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Conn's Syndrome (Primary Hyperaldosteronism)

Primary hyperaldosteronism is a rare disorder that occurs when your body produces too much aldosterone, a hormone that controls sodium and potassium levels in the blood. But what are its symptoms? How should it be treated? The following information should help you talk to a urologist about this condition.

What happens under normal conditions?

The adrenal glands are endocrine glands that are located on the top of both kidneys. The adrenal glands are triangular shaped and measure about one-half inch in height and three inches in length. Each gland consists of a medulla that is surrounded by the cortex. The medulla is responsible for producing epinephrine also known as adrenaline. The adrenal cortex produces other hormones necessary for fluid and salt balance in the body such as cortisone and aldosterone. Disorders of either the cortex or the medulla can result in hypertension.

What is primary hyperaldosteronism?

Also known as Conn's syndrome, this disorder occurs when the adrenal overproduces aldosterone, a hormone that controls sodium and potassium levels in the blood. Its overproduction leads to retention of salt (sodium) and loss of potassium, which leads to hypertension. It is due to either a benign cortical adenoma, or bilateral adrenal hyperplasia. Both diseases cause an over secretion of the salt retaining hormone aldosterone.

Primary hyperaldosteronism accounts for less than one percent of all cases of hypertension. It is more common in females than males (3:1 ratio). It can occur at any age, but most commonly when a person is in their 30s and 40s. The majority of cases are sporadic, but hereditary causes have been identified: one type is glucocorticoid remediable aldosteronism (GRA). GRA is caused by a rare gene where aldosterone production is controlled by the pituitary gland rather than by the kidney. Other causes of hyperaldosteronism include any condition that decreases blood flow to the kidney, including dehydration, kidney artery constriction, cardiac failure, shock, liver disease, pregnancy and renin-secreting kidney tumors. In this setting the term secondary hyperaldosteronism is used because the cause of the hypersecretion is not primarily a problem with the adrenal gland.

What are the symptoms of primary hyperaldosteronism?

In hyperaldosteronism, excess aldosterone leads to an inappropriate salt re-absorption, which increases the extracellular fluid volume until the kidneys can respond appropriately. Patients typically have mild to moderate hypertension. Primary hyperaldosteronism can be distinguished from basic hypertension through blood tests. In general, hyperaldosteronism is unresponsive to standard medical therapy used to prevent or reduce high blood pressure.

Mild hypernatremia (high blood sodium), hypokalemia (low blood potassium), hyperkaluria (high urine potassium) and high levels of alkalinity are the electrolyte abnormalities commonly seen with excess aldosterone. These contribute the following symptoms: muscle weakness, frequent urination, nighttime urination, headache, excessive thirst, pins and needles sensation, visual disturbances, temporary paralysis, muscle twitching and cramps. The severity of these symptoms may be highly variable depending on the degree of electrolyte abnormality.

How is primary hyperaldosteronism diagnosed?

A screening test may be conducted to pinpoint a diagnosis. Blood and urine tests can check for high aldosterone, and low potassium and renin activity. A CT scan or MRI may be ordered to detect tumors.

How is primary hyperaldosteronism treated?

Secondary hyperaldosteronism that is caused by kidney enlargement or heart disease is usually treated with medical therapy. Primary hyperaldosteronism resulting from a tumor is usually treated by removing an adrenal gland (unilateral adrenalectomy). This surgery is almost always performed with a laparoscopic approach. Bilateral hyperplasia is treated with potassium sparing diuretics such as spironolactone.

What can be expected after treatment for primary hyperaldosteronism?

In general, patients experience rapid and uneventful postoperative recovery. Of the patients who undergo unilateral adrenalectomy for primary hyperaldosteronism, hypertension is completely resolved or significantly improved in 80 to 90 percent. The preoperative plasma renin activity (PRA) level is the best predictor of postoperative blood pressure outcome. As a result, the cured patients have lower pre-surgical PRA levels than those who are not cured. Even after surgery, some patients have high blood pressure and require medication for hours or weeks until their blood pressure returns to normal. Blood pressure and serum electrolytes should be monitored after surgery and/or after the start of medical therapy.

