldosterone blockers (aldactone 100-200 mg 4 times a day orally for a week), diuretics are prescribed.

Aldosteronism is a complex of changes in the body caused by an increase in the secretion of aldosterone. Aldosteronism can be primary or secondary. Primary aldosteronism (Conn's syndrome) is caused by hyperproduction of aldosterone by a hormonally active tumor of the adrenal gland. It is clinically manifested by hypertension, muscle weakness, seizures, polyuria, a sharp decrease in the content of potassium in the blood serum and increased excretion of aldosterone in the urine; edema, as a rule, does not happen. Removal of the tumor leads to a decrease in blood pressure and normalization of electrolyte metabolism.

Secondary aldosteronism is associated with dysregulation of aldosterone secretion in the adrenal zona glomeruli. A decrease in the volume of the intravascular bed (as a result of hemodynamic disorders, hypoproteinemia or changes in the concentration of electrolytes in the blood serum), an increase in the secretion of renin, adrenoglomerulotropin, ACTH leads to hypersecretion of aldosterone. Secondary aldosteronism is observed in heart failure (congestion), liver cirrhosis, edematous and edematous-hypertonic forms of chronic diffuse glomerulonephritis. The increased content of aldosterone in these cases causes an increase in sodium reabsorption in the renal tubules and thus may contribute to the development of edema. In addition, an increase in the secretion of aldosterone in the hypertensive form of diffuse glomerulonephritis, pyelonephritis or occlusive lesions of the renal arteries, as well as in hypertension in the late stages of its development and a malignant variant of the course, leads to a redistribution of electrolytes in the walls of arterioles and to increased hypertension. Suppression of the action of aldosterone at the level of the renal tubules is achieved by using its antagonist - aldactone, 400-800 mg per day per os for a week (under the control of urinary electrolyte excretion) in combination with conventional diuretic drugs. To inhibit the secretion of aldosterone (with edematous and edematous-hypertensive forms of chronic diffuse glomerulonephritis, cirrhosis of the liver), prednisolone is prescribed.

Aldosteronism. There are primary (Conn's syndrome) and secondary hyperaldosteronism. Primary hyperaldosteronism was described by J. Conn in 1955. In the occurrence of this clinical syndrome, the leading role belongs to the production of excess aldosterone by the adrenal cortex.

In most patients (85%), the cause of the disease is an adenoma (synonymous with "aldosteroma"), less often bilateral hyperplasia (9%) or carcinoma of the adrenal cortex of the glomerular and fascicular zones.

More often the syndrome develops in women.

Clinical picture (symptoms and signs). With the disease, periodic seizures are noted in various muscle groups with normal levels of calcium and phosphorus in the blood, but with the presence of alkalosis outside the cells and acidosis inside the cells, positive signs of Trousseau and Tail, severe headaches, sometimes bouts of muscle weakness lasting from several hours to three weeks. The development of this phenomenon is associated with hypokalemia and depletion of potassium reserves in the body.

When the disease develops arterial hypertension, polyuria, polydipsia, nocturia, a pronounced inability to concentrate urine during dry eating, resistance to antidiuretic drugs, and. The content of antidiuretic hormone is normal. There are also hypochloremia, akhiliya, alkaline reaction of urine, periodic proteinuria, lowering the level of potassium and magnesium in the blood. The sodium content increases, less often remains unchanged. Edema, as a rule, is not present. On the ECG, myocardial changes characteristic of hypokalemia (see Hegglin's syndrome).

Urinary levels of 17-hydroxycorticoids and 17-ketosteroids are normal, as is plasma ACTH.

Children with Conn's syndrome have stunted growth.

The oxygen content in the arterial blood is reduced. The content of uropepsin in patients is increased.

Diagnostic methods. Suprapneumorenoroentgenography and tomography, determination of aldosterone and potassium in urine and blood.

Treatment is surgical, adrenalectomy is performed.

The prognosis is favorable, but only until malignant hypertension develops.

Secondary hyperaldosteronism. The signs are the same as in Conn's syndrome, which develops in a number of conditions in the form of aldosterone hypersecretion in response to stimuli originating outside the adrenal glands and acting through physiological mechanisms that regulate aldosterone secretion. Lead to secondary hyperaldosteronism associated with edematous conditions: 1) congestive heart failure; 2) nephrotic syndrome; 3) cirrhosis of the liver; 4) "idiopathic" edema.

The loss of significant amounts of fluids in untreated diabetes insipidus and diabetes mellitus, nephritis with salt loss, sodium restriction in the diet, the use of diuretics, excessive physical exertion also cause secondary hyperaldosteronism.

Conn's syndrome is a disease of the endocrine system, which is characterized by a large amount of aldosterone production. In medicine, it is referred to as primary aldosteronism. This ailment can be called a consequence of the main disease, which, progressing, causes complications. The main diseases include a tumor of the adrenal glands, cancer of the adrenal glands, neoplasm of the pituitary gland, adenoma and carcinoma.

General information

Aldosteronism is divided into primary and secondary. Both types occur due to excessive production of the hormone aldosterone, which is responsible for retaining sodium in the body and excreting potassium through the kidneys. This hormone is also called the hormone of the adrenal cortex and mineralocorticoid. The most common and severe companion of this disease is arterial hypertension. Primary and secondary aldosteronism are not two stages of the same disease, but two completely different diseases. They differ in the causes of appearance, the effect on the body and, accordingly, the method of treatment.

Primary (Conn's syndrome) aldosteronism

Opened by the city of Conn in 1955. Women are 3 times more likely to suffer from aldosteronism. In the risk zone, the fair sex at the age of 25-45 years. Primary aldosteronism occurs due to neoplasms of the adrenal cortex (unilateral adenoma). Much less often, the cause is hyperplasia or cancer of the adrenal glands. With increased production of aldosterone, there is an increase in the amount of sodium in the kidneys, and potassium, in turn, decreases.

