Primary aldosteronism (Conn’s syndrome)
Primary aldosteronism, or Conn’s syndrome, is a disease caused by excessive production of aldosterone by the tumor of the adrenal cortex. The disease is relatively rare, occurs more often in women aged 20-50 years.
Historical data. The disease was first described in 1955 Conn.
Etiology. Conn’s syndrome is most often caused by a hormone-active tumor of the glomerular zone of the adrenal cortex (aldosteroma), much less often by its bilateral hyperplasia.
The biosynthesis of aldosterone in the tumor is increased by 40 – 100 times, cortisol by 2-5 times and corticosterone – by 2-4 times. An increase in cortisol and corticosterone biosynthesis in a tumor is associated with heterogeneity of adenomas, which in some cases have cells similar to those of the puchal zone, and in others, resemble cells of the reticular zone. At the same time, in case of primary aldosteronism, not only the glomerular, but also the reticular zone is enlarged.
Pathogenesis. Due to increased production of aldosterone, there is an increase in sodium reabsorption in the tubules of the kidneys and an increase in the excretion of potassium and hydrogen ions with urine.
Depletion of the body with potassium leads to the development of muscle weakness, paresthesia, transient muscle paralysis, as well as renal symptoms (polyuria, polydipsia, nocturia, etc.) – Polyuria is caused by dystrophic changes in the renal tubules, as a result of which they lose the ability to respond to Tb on antidiuretic hormone. As a result of hypokalemia, intracellular potassium is replaced by sodium and hydrogen ions, and cTo leads to intracellular acidosis and extracellular alkalosis. This in turn causes tetany. Delay in
Ganism of sodium and water causes hypervolemia, which leads to arterial hypertension and related symptoms (headache, altered fundus, left ventricular hypertrophy of the heart, etc.).
Increased excretion of potassium in the urine leads to hypocaliemic alkalosis and hypokalemic tubular nephropathy.
Pathological anatomy. In the post-mortem examination, single or multiple tumors of the cortex of one and, more rarely, of both adrenal glands are found most often. The tumor is usually benign, in 5% of cases it can be malignant. Adenoma is often small in size, on the cut yellow or orange, sometimes with a grayish tint. Histologically, tumors most often reveal cells of both the glomerular and puchkovy zones of the adrenal cortex.
In 9% of autopsy cases, only bilateral diffuse hyperplasia of the adrenal cortex is found. Histologically marked thickening of the glomerular zone of the adrenal cortex. In rare cases, pathological changes in the adrenal cortex are absent. Hydropic and fatty degeneration of tubule cells and thickening of the basement membrane are often found in the kidneys. Dystrophic processes are found mainly in the proximal tubules and less frequently in the distal ones. Sometimes there is hyalinization of the glomeruli, sclerosis of the renal arterioles, pyelonephritis. A number of patients have focal necrosis of the heart muscle and skeletal muscles.