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Conn's syndrome

Conn's syndrome is 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 nonessential hypertension. It is named after Dr Jerome W. Conn (1907-1981), the American endocrinologist who first described the condition in 1955. It is the most common form of primary Hyperaldosteronism. more...

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Signs, symptoms and findings

Apart from high blood pressure, the symptoms may include muscle cramps and headaches (due to the low potassium), metabolic alkalosis (due to increased production of bicarbonate in 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 aldosteron are measured, and the ratio is diagnostic. Usually, renin levels are suppressed, leading to a very low renin-aldosterone ratio (<0.05). 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:

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

Therapy

Surgical removal of the offending adrenal (adrenalectomy) takes away the source of the excess hormones. Meanwhile, the blood pressure can be controlled with spironolactone (a diuretic that counteracts the actions of aldosterone) and other antihypertensives.

Reference

  • Conn JW, Louis LH. Primary aldosteronism: a new clinical entity. Trans Assoc Am Physicians 1955;68:215-31; discussion, 231-3. PMID 13299331.

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Aldosterone assay
From Gale Encyclopedia of Medicine, 4/6/01 by Janis O. Flores

Definition

This test measures the levels of aldosterone, a hormone produced by the outer part (cortex) of the two adrenal glands, organs which sit one on top of each of the kidneys. Aldosterone regulates the amounts of sodium and potassium in the blood. This helps maintain water balance and blood volume, which, in turn, affects blood pressure.

Purpose

Aldosterone measurement is useful in detecting a condition called aldosteronism, which is caused by excess secretion of the hormone from the adrenal glands. There are two types of aldosteronism: primary and secondary. Primary aldosteronism is most commonly caused by an adrenal tumor, as in Conn's syndrome. Idiopathic (of unknown cause) hyperaldosteronism is another type of primary aldosteronism. Secondary aldosteronism is more common and may occur with congestive heart failure, cirrhosis with fluid in the abdominal cavity (ascites), certain kidney diseases, excess potassium, sodium-depleted diet, and toxemia of pregnancy.

To differentiate primary aldosteronism from secondary aldosteronism, a plasma renin test should be performed at the same time as the aldosterone assay. Renin, an enzyme produced in the kidneys, is high in secondary aldosteronism and low in primary aldosteronism.

Description

Aldosterone testing can be performed on a blood sample or on a 24-hour urine specimen. Several factors, including diet, posture (upright or lying down), and time of day that the sample is obtained can cause aldosterone levels to fluctuate. Blood samples are affected by short-term fluctuations. A urine specimen collected over an entire 24-hour period lessens the effects of those interfering factors and provides a more reliable aldosterone measurement.

Preparation

Fasting is not required for either the blood sample or urine collection, but the patient should maintain a normal sodium diet (approximately 3 g/day) for at least two weeks before either test. The doctor should decide if drugs that alter sodium, potassium, and fluid balance (e.g., diuretics, antihypertensives, steroids, oral contraceptives) should be withheld. The test will be more accurate if these are suspended at least two weeks before the test. Renin inhibitors (e.g., propranolol) should not be taken one week before the test, unless permitted by the physician. The patient should avoid licorice for at least two weeks before the test, because of its aldosterone-like effect. Strenuous exercise and stress can increase aldosterone levels as well. Because the test is usually performed by a method called radioimmunoassay, recently administered radioactive medications will affect test results.

Since posture and body position affect aldosterone, hospitalized patients should remain in an upright position (at least sitting) for two hours before blood is drawn. Occasionally blood will be drawn again before the patient gets out of bed. Nonhospitalized patients should arrive at the laboratory in time to maintain an upright position for at least two hours.

Risks

Risks for this test are minimal, but may include slight bleeding from the blood-drawing site, fainting or feeling lightheaded after venipuncture, or hematoma (blood accumulating under the puncture site).

Normal results

Normal results are laboratory-specific and also vary with sodium intake, with time of day, source of specimen (e.g., peripheral vein, adrenal vein, 24-hour urine), age, sex, and posture.

Reference ranges for blood include:

  • Supine (lying down): 3-10 ng/dL
  • Upright (sitting for at least two hours): Female: 5-30ng/dL; Male: 6-22 ng/dL.

Reference ranges for urine: 2-80 mg/24 hr.

Abnormal results

Increased levels of aldosterone are found in Conn's disease (aldosterone-producing adrenal tumor), and in cases of Bartter's syndrome (a condition in which the kidneys overexcrete potassium, sodium and chloride, resulting in low blood levels of potassium and high blood levels of aldosterone and renin). Among other conditions, elevated levels are also seen in secondary aldosteronism, stress, and malignant hypertension.

Decreased levels of aldosterone are found in aldosterone deficiency, steroid therapy, high-sodium diets, certain antihypertensive therapies, and Addison's disease (an autoimmune disorder).

Key Terms

Aldosteronism
A condition in which the adrenal glands secrete excessive levels of the hormone aldosterone.
Renin
An enzyme produced in the kidneys that controls the activation of the hormone angiotensin, which stimulates the adrenal glands to produce aldosterone.

Further Reading

For Your Information

    Books

  • Jacobs, David S. Laboratory Test Handbook, 4th ed. Lexi-Comp Inc., 1996.
  • Pagana, Kathleen Deska. Mosby's Manual of Diagnostic and Laboratory Tests. Mosby, Inc., 1998.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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