The study is carried out by a pathologist who diagnoses a tumor of the adrenal cortex. It can be single or multiple and involve one or both of the adrenal glands. In more than 95% of cases, the tumor is benign. Also, as a study, doctors often prescribe an enzyme immunoassay, where venous blood is used as a biomaterial. An ELISA is prescribed to determine the amount of aldosterone in the body and to screen for primary hyperaldosteronism.

Conn's syndrome occurs with the pathology of the adrenal glands, tumor neoplasms.

The indications for the study are most often high blood pressure, which does not return to normal during therapeutic manipulations, suspicion of the development of renal failure. Getting ready to donate blood is essential. First, limit the consumption of foods rich in carbohydrates for 2-4 weeks. Also for this period to exclude diuretics, estrogens, oral contraceptives, steroids. Stop therapy with renin inhibitors for 1 week, remove for 3 days, in extreme cases - limit moral and physical overstrain. Do not smoke for three hours before the procedure. Having the results on hand, taking into account the amount of renin, aldosterone and cortisone hormones, the attending physician will be able to correctly diagnose and prescribe an effective drug treatment.

Secondary compensatory (symptomatic)

Unlike primary aldosteronism, secondary aldosteronism is not triggered by diseases associated with the adrenal glands, but by problems with the liver, heart, and kidneys. That is, it acts as a complication of some serious diseases. At risk are patients diagnosed with:

• adrenal cancer;
• a number of heart diseases;
• abnormalities in the work of the thyroid gland, intestines;
• idiopathic hyperaldosteronism;
• adrenal adenoma.

Also to the list should be added a tendency to bleeding, prolonged exposure to medications. But this does not mean that all patients who suffer from these ailments will add the diagnosis of “secondary aldosteronism” to their medical history, they just should be more attentive to their health.

Symptoms of the disease

Persistent arterial hypertension is a characteristic symptom of Conn's syndrome.

Primary and secondary aldosteronism are associated with the manifestation of such symptoms:

1. swelling that appears due to fluid retention in the body;
2. weakening of muscle strength, fatigue;
3. frequent urination, especially at night (pollakiuria);
4. hypertension (increased blood pressure);
5. thirst;
6. vision problems;
7. feeling unwell, headache;
8. muscle paralysis for short periods of time, numbness of body parts, mild tingling;
9. an increase in the size of the ventricles of the heart;
10. rapid weight gain - more than 1 kg per day.

The evacuation of potassium contributes to the appearance of weakness in the muscles, paresthesia, sometimes muscle paralysis and many other kidney diseases. The symptoms of aldosteronism are quite dangerous, but the consequences are no less dangerous. Therefore, do not hesitate, you need to contact the doctor for help as soon as possible.

Complications and consequences

Primary aldosteronism, in case of ignoring symptoms and refusing therapy, leads to a number of complications. First of all, the heart begins to suffer (ischemia), heart failure develops, intracranial bleeding develops. In rare cases, the patient has a stroke. Since the amount of potassium in the body decreases, hypokalemia develops, which provokes arrhythmia, and that, in turn, can lead to death. Secondary hyperaldosteronism itself is a complication of other serious ailments.

Diagnosis and differential diagnosis

Urine and blood tests are prescribed for the correct and accurate diagnosis of the disease.

If the attending physician suspects aldosteronism, a number of studies and analyzes are prescribed to confirm or refute the alleged diagnosis, as well as for the correct further drug therapy. First of all, urine and blood tests are performed. The laboratory establishes or refutes the presence of polyuria or analyzes its urine density. In the blood, the concentration of aldosterone, cortisol and renin is studied. In primary hyperaldosteronism, renin is low, cortisol is normal, and aldosterone is high. For secondary aldosteronism, a somewhat different situation is inherent, the presence of renin must be significant. For more accurate results, ultrasound is often used. Rarely - MRI and CT of the adrenal glands. Additionally, the patient should be examined by a cardiologist, ophthalmologist, nephrologist.

Treatment of aldosteronism

Treatment of primary or secondary aldosteronism should be comprehensive and include not only drug therapy, but also proper nutrition, and in some cases surgical intervention. The main goal of curing Conn's syndrome is to prevent complications after The diet for Crohn's disease depends on the severity of the disease's symptoms.

In parallel, you should follow a diet. It is based on an increase in potassium-containing foods and additional potassium-containing drugs. Eliminate or limit salt intake. Foods rich in potassium include:

• dried fruits (raisins, dried apricots, prunes);
• fresh fruits (grapes, melon, apricots, plums, apples, banana);
• fresh vegetables (tomatoes, potatoes, garlic, pumpkin);
• greenery;
• meat;
• nuts;
• Black tea.

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2017

Primary hyperaldosteronism (E26.0)

Endocrinology

general information

Short description

Approved
Joint Commission on the quality of medical services
Ministry of Health of the Republic of Kazakhstan
dated August 18, 2017
Protocol No. 26

PGA- a collective diagnosis characterized by an elevated aldosterone level that is relatively autonomous from the renin-angiotensin system and does not decrease with sodium loading. An increase in aldosterone levels is the cause of cardiovascular disorders, a decrease in plasma renin levels, arterial hypertension, sodium retention, and accelerated potassium excretion, which leads to hypokalemia. Among the causes of PHA are adrenal adenoma, unilateral or bilateral adrenal hyperplasia, in rare cases, hereditary HPA.

INTRODUCTION

ICD code(s):

Date of development/revision of the protocol: 2013 (revised 2017).