Up to 5 percent of patients may suffer from ongoing high blood pressure. The reason for this is not fully understood, but some experts believe this may be a result of chronic, irreversible kidney damage from the primary hyperaldosteronism.

Frequently asked questions:

Can a woman with primary hyperaldosteronism safely become pregnant?

Yes, pregnancy is perfectly feasible. Ironically, in pregnancy the high blood pressure of hyperaldosteronism settles without spironalactone due to the aldosterone effect being blocked by the naturally rising sex hormone produced by the pregnancy.

What is the prognosis for primary hyperaldosteronism?

The outcome is good with treatment.



Reviewed: January 2011

Last updated: April 2013

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Conn's Syndrome Glossary
  • abnormality: A variation from a normal structure or function of the body.

  • adenoma: A benign (non-cancerous) tumor that arises in or resembles glandular tissue.

  • adrenal: Glands that reside above the kidneys composed of an inner medulla and an outer cortex.

  • adrenal cortex: The outer layer of the adrenal gland.

  • adrenal gland: One of a pair of small glands, each of which sits on top of one of the kidneys. These glands produce hormones that help control heart rate, blood pressure, the way the body uses food and other vital functions.

  • adrenalectomy: Surgical removal of one or both adrenal glands.

  • adrenaline: Also known as epinephrine. Hormone secreted in the adrenal gland that raises blood pressure, produces a rapid heartbeat and acts a neurotransmitter when the body is subjected to stress or danger.

  • aldosterone: An adrenal hormone critical for salt (sodium) balance. Produced by the outer part of the adrenal cortex, its action causes a reabsorption of sodium and secretion of potassium and is critical for the maintenance of blood pressure.

  • alkalinity: The concentration of alkali, an acid-neutralizing chemical substance, in a solution measured in terms of pH.

  • artery: Blood vessel that carries blood from the heart to various parts of the body.

  • benign: Not malignant; not cancerous.

  • bilateral: Term describing a condition that affects both sides of the body or two paired organs.

  • cellular: Relating to small parts or groups.

  • chronic: Lasting a long time. Chronic diseases develop slowly. Chronic renal (kidney) failure may develop over many years and lead to end-stage renal (kidney) disease.

  • constriction: The process of becoming narrower.

  • cortex: The outer layer of an organ.

  • cortisone: A hormone secreted by the adrenal gland and used to treat rheumatoid arthritis and allergies.

  • CT scan: Also known as computerized tomography, computerized axial tomography or CT scan. A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images of the body. Shows detailed images of any part of the body, including bones, muscles, fat and organs. CT scans are more detailed than general X-rays.

  • dehydration: Dangerous lack of water in the body resulting from inadequate intake of fluids or excessive loss through sweating, vomiting or diarrhea.

  • diagnosis: The process by which a doctor determines what disease or condition a patient has by studying the patient's symptoms and medical history, and analyzing any tests performed (e.g., blood tets, urine tests, brain scans, etc.).

  • diuretic: A drug that increases the amount of water in the urine, removing excess water from the body.

  • electrolytes: Chemicals in the body fluids that result from the breakdown of salts, including sodium, potassium, magnesium and chloride. The kidneys control the amount of electrolytes in the body. When the kidneys fail, electrolytes get out of balance, causing potentially serious health problems. Dialysis can correct this problem.

  • endocrine: Relating to glands that secrete hormones internally directly into the lymph nodes or bloodstream.

  • endocrine glands: Any gland of the body that secretes hormones directly into the lymph nodes or bloodstream.

  • epinephrine: Also known as adrenaline. Hormone secreted in the adrenal gland that raises blood pressure, produces a rapid heartbeat and acts as a neurotransmitter when the body is subjected to stress or danger.

