Giperaldosteronizm – the pathological state caused by the raised production of an aldosteron - the main mineralokortikoidny hormone of bark of adrenal glands. At primary giperaldosteronizm arterial hypertension, headaches, a kardialgiya and violation of a warm rhythm, deterioration in sight, muscular weakness, paresteziya, spasms is observed. At a secondary giperaldosteronizm peripheral hypostases, a chronic renal failure, changes of an eye bottom develop. Diagnostics of various types of a giperaldosteronizm includes biochemical blood test and urine, functional load tests, ultrasonography, a stsintigrafiya, MRT, a selective venografiya, a research of a condition of heart, liver, kidneys and renal arteries. Treatment of a giperaldosteronizm at an aldosteroma, cancer of adrenal glands, a reninoma of kidneys – quick, at other forms – medicamentous.
Giperaldosteronizm includes the whole complex various on pathogenesis, but relatives on clinical signs of the syndromes proceeding with excess secretion of an aldosteron. Giperaldosteronizm can be primary (caused by pathology of adrenal glands) and secondary (caused by hyper secretion of a renin at other diseases). Primary giperaldosteronizm is diagnosed for 1-2% of patients with symptomatic arterial hypertension. In endocrinology of 60 — 70% of patients with primary giperaldosteronizm women at the age of 30 — 50 years make; not numerous cases of identification of a giperaldosteronizm among children are described.
Depending on an etiologichesky factor distinguish several forms of primary giperaldosteronizm from which 60-70% of cases are the share of a syndrome of Conn which reason the aldosteroma - aldosteronprodutsiruyushchy adenoma of bark of adrenal glands is. Existence of a bilateral diffusion and nodular giperplaziya of bark of adrenal glands leads to development of an idiopathic giperaldosteronizm.
There is a rare family form of primary giperaldosteronizm with autosomno-prepotent type of inheritance caused by defect of the enzyme 18 of a hydroxylase getting out of hand a renin-angiotenzinovoy of system and korrigiruyemy glucocorticoids (occurs at patients of young age with frequent cases of arterial hypertension in the family anamnesis). In rare instances primary giperaldosteronizm can be caused by the adrenal gland cancer capable to produce and .
The secondary giperaldosteronizm arises as a complication of a number of diseases of cardiovascular system, pathology of a liver and kidneys. The secondary giperaldosteronizm is observed at heart failure, a malignant arterial hypertension, cirrhosis, a syndrome of Barter, a dysplasia and a stenosis of renal arteries, a nephrotic syndrome, a reninoma of kidneys and a renal failure.
Sodium loss (at a diet, diarrhea) leads reduction of volume of the circulating blood to strengthening of secretion of a renin and development of a secondary giperaldosteronizm, at blood loss and dehydration, potassium overconsumption, long reception of some medicines (diuretics, the COOK, laxatives). Psevdogiperaldosteronizm develops at violation of reaction of disteel kidney tubules on when, despite its high level in blood serum, the giperkaliyemiya is observed. The Vnenadpochechnikovy giperaldosteronizm is noted rather seldom, for example, at pathology of ovaries, a thyroid gland and intestines.
Pathogenesis of a giperaldosteronizm
Primary giperaldosteronizm (nizkoreninovy) is usually connected with tumoral or hyper plastic defeat of bark of adrenal glands and is characterized by a combination of the increased secretion of an aldosteron to a gipokaliyemiya and arterial hypertension.
The basis of pathogenesis of primary giperaldosteronizm is made by influence of surplus of an aldosteron on water and electrolytic balance: increase in a reabsorption of ions of sodium and water in kidney tubules and the strengthened removal of ions of potassium with urine leading to a delay of liquid and a gipervolemiya, a metabolic alkaloz, decrease in development and activity of a renin of plasma of blood. Violation of haemo dynamics - increase in sensitivity of a vascular wall to action of endogenous pressor factors and resistance of peripheral vessels to current of blood is noted. At primary giperaldosteronizm the expressed and long gipokaliyemichesky syndrome leads to dystrophic changes in kidney tubules (a kaliyepenichesky nephropathy) and muscles.
The secondary (vysokoreninovy) giperaldosteronizm arises kompensatorno, in response to decrease in volume of a kidney blood-groove at various diseases of kidneys, a liver, hearts. The secondary giperaldosteronizm develops due to activation a renin-angiotenzinovoy of system and strengthening of production of a renin cages of the yukstaglomerulyarny device of the kidneys rendering excess stimulation of bark of adrenal glands. The characteristic of primary giperaldosteronizm expressed electrolytic violations at a secondary form do not arise.
The clinical picture of primary giperaldosteronizm reflects the violations of water and electrolytic balance caused by hyper secretion of an aldosteron. Owing to a delay of sodium and water patients with primary giperaldosteronizm have an expressed or moderate arterial hypertension, headaches, the aching pains in heart (kardialgiya), violations of a warm rhythm, change of an eye bottom with deterioration in visual function (a hypertensive angiopatiya, , a retinopathy).
Deficiency of potassium leads to emergence of bystry fatigue, muscular weakness, paresteziya, attacks of spasms in various groups of muscles, periodic pseudo-paralyzes; in hard cases – to development of dystrophy of a myocardium, a kaliyepenichesky nephropathy, nefrogenny not diabetes. At primary giperaldosteronizm in the absence of heart failure peripheral hypostases are not observed.