Abbreviations used in the protocol:

AG	-	arterial hypertension
HELL	-	blood pressure
APA	-	aldosterone-producing adenoma
APRA	-	aldosterone-producing renin-sensitive adenoma
ACE	-	angiotensin converting enzyme
ARS	-	aldosterone-renin ratio
GZGA	-	glucocorticoid-dependent hyperaldosteronism GPHA - glucocorticoid-suppressed hyperaldosteronism
IGA	-	idiopathic hyperaldosteronism
PGA	-	primary hyperaldosteronism
PGN	-	primary adrenal hyperplasia
RCC	-	direct concentration of renin
ultrasound	-	ultrasound procedure

Protocol Users: general practitioners, endocrinologists, internists, cardiologists, surgeons and vascular surgeons.

Level of evidence scale:

BUT	High-quality meta-analysis, systematic review of RCTs, or large RCTs with a very low probability (++) of bias whose results can be generalized to the appropriate population
AT	High-quality (++) systematic review of cohort or case-control studies or high-quality (++) cohort or case-control studies with very low risk of bias or RCTs with low (+) risk of bias, the results of which can be generalized to the appropriate population
With	Cohort or case-control or controlled trial without randomization with low risk of bias (+), whose results can be generalized to the appropriate population or RCTs with very low or low risk of bias (++ or +), whose results cannot be directly distributed to the relevant population
D	Description of a case series or uncontrolled study or expert opinion
GPP	Best Clinical Practice

Classification

Etiopathogenetic and clinical and morphological signs of PHA (E. G. Biglieri, J. D. Baxter, modification).
aldosterone-producing adenoma of the adrenal cortex (APA) - aldosteroma (Conn's syndrome);
Bilateral hyperplasia or adenomatosis of the adrenal cortex:
- idiopathic hyperaldosteronism (IHA, unsuppressed hyperproduction of aldosterone);
- indefinite hyperaldosteronism (selectively suppressed production of aldosterone);
- glucocorticoid-suppressed hyperaldosteronism (GPHA);
aldosterone-producing, glucocorticoid-suppressed adenoma;
carcinoma of the adrenal cortex;
extra-adrenal hyperaldosteronism (ovaries, intestines, thyroid gland).

Diagnostics

METHODS, APPROACHES AND DIAGNOSIS PROCEDURES

Diagnostic criteria

Complaints and anamnesis

: headaches, increased blood pressure, muscle weakness, especially in the calf muscles, convulsions, paresthesia in the legs, polyuria, nocturia, polydipsia. The onset of the disease is gradual, symptoms appear after 40 years, more often diagnosed in the 3rd-4th decade of life.

Physical examination:
Hypertensive, neurological and urinary syndromes.

Laboratory research:
Determination of potassium in blood serum;
determination of the level of aldosterone in blood plasma;
Determination of the aldosterone-renin ratio (ARC).
In patients with a positive APC, one of 4 confirmatory PHA tests is recommended before differential diagnosis of PHA forms (A).

Tests Confirming PHA

Confirming PGA test	Methodology	Interpretation	Comments
sodium test load	Increase sodium intake >200 mmol (~6 g) per day for 3 days, under the control of daily sodium excretion, constant control of normokalemia while taking potassium supplements. The daily excretion of aldosterone is determined from the morning of the 3rd day of the test.	PHA is unlikely with daily aldosterone excretion of less than 10 mg or 27.7 nmol (excluding cases of chronic renal failure, in which aldosterone excretion is reduced). The diagnosis of PHA is highly probable if daily aldosterone excretion is >12 mg (>33.3 nmol) according to the Mayo Clinic and >14 mg (38.8 nmol) according to the Cleveland Clinic.	The test is contraindicated in severe forms of hypertension, chronic renal failure, heart failure, arrhythmias, or severe hypokalemia. Inconvenient collection of daily urine. Diagnostic accuracy is reduced due to laboratory problems with radioimmunoassay (18-oxo - aldosterone glucuronide, an acid-labile metabolite). HPLC tandem mass spectrometry is currently available and most preferred. In chronic renal failure, there may not be an increased release of aldosterone 18-oxoglucuronide.
Saline test	Lying position 1 hour before the start of the morning (from 8:00 - 9:30) 4-hour intravenous infusion of 2 liters of 0.9% NaCI. Blood on rhenium, aldosterone, cortisone, potassium at basal point and 4 hours later. Monitoring of blood pressure, pulse during the test.	PHA is unlikely at a post-infusion aldosterone level of 10 ng/dL. Gray zone between 5 and 10 ng/dl	The test is contraindicated in severe forms of hypertension, chronic renal failure, heart failure, arrhythmias, or severe hypokalemia.
Captopril test	Patients receive 25-50 mg of captopril orally no earlier than one hour after the morning lift. Blood sampling for ARP, aldosterone and cortisol is carried out before taking the drug and after 1-2 hours (all this while the patient is sitting	Normally, captopril reduces aldosterone levels by more than 30% of the original. In PHA, aldosterone remains elevated at low ARP. With IHA, in contrast to APA, there may be a slight decrease in aldosterone.	There are reports of a significant number of false-negative and questionable results.

Instrumental research:

Ultrasound of the adrenal glands (however, the sensitivity of this method is insufficient, especially in the case of small formations less than 1.0 cm in diameter);
CT scan of the adrenal glands (the accuracy of detecting tumor formations by this method reaches 95%). Allows you to determine the size of the tumor, shape, topical location, assess the accumulation and washout of the contrast (confirms or excludes adrenocortical cancer). Criteria: benign formations are usually homogeneous, their density is low, contours are clear;
131 I-cholesterol scintigraphy - criteria: aldosteroma is characterized by asymmetric accumulation of the radiopharmaceutical (in one adrenal gland) in contrast to bilateral diffuse small-nodular hyperplasia of the adrenal cortex;
Selective catheterization of the adrenal veins and determination of the content of aldosterone and cortisol in the blood flowing from the right and left adrenal glands (blood samples are taken from both adrenal veins, as well as from the inferior vena cava). Criteria: A five-fold increase in the aldosterone/cortisol ratio is considered confirmation of the presence of aldosteroma.

