Conn syndrome

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Aldosterone.svg|

Conn's syndrome

ICD-10	E260
ICD-9	255.1
OMIM	[1]
DiseasesDB	3073
MedlinePlus	000330
eMedicine	med/432
MeSH	{{{MeshNumber}}}

Conn's syndrome is characterized by the overproduction of the mineralocorticoid hormone aldosterone by the adrenal glands. Aldosterone causes sodium and water retention and potassium excretion in the kidneys, leading to arterial hypertension (high blood pressure). It is a rare but recognised cause of hypertension.

Signs, symptoms and findings

Conn's syndrome is also known as primary hyperaldosteronism. Apart from high blood pressure, the symptoms may include muscle cramps (due to hyperexcitability of neurons), muscle weakness (due to hypoexcitability of skeletal muscles) and headaches (due to the low potassium), metabolic alkalosis (due to increased secretion of H+ ions by the kidney). The high pH of the blood makes calcium less available to the tissues and causes symptoms of hypocalcemia (low calcium levels).

It can be mimicked by liquorice ingestion (glycyrrhizin) and Liddle syndrome.

Diagnosis

Measuring aldosterone alone is not considered adequate to diagnose Conn's syndrome. Rather, both renin and aldosterone are measured, and the ratio is diagnostic.

Usually, renin levels are suppressed, leading to a very low renin-aldosterone ratio (<0.0005). This test is confounded by antihypertensive drugs, which have to be stopped up to 6 weeks.

If there is biochemic proof of hyperaldosteronism, CT scanning can confirm the presence of an adrenal adenoma.

Causes

The syndrome is due to:

• aldosterone-secreting adrenal adenoma (benign tumor, 50-60%)
• hyperplasia of the adrenal gland (40-50%)
• rare forms

Therapy

The treatment for hyperaldosteronism depends on the underlying cause. In patients with a single benign tumor (adenoma), surgical removal (adrenalectomy) is curative. This is usually performed laparoscopically, through several very small incisions. For patients with hyperplasia of both glands, successful treatment is often achieved with spironolactone or eplerenone, drugs that block the effect of aldosterone.

In the absence of proper treatment, individuals with hyperaldosteronism often suffer from poorly controlled high blood pressure, which may be associated with increased rates of stroke, heart disease, and kidney failure. With appropriate treatment, the prognosis is excellent.