Pathophysiology and clinical features of primary aldosteronism
- William F Young, Jr, MD, MSc
William F Young, Jr, MD, MSc
- Professor of Medicine
- Mayo Clinic College of Medicine
Nonsuppressible (primary) hypersecretion of aldosterone is an underdiagnosed cause of hypertension. The classic presenting signs of primary aldosteronism are hypertension and hypokalemia, but potassium levels are often normal in modern-day series of aldosteronomas.
The pathophysiology and clinical features of primary aldosteronism will be reviewed here. The treatment of this disorder and an approach to the diagnosis of hypertension and hypokalemia are discussed separately. (See "Treatment of primary aldosteronism" and "Diagnosis of primary aldosteronism".)
TYPES OF PRIMARY ALDOSTERONISM
Renin-independent, incompletely suppressible (primary) hypersecretion of aldosterone is an increasingly recognized, but still underdiagnosed, cause of hypertension ; it is estimated to be responsible for 5 to 13 percent of hypertension in humans. Many subtypes of primary aldosteronism have been described since Conn's original report of the aldosterone-producing adenoma (APA) in 1954 [2-5].
The most frequent causes of primary aldosteronism include:
●Bilateral idiopathic hyperaldosteronism (or idiopathic hyperplasia [IHA], 60 to 70 percent).
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- TYPES OF PRIMARY ALDOSTERONISM
- Renal actions of aldosterone
- Mutations in aldosterone-producing adenomas
- - Ion channel mutations
- KCNJ5 mutations
- - Activating mutations of CTNNB1 (beta-catenin)
- Idiopathic hyperplasia
- CLINICAL FEATURES
- Hypokalemia: An inconsistent finding
- Cardiovascular risk
- Renal effects
- - Mild hypernatremia
- - Hypomagnesemia
- - Muscle weakness
- Genotype-phenotype correlation