Indications for expert advice:
consultation with a cardiologist to select antihypertensive therapy;
consultation with an endocrinologist in order to choose a treatment strategy;
Consultation with a vascular surgeon to choose the method of surgical treatment.

Diagnostic algorithm:(scheme)




APC is currently the most reliable and affordable screening method for PHA. When determining APC, as with other biochemical tests, false positive and false negative results are possible. ARS is regarded as a test used in the primary diagnosis, with doubtful results due to various external influences (medication, non-compliance with blood sampling conditions). The effect of drugs and laboratory conditions on APC is shown in Table 2.

Table 2. Drugs with a minimal effect on the level of aldosterone, with the help of which we will control blood pressure in the diagnosis of PHA

medicinal group	International non-proprietary name of the drug	Mode of application	Comment
non-dihydropyridine calcium blocker channels	Verapamil, prolonged form	90-120 mg. twice a day	Used alone or with others medicines from this table
vasodilator	*Hydralazine	10-12.5 mg. twice a day with dose titration to effect	It is prescribed after verapamil, as reflex tachycardia stabilizer. Administration of low doses reduces the risk side effects (headache, tremor)
Blocker a-adreno- receptors	*Prazosin hydrochloride	0.5-1 mg two - three times a day with dose titration before effect	Postural hypotension control!

Aldosterone-renin ratio measurement:
A. Preparing for ADR determination

1. Correction of hypokalemia after measurement of plasma potassium is necessary. To exclude artifacts and overestimate the real level of potassium, blood sampling must meet the following conditions:
carried out by syringe method (undesirable with a vacutainer);
Avoid clenching your fist
draw blood not earlier than 5 seconds after the tourniquet is removed;
Separation of plasma for at least 30 minutes after collection.
2. The patient should not restrict sodium intake.
3. Cancel drugs that affect APC for at least 4 weeks:
spironolactone, triamterene;
· diuretics;
products from licorice root.
4. If the results of APC while taking the above drugs are not diagnostic, and if the control of hypertension is carried out by drugs with a minimal effect on aldosterone levels (see table 2), stop other drugs that can affect the level of APC for at least 2 weeks :
beta-blockers, central alpha-agonists (clonidine, a-methyldopa), NSAIDs;
ACE inhibitors, angiotensin receptor blockers, renin inhibitors, dihydropyridine calcium channel blockers.
5. If it is necessary to control hypertension, treatment is carried out with drugs with a minimal effect on aldosterone levels (see table 2).
6. It is necessary to have information about taking oral contraceptives (OC) and hormone replacement therapy, because. Estrogen-containing drugs can lower the level of direct renin concentration, which will cause a false positive APC result. Do not cancel OK, in this case use the ATM level, not the RCC.

B. Collection conditions:
sampling in the morning, after the patient has been in an upright position for 2 hours, after being in a sitting position for about 5-15 minutes.
Sampling in accordance with A.1, stasis and hemolysis require re-sampling.
· Before centrifugation, keep the tube at room temperature (and not on ice, because the cold regime increases the APP), after centrifugation, fast freeze the plasma component.

C. Factors affecting the interpretation of results:
age > 65 years affects the decrease in renin levels, APC is artificially overestimated;
time of day, food (salt) diet, time period of postural position;
medicines;
Violations of the method of blood sampling;
The level of potassium
creatinine levels (renal failure leads to false positive APC).

Differential Diagnosis

Differential diagnosis and rationale for additional studies

Table 3. Diagnostic tests for PHA

diagnostic test	Adrenal adenoma		adrenal hyperplasia
	APA	APRA	IGA	PGN
Orthostatic test (determination of plasma aldosterone after staying in a vertical position for 2 hours	Decrease or no change	Increase	Increase	Decrease or no change
Serum 18-hydrocorti-costerone	> 100 ng/dl	> 100 ng/dl	< 100 нг/дл	> 100 ng/dl
Excretion of 18-hydroxycortisol	> 60 mcg/day	< 60 мкг/сут	< 60 мкг/сут	> 60 mcg/day
Excretion of tetra-hydro-18-hydroxy-cortisol	> 15 mcg/day	< 15 мкг/сут	< 15 мкг/сут	< 15 мкг/сут
Computed tomography of the adrenal glands	Knot on one side	Knot on one side	Bilateral hyperplasia, ± nodes	One-sided hyperplasia, ± knots
Adrenal vein catheterization	Lateralization	Lateralization	No lateralization	No lateralization

Treatment

Drugs (active substances) used in the treatment

Groups of drugs according to ATC used in the treatment

Treatment (ambulatory)

TREATMENT TACTICS AT OUTPATIENT LEVEL: only in case of preoperative preparation (see the step-by-step management chart):
1) the appointment of an aldosterone antagonist - spironolactone at an initial dose of 50 mg 2 times a day with a further increase after 7 days to an average dose of 200 - 400 mg / day in 3 - 4 doses. With inefficiency, the dose is increased to 600 mg / day;
2) in order to reduce blood pressure to normalize the level of potassium, dihydropyridine calcium channel blockers can be prescribed at a dose of 30-90 mg / day;
3) correction of hypokalemia (potassium-sparing diuretics, potassium preparations);
4) Spironolactone is used to treat IHA. In cases of erectile dysfunction in men, it can be replaced with amiloride * at a dose of 10-30 mg / day in 2 divided doses or triamterene up to 300 mg / day in 2-4 divided doses. These drugs normalize potassium levels, but do not reduce blood pressure, and therefore the addition of saluretics, calcium antagonists, ACE inhibitors and angiotensin II antagonists is necessary;
5) in the case of HPHA, dexamethasone is prescribed in individually selected doses necessary to eliminate hypokalemia, possibly in combination with antihypertensive drugs.
* apply after registration on the territory of the Republic of Kazakhstan

Non-drug treatment:
mode: sparing mode;
< 2 г/сут.