At a secondary giperaldosteronizm the high level of arterial pressure (with diastolic HELL> 120 mm Hg) which is gradually leading to defeat of a vascular wall and ischemia of fabrics, deterioration in function of kidneys and development of HPN, changes of an eye bottom (hemorrhages, a neuroretinopathy) is observed. The most frequent sign of a secondary giperaldosteronizm are hypostases, the gipokaliyemiya meets in rare instances. The secondary giperaldosteronizm can proceed without arterial hypertension (for example, at a syndrome of Barter and a psevdogiperaldosteronizm). At some patients the malosimptomny current of a giperaldosteronizm is observed.
Diagnostics of a giperaldosteronizm
Diagnostics provides differentiation of various forms of a giperaldosteronizm and definition of their etiology. Within initial diagnostics the analysis of a functional state system renin-angiotensin-aldosteronovoy with definition of an aldosteron and renin in blood and urine at rest and after load tests, potassium - the sodium balance and AKTG regulating secretion of an aldosteron is carried out.
Increase in level of an aldosteron in blood serum, decrease of the activity of a renin of plasma (ARP), a high ratio / a renin, a gipokaliyemiya and a gipernatriyemiya, low relative density of urine, considerable strengthening of a daily ekskretion of potassium and an aldosteron with urine is characteristic of primary giperaldosteronizm. The main diagnostic criterion of a secondary giperaldosteronizm is the raised ARP indicator (at a reninoma - more than 20-30 ng/ml/h).
For the purpose of differentiation of separate forms of a giperaldosteronizm carry out test with spironolaktony, test with loading a hydrochlorothiazide, "mid-flight" test. For the purpose of identification of a family form of a giperaldosteronizm carry out genomic typing by the PTsR method. At a giperaldosteronizm, korrigiruyemy glucocorticoids, diagnostic value has trial treatment by dexamethasone (Prednisolonum) at which displays of a disease are eliminated, and arterial pressure is normalized.
For clarification of the nature of defeat (an aldosteroma, a diffusion and nodular giperplaziya, cancer) use methods of topichesky diagnostics: Ultrasonography of adrenal glands, a stsintigrafiya, KT and MRT of adrenal glands, a selective venografiya with simultaneous determination of levels of an aldosteron and cortisol in blood of nadpochechnikovy veins. It is also important to establish the disease which caused development of a secondary giperaldosteronizm by means of researches of a condition of heart, liver, kidneys and renal arteries (EhoKG, the ECG, ultrasonography of a liver, ultrasonography of kidneys, UZDG and duplex scanning of renal arteries, multispiral KT, the MR-angiography).
Treatment of a giperaldosteronizm
The choice of a method and tactics of treatment of a giperaldosteronizm depends on the reason of hyper secretion of an aldosteron. Examination of patients is conducted by the endocrinologist, the cardiologist, the nephrologist, the ophthalmologist. Drug treatment by kaliysberegayushchy diuretics (spirolactone) is carried out at different forms of a giporeninemichesky giperaldosteronizm (a giperplaziya of bark of adrenal glands, an aldosteroma) as a preparatory stage to operation that contributes to normalization of arterial pressure and elimination of a gipokaliyemiya. The low-salt diet with the increased contents in a diet of the products rich with potassium, and also introduction of medicines of potassium is shown.
Treatment of an aldosteroma and cancer of adrenal glands – quick, consists the affected adrenal gland (adrenalektomiya) with preliminary restoration of water and electrolytic balance at a distance. Patients from bilateral giperplaziy bark of adrenal glands are usually treated conservatively () in a combination with APF inhibitors, antagonists of calcic channels (nifedipine). At hyper plastic forms of a giperaldosteronizm the full bilateral adrenalektomiya and a right-hand adrenalektomiya in combination with a subtotal resection of the left adrenal gland are ineffective. Gipokaliyemiya disappears, but there is no desirable hypotensive effect (HELL is normalized only in 18% of cases) and there is a high risk of development of sharp nadpochechnikovy insufficiency.
At the giperaldosteronizm which is giving in to correction of glucocorticoid therapy for elimination of hormonal and metabolic violations and normalization HELL is appointed by a hydrocortisone or dexamethasone. At a secondary giperaldosteronizm the combined antigipertenzivny therapy is carried out against the background of pathogenetic treatment of the main disease under obligatory control of the ECG and level of potassium in blood plasma.
In case of a secondary giperaldosteronizm owing to a stenosis of renal arteries for normalization of blood circulation and functioning of a kidney carrying out chreskozhny X-ray endovascular balloon dilatation, stenting of the affected renal artery open for reconstructive operation is possible. At identification of a reninoma of a kidney surgical treatment is shown.
Forecast and prevention of a giperaldosteronizm
The forecast of a giperaldosteronizm depends on weight of a disease prime cause, extent of damage of a cardiovascular and urinary system, timeliness and treatment. Radical expeditious treatment or adequate medicamentous therapy provide high probability of recovery. At adrenal gland cancer the forecast adverse.
For the purpose of prevention of a giperaldosteronizm constant dispensary observation of persons with arterial hypertension, diseases of a liver and kidneys is necessary; observance of medical recommendations concerning reception of medicines and character of food.