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- $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.
-
-