Hyperaldosteronism

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Hyperaldosteronism Main page

Patient Information

Overview

Classification

1- Primary hyperaldosteronism
2- Secondary hyperaldosteronism
3- Pseudohyperaldosteronism causes (low renin)

Differentiating diagonsis

History and symptoms

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

This page contains general information about Hyperaldosteronism. For more information on specific types, please visit the pages on Primary hyperaldosteronism, and Secondary hyperaldosteronism.

Synonyms and keywords: Aldosteronism

Overview

Classification

Aldosteronism and mineralocorticoid excess may be classified into two types, primary hyperaldosteronism (conn's syndrome) and secondary hyperaldosteronism. The different types of aldosteronism described in the below table:


Primary hyperaldosteronism (conn's syndrome)

Primary hyperaldosteronism Category Diseases
Adrenal causes Aldosterone-secreting adrenal adenoma
Idiopathic hyperaldosteronism
Extra-adrenal causes Ectopic secretion of aldosterone
Familial hyperaldosteronism Familial hyperaldosteronism type I
  • Glucocorticoid-remediable aldosteronism [GRA]
Familial hyperaldosteronism II
Familial hyperaldosteronism type III
  • Associated with the germline mutation in the KCNJ5 potassium channel)
Other Pure aldosterone-producing adrenocortical carcinomas
Unilateral adrenal hyperplasia


Secondary hyperaldosteronism

Secondary hyperaldosteronism Category Diseases
Genetic mutation Bartter and Gitelman syndromes (hyperplasia of the juxtaglomerular apparatus, the source of renin in the kidney)
Endocrine causes Cushing syndrome
  • The main pathogenetic mechanism is linked to the excess

of cortisol which saturates 11-HSD2 activity,

Ectopic ACTH production (Secondary to carcinomas such as lung cancer)
Renovascular Kidney transplant
Renin-secreting juxtaglomerular cell tumors
Scleroderma renal crisis
Malignant hypertension
Tumors Reninoma
Intravascular hypovolemia
  • Heart failure
  • Hepatic cirrhosis
  • Nephrotic syndrome

Pseudohyperaldosteronism causes (low renin):

Pseudohyperaldosteronism causes Disease Etiology Clinical features Labratory
Elevated mineralocorticoid Renin Aldosterone Other Treatment
Endogenous causes 17 alpha-hydroxylase deficiency Mutations in the CYP17A1 gene Deoxycorticosterone (DOC) Cortisol ↓ Corticosteroids
11β-hydroxylase deficiency Mutations in the CYP11B1 gene Cortisol ↓
Apparent mineralocorticoid excess syndrome (AME) Genetic or acquired defect of 11-HSD
  • Cortisone decreases and cortisol accumulates and binds to aldosterone receptors
  • Severe juvenile hypertension
  • Hypercalciuria, nephrocalcinosis, polyuria (due to hypokalemia-induced nephrogenic diabetes insipidus)
  • Renal failure
Cortisol has mineralocorticoid effects Urinary free cortisone ↓↓ Dexamethasone and/or mineralocorticoid blockers
Liddle’s syndrome (Pseudohyperaldosteronism type 1) Mutation of the epithelial sodium channels (ENaC) gene in the distal renal tubules
  • Hypertension
  • Hypokalemia
No extra mineralocorticoid presents, and mutations in Na channels mimic aldosterone mechanism Cortisol ↓ amiloride or triamterene can reverse the clinical picture reactivating the renin aldosterone
Cushing’s syndrome The main pathogenetic mechanism is linked to the excess

of cortisol which saturates 11-HSD2 activity, allowing cortisol to bind MR. A similar picture is also related to over secretion of cortisol by adrenocortical carcinomas. In some cases the disease is associated with secondary hyperaldosteronism due to a direct activation of the renin angiotensin system by glucocorticoids.

Rapid weight gain, particularly of the trunk and face with limbs sparing (central obesity) Cortisol has mineralocorticoid effects
  • ↓ if excess cortisol saturates 11-HSD2 enzyme activity
  • ↑ in direct activation of renin angiotensin system activation by glucocorticoids
Urinary free cortisol markedly ↑↑
  • Adrenalectomy
Insensitivity to glucocorticoids (Chrousos syndrome) Mutations in glucocorticoid receptor (GR) gene
  • Hypertension
  • Adrenal hyperandrogenism
Deoxycorticosterone (DOC) Cortisol Dexamethasone
Aldosterone-secreting adrenocortical carcinoma Cortisol has mineralocorticoid effects
  • ↓ if excess cortisol saturates 11-HSD2 enzyme activity
  • ↑ in direct activation of renin angiotensin system activation by glucocorticoids
Urinary free cortisol markedly ↑↑
Geller’s syndrome mutation of MR that alters its specificity and allows progesterone to bind MR severe hypertension particularly during pregnancy
Gordon’s syndrome (Pseudohypoaldosteronism type 2) Mutations of at least four genes have been identified, including WNK1 and WNK4
  • Hypertension
  • Hyperkalemia
  • Normal renal function
No excess mineralocorticoid; an increased activity of the thiazide-sensitive Na–Cl co-transporter in the distal tubule Normal Hyperkalemia thiazide diuretics and/or dietary sodium restriction
Exogenous causes Corticosteroids with mineralocorticoid activity Fludrocortisone or fluoroprednisolone can mimic the action of aldosterone,
  • Hypertension
  • Hypokalemia
Medications such as Fludrocortisone - Change the treatment
Licorice ingestion glycyrrhetinic acid that binds MR and blocks 11-HSD2 at the level of classical target tissues of aldosterone Urinary free cortisol Moderate ↑
grapefruit High assumption of naringenin, a component of grapefruit, can also block 11-HSD
Contraceptives Estrogens can retain sodium and water by different mechanisms, causing increased blood pressure values and suppressing the renin aldosterone system. However, contraceptives usually cause hypertension through a secondary hyperaldosteronism due to the stimulation of the synthesis of angiotensinogen
Particular causes of hypertension Sclerosis of juxtaglomerular apparatus (diabetic microangiopathy and/or of the elderly)
FANS
B-Adrenergic agonists
Aging
Low-renin essential hypertension
Autonomic dysfunction
Partial/total nephrectomy or removal of renal tissue

Differentiating Diagnosis

Hyperaldosteronism should be differentiated from other diseases causing hypertension and hypokalemia for example:

 
 
 
 
 
 
 
 
Hypertension and Hypokalemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Plasma renin activity
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal or High (Plasma Renin/Aldosterone ratio <10
 
 
 
 
 
 
 
 
 
 
 
Suppressed (Plasma Renin/Aldosterone ratio >20
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
*Renin-secreting tumors
*Diuretic use
*Renovascular hypertension
*Coarctation of aorta
*Malignant phase hypertension
 
 
 
 
 
 
 
 
 
 
 
Urinary aldosterone
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Elevated
 
Normal
 
 
Low
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Conn's syndrome (Primary aldosteronism)
 
Profound K+ depletion
 
 
• 17 alpha hydroxylase deficiency
• 11 beta hydroxylase deficiency
• Liddle's syndrome
• Licorice ingestion
• Deoxycortisone producing tumor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Add Mineralocrticoid antagonist for 8 weeks
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)
• Licorice ingestion
•Glucocorticoid resistance
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Liddle's syndrome)

History and symptoms

de:Hyperaldosteronismus it:Iperaldosteronismo


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