Primary hyperaldosteronism: a review of information
Last reviewed: 23.04.2024
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Primary aldosteronism (Connes syndrome) is aldosteronism caused by the autonomous production of aldosterone by the adrenal cortex (due to hyperplasia, adenoma or carcinoma). Symptoms and symptoms include episodic weakness, increased blood pressure, hypokalemia. Diagnosis includes the determination of plasma aldosterone level and plasma renin activity. Treatment depends on the cause. The tumor is removed as far as possible; when hyperplasia spironolactone or close drugs can normalize blood pressure and cause the disappearance of other clinical manifestations.
Aldosterone is the most potent mineralocorticoid, produced by the adrenal glands. It regulates sodium retention and potassium loss. In kidneys, aldosterone causes the transfer of sodium from the lumen of the distal tubules into the tubular cells in exchange for potassium and hydrogen. The same effect is observed in the salivary, sweat glands, cells of the intestinal mucosa, the exchange between the inside and extracellular fluid.
The secretion of aldosterone is regulated by the renin-angiotensin system and to a lesser extent - ACTH. Renin, a proteolytic enzyme, accumulates in juxtaglomerular kidney cells. Reduction of the volume and velocity of blood flow in the afferent renal arterioles induces the secretion of renin. Renin converts the angiotensinogen of the liver into angiotensin I, which by means of an angiotensin-converting enzyme is transformed into angiotensin II. Angiotensin II causes the secretion of aldosterone and to a lesser extent the secretion of cortisol and deoxycorticosterone, which also have pressor activity. Delays in sodium and water caused by increased secretion of aldosterone, increase the volume of circulating blood and reduce the release of renin.
The syndrome of primary hyperaldosteronism was described by J. Conn (1955) in connection with the aldosterone-producing adenoma of the adrenal cortex (aldosterome), the removal of which led to complete recovery of the patient. At the present time, the collective concept of primary hyperaldosteronism combines a number of closely related clinical and biochemical features, but pathogenesis-different diseases based on excessive and independent (or partially dependent) renin-angiotensin production of aldosterone by the adrenal cortex.
[What causes primary aldosteronism?
Primary aldosteronism can be caused by an adenoma, usually one-sided, cells of the glomerular layer of the adrenal cortex, or less commonly by carcinoma or adrenal hyperplasia. With hyperplasia of the adrenal gland, which is more common in older men, both adrenal glands are hyperactive, there is no adenoma. The clinical picture can also be observed with congenital adrenal hyperplasia due to a deficiency of 11-hydroxylase and with dominantly inherited dexamethasone-suppressed hyperaldosteronism.
Symptoms of primary aldosteronism
There may be hypernatremia, hypervolemia and hypokalemic alkalosis causing episodic weakness, paresthesia, transient paralysis and tetany. Often there is diastolic hypertension, hypokalemic nephropathy with polyuria and polydipsia. In many cases, the only manifestation is hypertension, from mild to moderate. Edema is not characteristic.
Symptoms of primary hyperaldosteronism
Clinical case of primary hyperaldosteronism
Patient M., female, 43 years old, entered the endocrinology department of the RCB of Kazan on January 31, 1212 with complaints of headaches, dizziness when lifting blood pressure, maximal up to 200/100 mm Hg. Art. (with a comfortable blood pressure of 150/90 mm Hg), generalized muscle weakness, leg cramps, general weakness, rapid fatigue.
Anamnesis of the disease. The disease developed gradually. Within five years, the patient notes an increase in blood pressure, which was observed by the therapist at the place of residence, received antihypertensive therapy (enalapril). About 3 years ago, began to worry about periodic pain in the legs, convulsions, muscle weakness, arising without visible provoking factors, passing independently for 2-3 weeks. Since 2009, 6 times received inpatient treatment in neurological departments of various health facilities with the diagnosis: Chronic demyelinating polyneuropathy, subacute developing generalized muscle weakness. One episode was with the weakness of the neck muscles and the dangling of the head.
Against the background of infusion of prednisolone and a polarizing mixture, the improvement occurred within a few days. According to the blood test, potassium is 2.15 mmol / l.
From 26.12.11 to 25.01.12 she was on inpatient treatment in the RCS, where she received complaints with generalized muscle weakness, periodic cramps in her legs. A blood test was performed on 27.12.11: ALT - 29 ED / L, AST - 14 U / L, creatinine - 53 μmol / L, potassium 2.8 mmol / L, urea - 4.3 mmol / L, Society. Protein 60 g / l, bilirubin total. - 14,7 μmol / l, CKF - 44,5, LDH - 194, phosphorus 1,27 mmol / l, Calcium - 2,28 mmol / l.
Urinalysis from 27.12.11; 1002 weight, protein - traces, leukocytes - 9-10 in p / z, epit. Pl - 20-22 in p / z.
Hormones in the blood: T3cv - 4.8, T4 - 13.8, TTG - 1.1 μmE / l, cortisol - 362.2 (norm 230-750 nmol / l).
US: Kidney lion: 97x46 mm, parenchyma 15 mm, echogenicity increased, CML-20 mm. Echogenicity increased. The cavity is not expanded. Right 98x40 mm. Parenchyma 16 mm, echogenicity increased, CHLS 17 mm. Echogenicity increased. The cavity is not expanded. Around the pyramids on both sides a hyperechoic rim is visualized. On the basis of physical examination and laboratory data for the elimination of endocrine pathology of adrenal origin, further examination was recommended.