Medical treatment(preoperative preparation)

List of Essential Medicines(having a 100% cast chance) :

medicinal group	International non-proprietary name of drugs	Indications	Level of Evidence
Aldosterone antagonists	spironolactone	preoperative preparation	BUT
calcium antagonists	nifedipine, amlodipine	reduction and correction of blood pressure	BUT
Sodium channel blockers	triamterene amiloride	potassium level correction	With

List of additional drugs (less than 100% probability of use): none.

Further management:
referral to hospital for surgical treatment.

Surgical intervention: no.

Stabilization of the level of blood pressure;
normalization of potassium levels.

Treatment (hospital)

TACTICSTREATMENT AT THE STATIONARY LEVEL

Surgery(patient routing)

Non-drug treatment:
mode: sparing mode;
diet: salt restriction< 2 г/сут.

Medical treatment:

List of essential medicines (having 100% probability of use):

List of additional medicines (less than 100% probability of use):

Further management: control of blood pressure to exclude relapses of the disease, lifelong use of antihypertensive drugs in patients with IHA and HPHA, observation by a therapist and a cardiologist.

Treatment effectiveness indicators:
Controlled blood pressure, normalization of potassium levels in the blood.

Hospitalization

INDICATIONS FOR HOSPITALIZATION WITH INDICATING THE TYPE OF HOSPITALIZATION

Indications for planned hospitalization:

for surgical treatment.

Indications for emergency hospitalization:
· hypertensive crisis/stroke;
severe hypokalemia.

Information

Sources and literature

1. Minutes of the meetings of the Joint Commission on the quality of medical services of the Ministry of Health of the Republic of Kazakhstan, 2017
   1. 1) Primary hyperaldosteronism. clinical guidelines. Endocrine Surgery No. 2 (3), 2008, pp. 6-13. 2) Clinical endocrinology. Guide / Ed. N. T. Starkova. - 3rd ed., revised. and additional - St. Petersburg: Peter, 2002. - S. 354-364. - 576 p. 3) Endocrinology. Volume 1. Diseases of the pituitary, thyroid and adrenal glands. St. Petersburg. Special Lit., 2011. 4) Endocrinology. Edited by N. Lavin. Moscow. 1999. pp. 191-204. 5) Functional and topical diagnostics in endocrinology. S.B. Shustov., Yu.Sh. Khalimov., G.E. Trufanov. Page 211-216. 6) Internal diseases. R. Harrison. Volume No6. Moscow. 2005. Pp. 519-536. 7) Endocrinology according to Williams. Diseases of the adrenal cortex and endocrine arterial hypertension. Henry M. Cronenberg, Shlomo Melmed, Kenneth S. Polonsky, P. Reed Larsen. Moscow. 2010. P. 176-194. 8) Clinical guidelines "Incidentaloma of the adrenal glands (diagnosis and differential diagnosis)". Guidelines for primary care physicians. Moscow, 2015. 9) Case Detection, Diagnosis, and Treatment of Patients with Primary Aldosteronism: An Endocrine Society Clinical Practice Guideline 10) John W. Funder, Robert M. Carey, Franco Mantero, M. Hassan Murad, Martin Reincke, Hirotaka Shibata , Michael Stowasser, William F. Young, Jr; The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2016; 101(5): 1889-1916. doi: 10.1210/jc.2015-4061 11) Parthasarathy HK , Ménard J , White WB , Young WF , Williams GH , Williams B , Ruilope LM , McInnes GT , Connell JM and MacDonald TM. A double-blind, randomized study comparing the antihypertensive effect of eplerenone and spironolactone in patients with hypertension and evidence of primary aldosteronism. Journal of hypertension, 2011, 29(5), 980 12) Mulatero P, Rabbia F, Milan A, Paglieri C, Morello F, Chiandussi L, Veglio F. Drug effects on aldosterone/plasma renin activity ratio in primary aldosteronism. hypertension. 2002 Dec;40(6):897-902. 13) Pechère-Bertschi A, Herpin D, Lefebvre H. SFE/SFHTA/AFCE consensus on primary aldosteronism, part 7: Medical treatment of primary aldosteronism. Ann Endocrinol (Paris). 2016 Jul;77(3):226-34. doi: 10.1016/j.ando.2016.01.010. Epub 2016 Jun 14.

Information

ORGANIZATIONAL ASPECTS OF THE PROTOCOL

List of protocol developers:

1) Danyarova Laura Bakhytzhanovna - Candidate of Medical Sciences, endocrinologist, head of the endocrinology department of the Republican State Enterprise on the REM "Research Institute of Cardiology and Internal Diseases".
2) Raisova Aigul Muratovna - Candidate of Medical Sciences, Head of the Therapeutic Department of the Republican State Enterprise on the Right of Economic Use "Research Institute of Cardiology and Internal Diseases".
3) Smagulova Gaziza Azhmagievna - Candidate of Medical Sciences, Head of the Department of Propaedeutics of Internal Diseases and Clinical Pharmacology of the RSE on REM "M. Ospanov West Kazakhstan State Medical University".

Indication of no conflict of interest: no.

Reviewers:
Bazarbekova Rimma Bazarbekovna - Doctor of Medical Sciences, Professor, Head of the Department of Endocrinology of JSC "Kazakh Medical University of Continuing Education".

Indication of the conditions for revising the protocol: revision of the protocol 5 years after its publication and from the date of its entry into force or in the presence of new methods with a level of evidence.

Attached files

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Primary hyperaldosteronism (Conn's syndrome)

What is Primary Hyperaldosteronism (Conn's Syndrome) -

In 1955, Conn described a syndrome accompanied by arterial hypertension and a decrease in the level of potassium in the blood, the development of which is associated with a tumor (adenoma) of the adrenal cortex that produces the hormone aldosterone. This pathology is called Conn's syndrome.

