Primary hyperaldosteronism: Difference between revisions
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== Etiology ==
== Etiology ==
[[File:Adrenal gland Conn syndrome4.jpg|thumb|242x242px|Adrenal cortex in primary hyperaldosteronism]]
[[File:Adrenal gland Conn syndrome4.jpg|thumb|242x242px|Adrenal cortex in primary hyperaldosteronism]]
* 50–60% – bilateral hyperplasia of the zona glomerulosa (idiopathic hyperaldosteronism)
* '''50–60%''' – bilateral hyperplasia of the zona glomerulosa (idiopathic hyperaldosteronism)
* 35–40% – '''Conn's syndrome''' – a label for a unilateral aldosterone-producing adenoma
* '''35–40%''' – '''Conn's syndrome''' – a label for a unilateral aldosterone-producing adenoma
* 5–8% – unilateral hyperplasia
* '''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''' – 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
* '''rare''' – carcinoma of the adrenal cortex
{{Note|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.}}
{{Note|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.}}


== Clinical picture ==
== Clinical picture ==
[[File:Aldosteron.svg|thumb|240x240px|Aldosterone]]
[[File:Aldosteron.svg|thumb|240x240px|Aldosterone]]
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 a reduced concentration ability of the kidneys manifested by nocturia) to life-threatening heart rhythm disorders.
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 '''[[Blood pressure|increase in blood pressure]]''' with all the manifestations and complications (headaches, fatigue, nosebleeds ).


: So the patient has: hypertension, hypokalemia, hypernatremia.
Long-term elevated blood pressure can be the cause of '''heart failure''' . A reduced potassium level can manifest itself in any way - from [[Constipation|''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.'''


:
:
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; Imaging methods
; Imaging methods


* ultrasound of the kidneys (reninoma) and adrenal glands (tumor, bilateral hyperplasia)
* [[Ultrasound imaging|ultrasound]] of the ''kidneys (reninoma)'' and ''adrenal glands (tumor, bilateral hyperplasia)''
* CT, MRI of the adrenal glands
* [[Computer tomography|CT]], [[Magnetic Resonance Imaging|MRI]] of the ''adrenal glands''


'''Laboratory examination'''
'''Laboratory examination'''


* increased level of Na + and decreased K + in the blood
* '''increased level of Na<sup><big>+</big></sup>''' and '''decreased K<sup>+</sup>''' in the blood


* hormone levels:
* '''hormone levels:'''
** if both aldosterone and renin are high, it means that the cause of the high aldosterone is in the kidneys
** 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
** 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
* '''examination''' of ''plasma renin activity''
* stimulation tests (physical stress, furosemide)
* '''stimulation tests''' (physical stress, furosemide)


== Treatment ==
== Treatment ==
We choose the treatment procedure according to the cause.
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


<noinclude>
* '''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 ==
== Links ==
=== Related articles ===
=== Related articles ===
* [[Dysbalance natria]]
* [[Dysbalance natria (pediatrics)|Dysbalance natria]]
* [[Diabetes insipidus]]
* [[Diabetes insipidus]]
* [[Cushingův syndrom]]
* [[Addison ́s Disease|Addison´s choroba]]
* [[Addisonova choroba]]
* [[Endokrinní hypertenze]]
=== Source ===
=== Source ===
* {{Citace
* {{Citace

Revision as of 22:59, 21 December 2022

  • Excessive secretion of aldosterone by the adrenal cortex.

Etiology

Adrenal cortex in primary hyperaldosteronism
  • 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.

Clinical picture

Aldosterone

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 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

Source

References