Primary hyperaldosteronism

• Excessive secretion of aldosterone by the adrenal cortex.

Adrenal cortex in primary hyperaldosteronism

Etiology

• 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 this 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 reninoma can form. Its cells produce more renin and this leads to an increase in the production of aldosterone in the kidneys.

Primary hyperaldosteronism:

Reninoma:

Clinical picture

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 ).

Aldosterone

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 a reduced concentration ability of the kidneys manifested by nocturia) to life-threatening heart rhythm disorders.

So the patient has: hypertension, hypokalaemia, hypernatremia.

Diagnostics

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

Imaging methods

• ultrasound of the kidneys (reninoma) and adrenal glands (tumor, bilateral hyperplasia)
• CT, MRI of the adrenal glands

Laboratory examination

• increased level of 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 the aldosterone level is high but the renin level is low, it means that the problem is in the adrenal glands
• examination of plasma renin activity
• stimulation tests (physical stress, furosemide)

Treatment

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

Links

Related articles

• Dysbalance natria
• Diabetes insipidus
• Addison´s choroba

Source

• ŠTEFÁNEK, Jiří. Medicína, nemoci, studium na 1. LF UK [online]. ©2011. [cit. 14. 4. 2011]. <https://www.stefajir.cz/?q=connuv-syndrom>.

References

• NEČAS, Emanuel. Patologická fyziologie orgánových systémů. Čast 2. 2. edition. Karolinum, 2009. 760 pp. pp. 582. ISBN 978-80-246-1712-1.

• ČEŠKA, Richard – TESAŘ, Vladimír, et al. Interna. 132. edition. Triton, 2012. 855 pp. pp. 153. ISBN 9788073876296.