Primary hyperaldosteronism (Conn's syndrome)- a disease characterized by an increase in the secretion of aldosterone by the adrenal glands, manifested in a decrease in the activity of a specific substance - blood plasma renin - which plays an important role in regulating the functioning of the body, arterial hypertension and a decrease in the content of potassium in the blood. Later, many other cases of hyperplasia (excess tissue growth and change) of the adrenal cortex with increased secretion of aldosterone were described, and now the term "primary hyperaldosteronism" is used both to describe Conn's syndrome itself and other pathologies accompanied by hypersecretion of aldosterone, for example, cortical hyperplasia adrenal glands. Currently, primary hyperaldosteronism (PHA), and in particular Conn's syndrome, is the most common cause of secondary arterial hypertension.

What provokes / Causes of primary hyperaldosteronism (Conn's syndrome):

At the moment, two main causes of PHA, accompanied by an increase in aldosterone secretion, have been identified:

• unilateral aldosterone-producing tumor - adenoma or Conn's syndrome (50-60% of cases);
• bilateral hyperplasia of the adrenal cortex or idiopathic hyperaldosteronism (40-50% of cases).

There are rare types of diseases and tumors that are similar in symptoms, including a hereditary disease, accompanied by an increase in the concentration of aldosterone.

Even less common are aldosterone-secreting adrenocortical cancers or ovarian tumors.

The most common cause of PHA is Conn's syndrome, with the adenoma usually less than 3 cm in diameter, unilateral, and renin-independent. This means that the secretion of aldosterone is independent of changes in body position. More rarely, the adenoma may be renin-dependent (i.e., aldosterone levels increase when standing). Conn's syndrome occurs in 50-60% of cases.

The remaining 40-50% of cases are bilateral adrenal hyperplasia, when aldosterone levels increase in an upright position. Less common is primary adrenal hyperplasia, in which aldosterone levels do not depend on body position, as in renin-independent adenoma.

Aldosterone can be secreted by tumors of extra-adrenal localization - in the kidneys or ovaries.

Symptoms of primary hyperaldosteronism (Conn's syndrome):

Complaints of patients with severe hypokalemia: fatigue, muscle weakness, muscle cramps, headaches and palpitations. Such patients may also experience increased thirst: as a result, they drink a lot, and polyuria (a lot of urine) due to the so-called diabetes insipidus, which has developed as a result of hypokalemia and the corresponding changes in the kidneys due to the influence of aldosterone on them.

Relative hypocalcemia develops (decrease in blood calcium) with the development of a feeling of numbness in the limbs and around the mouth, muscle spasms in the hands and feet, and, in an extreme degree, spasm of the larynx with a feeling of suffocation and convulsions. At the same time, calcium preparations are not prescribed, since the total calcium content in the blood is normal, but due to hormonal disorders, the calcium balance in the body changes.

Long-term arterial hypertension can lead to complications from the cardiovascular and nervous systems with all the accompanying symptoms.

Diagnosis of primary hyperaldosteronism (Conn's syndrome):

There are no specific manifestations of Conn's syndrome.

When patients develop heart failure, stroke, or intracranial hemorrhage due to increased blood pressure, corresponding symptoms appear.

Laboratory research

• Studies of the content of sodium, potassium and calcium in blood plasma (biochemical analysis) can show an increase in sodium in the blood, the presence of hypokalemia and "alkalinization" of the blood, which is a consequence of the action of aldosterone on the kidneys. A relative decrease in blood calcium can also be easily detected. In almost 20% of patients, a violation of carbohydrate metabolism (increased blood glucose levels) can be detected, although diabetes rarely develops. It should be noted that a normal blood potassium level does not rule out PHA. Studies show that 7 to 38% of PHA patients have normal serum potassium levels. Hypokalemia develops when eating a significant amount of sodium.
• A decrease in the level of renin in the blood plasma in patients with PHA is characteristic, and this figure does not rise above certain values ​​​​with the introduction of diuretics or the transition to a vertical position (which usually occurs in the norm). Some experts suggest that the analysis of the level of renin in the blood plasma be considered a special test for the detection of PHA. However, according to some data, a reduced level of renin occurs in 30% of patients with hypertension. Therefore, low plasma renin levels should not be considered a specific test for PHA.
• A fairly sensitive test for PHA should be considered the ratio of plasma aldosterone activity (AAP) to plasma renin activity (ARP). Consideration should be given to possible interactions between different drugs during the test.
• With a positive test for AAP / ARP ratio, additional tests are performed: determination of the level of aldosterone in the daily portion of urine, adjusted for the level of potassium in the blood serum (since these indicators influence each other).

Instrumental examination

• Computed tomography (CT) of the abdominal cavity. It is a mandatory method of examination in the case of PHA. When PHA is diagnosed, the purpose of CT is to determine the type of pathology and the possibility of its surgical treatment (adrenal adenoma or bilateral hyperplasia). With CT, the volume of the operation is determined.
• 131-I-iodocholesterol scintigraphy is used to detect a unilateral functional (hormone-secreting) adrenal mass. However, this procedure is not widely used due to the need for careful patient preparation, high cost, and the fact that the method rarely detects a mass larger than 1.5 cm in diameter.
• Magnetic resonance imaging (MRI). It is not more sensitive than CT.

Other diagnostic methods

Postural test (transition from a horizontal to a vertical position of the body). It can be used in the clinic for the primary diagnosis of renin-dependent adrenal adenoma. Currently rarely used.

Due to the complexity of differentiating the diagnosis between adrenal hyperplasia and adenoma, after a CT scan, a procedure for taking an analysis directly from the adrenal vein can be carried out. In this case, a catheter is inserted into the adrenal vein through a vein in the thigh. Blood samples are taken from both adrenal veins, as well as from the inferior vena cava. The level of aldosterone is determined after maximum ACTH stimulation.