  • gene: The basic unit capable of transmitting characteristics from one generation to the next.

  • gland: A mass of cells or an organ that removes substances from the bloodstream and excretes them or secretes them back into the blood with a specific physiological purpose.

  • glucocorticoid: A steroid hormone that controls the metabolism of carbohydrates, proteins and fats.

  • glucocorticoid remediable aldosteronism: Also known as GRA. A trait characterized by moderate to severe high blood pressure (hypertension).

  • GRA: Also known as glucocorticoid remediable aldosteronism. A trait characterized by moderate to severe high blood pressure (hypertension).

  • high blood pressure: Medical term is hypertension.

  • hormone: A natural chemical produced in one part of the body and released into the blood to trigger or regulate particular functions of the body. Antidiuretic hormone tells the kidneys to slow down urine production.

  • hyperaldosteronism: Syndrome associated with increased production of the hormone aldosterone, a hormone that controls sodium and potassium levels in the blood.

  • hyperplasia: Excessive growth of normal cells of an organ.

  • hypertension: High blood pressure, which can be caused either by too much fluid in the blood vessels or by the narrowing of blood vessels.

  • ions: Electrically charged atoms.

  • kidney: One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.

  • kidneys: One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.

  • laparoscopic: Using an instrument in the shape of a tube that is inserted through the abdominal wall to give an examining doctor a view of the internal organs.

  • liver: A large, vital organ that secretes bile, stores and filters blood, and takes part in many metabolic functions, for example, the conversion of sugars into glycogen. The liver is reddish-brown, multilobed, and in humans is located in the upper right part of the abdominal cavity.

  • medulla: The innermost area of an organ.

  • MRI: Also referred to a magnetic resonance imaging. A diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.

  • pituitary: Relating to or produced by the pituitary gland.

  • pituitary gland: The main endocrine gland. It is a small oval shaped structure in the head and it regulates growth, sexual maturing and metabolism.

  • plasma renin activity: Also known as PRA. Measures the level of renin in the blood. Renin is an enzyme released by the kidney to help control the body's sodium-potassium balance, fluid volume, and blood pressure.

  • postoperative: Occurring after a surgical operation.

  • potassium: An alkali element.

  • PRA: Also known as plasma renin activity. Measures the level of renin in the blood. Renin is an enzyme released by the kidney to help control the body's sodium-potassium balance, fluid volume, and blood pressure.

  • PRA level: Also referred to as plasma renin activity level. Used to screen high blood pressure or diagnose excess aldosterone in a condition called Conn's syndrome.

  • pregnancy: The condition of being pregnant.

  • renal: Pertaining to the kidneys.

  • renin: A hormone made by the kidneys that helps regulate the volume of fluid in the body and blood pressure.

  • retention: In ability to empty urine from the bladder, which can be caused by atonic bladder or obstruction of the urethra.

  • secretion: Process of producing a substance from the cells and fluids within a gland or organ and discharging it.

  • serum: Clear, watery body fluid.

  • serum electrolytes: A compound that conducts electricity and is simultaneously decomposed by it.

  • spironalactone: A steroid used with other drugs in treating high blood pressure (hypertension).

  • tumor: An abnormal mass of tissue or growth of cells.

  • unilateral: Affecting only one side.

  • unilateral adrenalectomy: Removal of one adrenal gland.

  • urge: Strong desire to urinate.

  • urination: The passing of urine.

  • urine: Liquid waste product filtered from the blood by the kidneys, stored in the bladder and expelled from the body through the urethra by the act of urinating (voiding). About 96 percent of which is water and the rest waste products.

  • urologist: A doctor who specializes in diseases of the male and female urinary systems and the male reproductive system. Click here to learn more about urologists. (Download the free Acrobat reader.)

  • urology: Branch of medicine concerned with the urinary tract in males and females and with the genital tract and reproductive system of males.

Conn's Syndrome Anatomical Drawings

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