$Unique_ID{BRK03622} $Pretitle{} $Title{Conn Syndrome} $Subject{Conn Syndrome Aldosteronism, Primary Hyperaldosteronism, Primary Aldosteronism, Secondary Bartter Syndrome } $Volume{} $Log{} Copyright (C) 1989 National Organization for Rare Disorders, Inc. 628: Conn Syndrome ** IMPORTANT ** It is possible that the main title of this article (Conn Syndrome) is not the name you expected. Please check the SYNONYM list to find the alternate names and disorder subdivisions covered by this article. Synonyms Aldosteronism, Primary Hyperaldosteronism, Primary Information on the following disorders can be found in the Related Disorders section of this report: Aldosteronism, Secondary Bartter Syndrome General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your physician and/or the agencies listed in the "Resources" section of this report. Conn Syndrome is characterized by an increased level of the hormone aldosterone in the blood causing increased sodium levels in the blood. An increase in blood volume (hypervolemia), and a low potassium level (hypokalemic alkalosis) also occur. This disorder is characterized by periods of weakness, unusual sensations such as tingling and warmness, a transient paralysis, and muscle cramps. An increase in blood pressure (hypertension), excessive urination (polyuria), and excessive thirst (polydipsia) can also occur. Symptoms Conn Syndrome (Primary Hyperaldosteronism) is a rare metabolic endocrine disorder characterized by oversecretion of the hormone aldosterone. This hormone is produced by the adrenal glands. The disorder is caused by an abnormal growth (adenoma) in the cortex of the adrenal glands. Aldosterone causes salt (sodium or Na) retention and potassium (K) loss. In the kidneys, salivary and sweat glands, and in the cells of the mucous membranes in the intestines, aldosterone causes transfer of sodium in exchange for potassium and hydrogen. Aldosterone secretion is regulated by the renin-angiotensin mechanism in the kidneys and liver, and to a lesser extent by adrenocorticotropin hormone (ACTH). The sodium and water retention resulting from increased aldosterone secretion increases the blood volume and reduces renin secretion. Increased blood levels of sodium (hypernatremia), an increase in blood volume (hypervolemia), and low potassium (hypokalemic alkalosis), can cause periods of weakness, unusual sensations such as tingling and warmness, a transient paralysis, and muscle spasms. An increase in blood pressure (hypertension), kidney disease with excessive urination (polyuria), and excessive thirst (polydipsia) can also occur. With removal of the abnormal adrenal growth, remission usually occurs. Causes Conn Syndrome is caused by an abnormal growth or tumor (adenoma) in the adrenal glands. The exact cause of this growth is unknown. Related Disorders Symptoms of the following disorders can resemble those of Conn Syndrome. Comparisons may be useful for a differential diagnosis: Bartter Syndrome (Aldosteronism with Normal Blood Pressure) is a rare metabolic disorder which may involve the kidneys. It is characterized by an overproduction of the adrenal hormone aldosterone. Major symptoms may include mental retardation, slowed growth, weakness, dwarfism, excessive thirst and excessive urination. Vomiting, diarrhea, and weight loss may also occur. (For more information, choose "Bartter" as your search term in the Rare Disease Database.) Secondary Aldosteronism is a metabolic endocrine disorder characterized by increased production of aldosterone by the cortex of the adrenal glands caused by stimuli originating outside the adrenal glands. It is similar to Conn Syndrome and related to high blood pressure (hypertension) and disorders with fluid retention and/or swelling (edema) such as heart failure and cirrhosis of the liver with fluid in the abdomen (kidney syndrome). It is believed to be caused by excessive secretion of the enzyme renin, secondary to constriction of the blood vessels in the kidney. This syndrome also occurs as a symptom of other kidney disorders. Conn Syndrome is not marked by the decreased salt (sodium) levels and increased plasma-renin activity as is Secondary Aldosteronism. Therapies: Standard Treatment of Conn Syndrome consists in surgical removal of the adrenal tumor that causes the symptoms. Additional therapy with the adrenocorticolytic drug, mitotane, may be helpful. Treatment with the aldosterone antagonist drug, spironolactone, and the diuretic antihypertensive drug, hydrochlorothiazide, may also be recommended. Therapies: Investigational This disease entry is based upon medical information available through April 1989. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Conn Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The National Adrenal Diseases Foundation, Inc. 505 Northern Blvd., Suite 200 Great Neck, NY 11021 (516) 487-4992 National Digestive Diseases Information Clearinghouse Box NDDIC Bethesda, MD 20892 (301) 468-6344 NIH/National Heart, Lung, and Blood Institute (NHLBI) 9000 Rockville Pike Bethesda, MD 20892 (302) 496-4236 References CLINICAL IMPLICATIONS OF PRIMARY ALDOSTERONISM WITH RESISTANT HYPERTENSION: E.L. Bravo, et al.; Hypertension (February 1988: issue 11(2 Pt 2). Pp. 1207-1211. PURE PRIMARY HYPERALDOSTERONISM DUE TO ADRENAL CORTICAL CARCINOMA: D.J. Greathouse, et al.; Amer Journal Med (June 1984: issue 76(6)). Pp. 1132- 1136. AGING AND ALDOSTERONE: R. Hegstad, et al.; Amer Journal Med (March 1983: issue 74(3)). Pp. 442-448. ISOLATED CLINICAL SYNDROME OF PRIMARY ALDOSTERONISM IN FOUR PATIENTS WITH ADRENOCORTICAL CARCINOMA: D. Farge, et al.; Amer Journal Med (October 1987: issue 83(4)). Pp. 635-640.