Treatment of primary hyperaldosteronism (Conn's syndrome):

The main goal is to prevent the occurrence of complications due to hypokalemia and arterial hypertension.

If in Conn's syndrome, hypertension is corrected with a unilateral adrenalectomy, then a bilateral lesion is most often treated conservatively, since the effectiveness of unilateral or bilateral adrenalectomy is only 19%. In the case of an adenoma, drug therapy is also carried out to control blood pressure and correct hypokalemia, which reduces the risk of subsequent surgery.

The main components of therapy:

• Sodium-restricted diet (< 2 г натрия в день), поддержание оптимальной массы тела, регулярные аэробные физические нагрузки.
• Treatment of hypokalemia and hypertension consists of prescribing potassium-sparing drugs such as spironolactone. Moreover, if hypokalemia disappears almost immediately, then 4-8 weeks of therapy may be required to reduce blood pressure. Additional administration of potassium preparations is not required. If, despite treatment, elevated blood pressure persists, second-line drugs are added to therapy.
• Second-line drugs are: diuretics, drugs that reduce blood pressure.

Surgery

Surgery is the main treatment for Conn's syndrome. Laparoscopic adrenalectomy is performed whenever possible (see below).

In patients with Conn's syndrome, an indicator of the effectiveness of a future unilateral adrenalectomy in relation to arterial hypertension is a decrease in blood pressure in response to spironolactone in the preoperative period. Spironolactone is prescribed for at least 1-2 weeks (preferably 6 weeks) prior to surgery to reduce the risk of surgery, correct hypokalemia, and control blood pressure.

It should be noted that hypertension usually does not disappear immediately after surgery. Blood pressure gradually decreases over 3-6 months. Almost all patients note a decrease in blood pressure numbers after surgery. Long-term therapeutic effect is observed after unilateral adrenalectomy for Conn's syndrome in an average of 69% of patients.

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Other diseases from the group Diseases of the endocrine system, eating disorders and metabolic disorders:

Addisonian crisis (acute adrenal insufficiency)

breast adenoma

Adiposogenital dystrophy (Perchkrantz-Babinski-Fröhlich disease)

Adrenogenital syndrome

Acromegaly

Alimentary insanity (alimentary dystrophy)

Alkalosis

Alkaptonuria

Amyloidosis (amyloid degeneration)

Amyloidosis of the stomach

Intestinal amyloidosis

Amyloidosis of the pancreatic islets

Liver amyloidosis

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Acidosis

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Wilson-Konovalov disease (hepatocerebral dystrophy)

Gaucher disease (glucocerebroside lipidosis, glucocerebrosidosis)

Itsenko-Cushing's disease

Krabbe disease (globoid cell leukodystrophy)

Niemann-Pick disease (sphingomyelinosis)

Fabry disease

Gangliosidosis GM1 type I

Gangliosidosis GM1 type II

Gangliosidosis GM1 type III

Gangliosidosis GM2

GM2 gangliosidosis type I (Tay-Sachs amaurotic idiocy, Tay-Sachs disease)

Gangliosidosis GM2 type II (Sandhoff's disease, Sandhoff's amaurotic idiocy)

Gangliosidosis GM2 juvenile

Gigantism

Hyperaldosteronism

Hyperaldosteronism secondary

Hypervitaminosis D

Hypervitaminosis A

Hypervitaminosis E

Hypervolemia

Hyperglycemic (diabetic) coma

Hyperkalemia

Hypercalcemia

Type I hyperlipoproteinemia

Hyperlipoproteinemia type II

Hyperlipoproteinemia type III

Type IV hyperlipoproteinemia

Type V hyperlipoproteinemia

Hyperosmolar coma

Hyperparathyroidism secondary

Hyperparathyroidism primary

Hyperplasia of the thymus (thymus gland)

Hyperprolactinemia

testicular hyperfunction

Hypercholesterolemia

hypovolemia

Hypoglycemic coma

hypogonadism

Hypogonadism hyperprolactinemic

Hypogonadism isolated (idiopathic)

Hypogonadism primary congenital (anorchism)

Hypogonadism, primary acquired

hypokalemia

Hypoparathyroidism

hypopituitarism

Hypothyroidism

Glycogenosis type 0 (aglycogenosis)

Glycogenosis type I (Girke's disease)

Glycogenosis type II (Pompe disease)

Glycogenosis type III (Measles disease, Forbes disease, limit dextrinosis)

Type IV glycogenosis (Andersen's disease, amylopectinosis, diffuse glycogenosis with liver cirrhosis)

Glycogenosis type IX (Hag's disease)

Type V glycogenosis (McArdle disease, myophosphorylase deficiency)

Type VI glycogenosis (Hers disease, hepatophosphorylase deficiency)

Type VII glycogenosis (Tarui disease, myophosphofructokinase deficiency)

Glycogenosis type VIII (Thomson's disease)

Glycogenosis type XI

Type X glycogenosis

Deficiency (insufficiency) of vanadium

Deficiency (insufficiency) of magnesium

Deficiency (insufficiency) of manganese

Deficiency (insufficiency) of copper

Deficiency (insufficiency) of molybdenum

Deficiency (insufficiency) of chromium

iron deficiency

Calcium deficiency (alimentary calcium deficiency)

Zinc deficiency (alimentary zinc deficiency)

diabetic ketoacidotic coma

Ovarian dysfunction

Diffuse (endemic) goiter

Delayed puberty

Excess estrogen

Involution of mammary glands

Dwarfism (short stature)

Kwashiorkor

Cystic mastopathy

xanthinuria

Lactic coma

Leucinosis (Maple Syrup Disease)

Lipidoses

Farber's lipogranulomatosis

Lipodystrophy (fatty degeneration)

Generalized congenital lipodystrophy (Sape-Lawrence syndrome)

Lipodystrophy hypermuscular

Lipodystrophy post-injection

Lipodystrophy progressive segmental

Lipomatosis

Lipomatosis painful

Metachromatic leukodystrophy

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

Primary hyperaldosteronism is a consequence of primary excessive 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 outside the adrenal glands. In addition, there is a group of diseases that are characterized by similar symptoms, not accompanied by an increased level of aldosterone (syndromes that mimic hyperaldosteronism).