US of adrenal glands: in the projection of the left adrenal isoechoic round formation is visualized 23x19 mm. In the projection of the right adrenal gland pathological formations is not reliably visualized.
Urine on catecholamines: Diuresis - 2,2 l, epinephrine - 43,1 nmol / day (norm 30-80 nmol / day), noradrenaline - 127,6 nmol / l (norm 20-240 nmol / day). These results excluded the presence of pheochromocytoma as a possible cause of uncontrolled hypertension. Renin from 13.01.12-1.2 μIU / ml (N vert-4,4-46,1 ;, horiz 2,8-39,9), aldosterone 1102 pg / ml (norm: lying 8-172, sitting 30 -355).
RKT from 01/18/12: RCC signs of the formation of the left adrenal gland (in the medial pedicle of the left adrenal gland, the isodent formation of an oval shape measuring 25 * 22 * 18 mm, homogeneous, density 47 NU is determined.
Based on anamnesis, clinical picture, laboratory and instrumental research methods, a clinical diagnosis was established: Primary hyperaldosteronism (left adrenal aldosteroma), first identified as hypokalemic syndrome, neurologic symptoms, sinus tachycardia. Hypokalemic periodic cramps with generalized muscle weakness. Hypertensive disease 3 degrees, 1 stage. CHF 0. Sinus tachycardia. Urinary tract infection in the resolution stage.
The syndrome of hyperaldosteronism occurs with clinical manifestations, caused by three main symptom-complexes: arterial hypertension, which can have both a crisis current (up to 50%) and persistent; violation of neuromuscular conduction and excitability, which is associated with hypokalemia (in 35-75% of cases); impaired renal tubule function (50-70% of cases).
The patient was recommended surgical treatment to remove the hormone-producing tumor of the adrenal gland - laparoscopic adrenalectomy on the left. The operation was performed - laparoscopic adrenalectomy on the left in the conditions of the abdominal surgery department of the RCB. The postoperative period was uneventful. On the 4th day after the operation (11.02.12), the blood potassium level was 4.5 mmol / l. Blood pressure 130/80 mm Hg. Art.
Secondary aldosteronism
Secondary aldosteronism is an increased production of adrenal aldosterone in response to non-hypophyseal, extraadrenal stimuli, including renal artery stenosis and hypovolemia. Symptoms are similar to those of primary aldosteronism. Treatment includes correction of the cause.
Secondary aldosteronism is caused by a decrease in renal blood flow, which stimulates the renin-angiotensin mechanism with the resulting hypersecretion of aldosterone. The causes of decreased renal blood flow include obstructive diseases of the renal artery (for example, atheroma, stenosis), renal vasoconstriction (with malignant hypertension), diseases accompanied by edema (eg, heart failure, cirrhosis with ascites, nephrotic syndrome). Secretion can be normal with heart failure, but hepatic blood flow and aldosterone metabolism are reduced, so the levels of circulating hormone are high.
Diagnosis of primary aldosteronism
The diagnosis is suspected in patients with hypertension and hypokalemia. A laboratory study consists of determining the level of plasma aldosterone and plasma renin activity (ARP). Tests should be conducted if the patient refuses from drugs that affect the renin-angiotensin system (eg, thiazide diuretics, ACE inhibitors, angiotensin antagonists, blockers) for 4-6 weeks. ARP is usually measured in the morning in the supine position of the patient. Usually, in patients with primary aldosteronism, the plasma aldosterone level is greater than 15 ng / dl (> 0.42 nmol / L) and low levels of ARP, with a plasma aldosterone ratio (in nanograms / dL) to APP [in nanograms / (mlhh)] greater than 20 .
Low levels of ARP and aldosterone indicate a non-aldosterone excess of mineralocorticoids (for example, due to licorice, Cushing's syndrome, Liddle syndrome). High levels of ARP and aldosterone indicate secondary hyperaldosteronism. In children, the syndrome of Barter is different from the primary hyperaldosteronism in the absence of hypertension and increased renin.
Patients with results of studies suggesting primary hyperaldosteronism should undergo CT or MRI to find out what is the cause: a tumor or hyperplasia. The levels of aldosterone measured in the morning when the patient woke up, and after 2-4 hours, standing, can also help in differentiation: with adenoma levels decrease, and with hyperplasia - increase. In controversial cases, bilateral adrenal vein catheterization is performed to measure cortisol and aldosterone levels. With one-sided excess - a tumor, with bilateral - hyperplasia.
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Treatment of primary aldosteronism
Tumors can be removed laparoscopically. After removal of the adenoma, blood pressure decreases in all patients; complete remission is observed in 50-70%. With hyperplasia of the adrenal glands, 70% have hypertension after bilateral adrenalectomy; therefore, surgical treatment is not recommended. Hyperaldosteronism in these patients can be controlled by spironolactone, starting from 300 mg orally once a day and decreasing to a maintenance dose, usually about 100 mg once a day for more than 1 month; or amiloride (5-10 mg) or other K-saving diuretics. About half of these patients need additional antihypertensive therapy.