Primární hyperaldosteronismus

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Etiology[edit | edit source]

  • 50-60% - bilateral hyperplasia of the zona glomerulosa (idiopathic hyperaldosteronism)
  • 35-40% - Conn's syndrome - a label for a unilateral aldosterone-producing adenoma
  • 5-8% - unilateral hyperplasia
  • rare - familial hyperaldosteronism type I - dexamethasone suppressible (DSH) - there is a fusion of the regulatory part of the 11beta-hydroxylase gene with the coding part of the aldosterone synthase gene; the resulting chimeric gene produces large amounts of aldosterone, but it is under the control of ACTH.
  • rare – carcinoma of the adrenal cortex

A condition similar to primary hyperaldosteronism can also be caused by the kidney, where a benign tumor called reninoma can develop. Its cells produce more renin and this leads to an increase in the production of aldosterone in the kidneys.

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Clinical[edit | edit source]

Regardless of the cause, the symptoms of the disease are the same – a high level of aldosterone has an effect on reduced excretion of sodium and, conversely, increased excretion of potassium by the kidneys. Sodium accumulates in the body, which leads to an increase in the volume of extracellular fluid (including plasma). The increase in fluid in the blood vessels leads to an increase in blood pressure with all the manifestations and complications (headaches, fatigue, nosebleeds). Long-term elevated blood pressure can be the cause of heart failure. A reduced potassium level can manifest itself in any way - from constipation, through muscle weakness, hypokalemic nephropathy (with reduced concentration ability of the kidneys manifested by nocturia) to life-threatening heart rhythm disorders.

The patient therefore has: hypertension, hypokalemia, hypernatremia.

Diagnostics[edit | edit source]

Conn's syndrome must be considered in arterial hypertension that does not respond well to treatment.

Medical images
  • ultrasound of the kidneys (reninoma) and adrenal glands (tumor, bilateral hyperplasia)
  • CT, MRI of the adrenal glands
Laboratory examination
  • increased Na+ and decreased K+ in the blood
  • hormone levels:
    • if both aldosterone and renin are high, it means that the cause of the high aldosterone is in the kidneys
    • if aldosterone is high but renin is low, it means the problem is in the adrenal glands
  • examination of plasma renin activity
  • stimulation tests (physical stress, furosemide)

Therapy[edit | edit source]

We choose the treatment procedure according to the cause.

  • adrenal tumors − surgical removal (adrenalectomy)
  • bilateral hyperplasia of the adrenal glands − conservative therapy: pharmacotherapy (spironolactone and eplerenone, which dampen the effect of aldosterone) − bilateral surgical removal of the adrenal glands is not suitable due to the great importance of their hormones.
  • familial hyperaldosteronism type I - small doses of ACTH-suppressing glucocorticoids


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Kategorie:Patofyziologie Kategorie:Endokrinologie Kategorie:Vnitřní lékařství