Primary hyperaldosteronism, first described by Conn in 1956, is in most cases the result of autonomous solitary aldosterone-producing adrenal adenoma ( Conn's syndrome), less often - 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 is more common 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 due to hyperplasia of the glomerular zone of the adrenal cortex is characterized by the preservation of sensitivity to stimulation by angiotensin II.

In addition, familial hyperaldosteronism is isolated, suppressed by glucocorticoids and with preserved sensitivity to pituitary ACTH (type I familial hyperaldosteronism), which develops as a result of 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 glomeruli, 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 an increase in potassium excretion 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 noted, 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 hypostenuria 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. It is fundamental that, despite sodium and fluid retention, edema (escape phenomenon) does not develop in primary hyperaldosteronism, which is explained by an increase in cardiac output, arterial hypertension and hypertensive diuresis.

Long-term presence of hyperaldosteronism is accompanied by complications caused by arterial hypertension (myocardial infarction, stroke) and specific myocardial hypertrophy. As mentioned above, the 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 medium therapeutic doses, hypertensive crises can be provoked by thiazide or loop diuretics and be accompanied by cardiac or cerebral symptoms.

An increase in blood pressure in combination with hypokalemia causes electrocardiographic disorders: a flattening or inversion of the T wave appears, a decrease in the ST segment, a Q-T interval lengthens, a pronounced U wave (wave) appears. Cardiac arrhythmias and extrasystoles and signs of left ventricular hypertrophy are recorded. With primary hyperaldosteronism, edema is absent, while with secondary 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 dramatically with exercise or suddenly (for no reason). At the same time, the degree of weakness at the time of the attack limits the possibilities of movement or minimal physical work. Paresthesia, local convulsions are possible.

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

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

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

Diagnostics

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

The following indicators serve as evidence of primary hyperaldosteronism:

1. serum potassium level
2. the level of renin is reduced (plasma renin activity);
3. the level of aldosterone in the blood is increased;
4. daily excretion of aldosterone metabolites in the urine (aldosterone-18-glucoronite) is increased.

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

1. 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;
2. test with spironolactone: within 3 days the patient receives 400 mg / day of spironolactone orally. An increase in the level of potassium by more than 1 mmol / l confirms hyperaldosteronism;
3. test with furosemide: 0.08 g of furosemide is administered orally to the patient. After 3 hours, there is a decrease in plasma renin activity and an increase in the level of aldosterone with hyperaldosteronism;
4. test with 9α-fluorocortisol: for 3 days, the patient receives orally 400 mcg / day of 9α-fluorocortisol (cortinef) and examines the level of aldosterone before and after the test. With bilateral hyperplasia of the glomerular layer of the adrenal cortex, a decrease in the level of aldosterone is noted, and with aldosteroma, there is no decrease in the level of aldosterone:
5. test with dexamethasone: used to differentiate glucocorticoid-suppressed hyperaldosteronism, the appointment of 0.5 - 1.0 mg 2 r / day for a week leads to a decrease in the manifestations of the disease;
6. orthostatic test (allows you to differentiate primary hyperaldosteronism with unilateral aldosteroma and bilateral adrenal hyperplasia): after a 3-4-hour stay of the patient in an upright position (standing, walking), aldosterone levels and plasma renin activity are assessed. With autonomous aldosterone, plasma renin activity does not change (it remains low), and the level of aldosterone decreases or changes slightly (normally, plasma renin and aldosterone activity increase by 30% above basal values).

Indirect signs of hyperaldosteronism:

• hypernatremia;
• hyperkaliuria, hypokalemia;
• polyuria, iso- and hypostenuria;
• metabolic alkalosis and increased serum bicarbonate levels (resulting from the loss of hydrogen ions in the urine and impaired bicarbonate reabsorption), as well as an alkaline urine reaction;
• 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 renin secretion (low plasma renin activity) without a tendency to increase adequately in conditions of volume reduction (orthostasis, sodium restriction);
• hypersecretion of aldosterone that is not sufficiently reduced under conditions of volume expansion (salt loading).

As mentioned above, the cause of primary hyperaldosteronism can be established during certain functional tests (orthostatic test, test with 9α-fluorocortisol). In addition, in familial hyperaldosteronism, suppressed by glucocorticoids and with preserved sensitivity to pituitary ACTH (type I familial hyperaldosteronism) and bilateral adrenal hyperplasia, there is an increase in the levels of the precursor in the synthesis of aldosterone - 18-hydroxycorticosterone > 50 - 100 ng / dl and an increase in excretion with 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, an additional examination is carried out aimed at clarifying the nosological form of primary hyperaldosteronism and topical diagnosis. The first step is to visualize the area of ​​the adrenal glands. The preferred methods are CG, MRI and PET. Revealed bilateral symmetrical pathology or unilateral volumetric formation in the adrenal gland allows you to establish the cause of primary hyperaldosteronism. It should be remembered that the visualization of the adrenal glands is only important in view of the identified metabolic disorders.

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

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

Syndromes that mimic primary hyperaldosteronism include a number of diseases characterized by arterial hypertension and a myopathic syndrome caused by hypochloremic alkalosis and a low level of renin (pseudohyperaldosteronism), are rare and are caused by various fermentopathies. At the same time, 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 manifest themselves 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.

If aldosteroma is detected, the only treatment 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, replacement therapy with glucocorticosteroids is not indicated in the vast majority of cases. After removal of the adenoma, the cure for hypertension is observed in 55-60% of patients. However, arterial hypertension may persist in approximately 30% of operated patients.

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

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