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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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Shigellosis
From Gale Encyclopedia of Medicine, 4/6/01 by David Stanley Kaminstein

Definition

Shigellosis is an infection of the intestinal tract by a group of bacteria called Shigella. The bacteria is named in honor of Shiga, a Japanese researcher, who discovered the organism in 1897. The major symptoms are diarrhea, abdominal cramps, fever, and severe fluid loss (dehydration). Four different groups of Shigella can affect humans; of these, S. dysenteriae generally produces the most severe attacks, and S. sonnei the mildest.

Description

Shigellosis is a well-known cause of traveler's diarrhea and illness throughout the world. Shigella are extremely infectious bacteria, and ingestion of just 10 organisms is enough to cause severe diarrhea and dehydration. Shigella accounts for 10 -20% of all cases of diarrhea worldwide, and in any given year infects over 140 million persons and kills 600,000, mostly children and the elderly. The most serious form of the disease is called dysentery, which is characterized by severe watery (and often blood- and mucous-streaked) diarrhea, abdominal cramping, rectal pain, and fever. Shigella is only one of several organisms that can cause dysentery, but the term bacillary dysentery is usually another name for shigellosis.

Most deaths are in less-developed or developing countries, but even in the United States, shigellosis can be a dangerous and potentially deadly disease. Poor hygiene, overcrowding, and improper storage of food are leading causes of infection. The following statistics show the marked difference in the frequency of cases between developed and less-developed countries; in the United States, about 30,000 individuals are hit by the disease each year or about 10 cases/100,000 population. On the other hand, infection in some areas of South America is 1,000 times more frequent. Shigellosis is most common in children below age five, and occurs less often in adults over 20.

Causes & Symptoms

Shigella share several of the characteristics of a group of bacteria that inhabit the intestinal tract. E. coli, another cause of food-borne illness, can be mistaken for Shigella both by physicians and the laboratory. Careful testing is needed to assure proper diagnosis and treatment. Shigella are very resistant to the acid produced by the stomach, and this allows them to easily pass through the gastrointestinal tract and infect the colon (large intestine). The result is a colitis that produces multiple ulcers, which can bleed. Shigella also produce a number of toxins (Shiga toxin and others) that increase the amount of fluid secretion by the intestinal tract. This fluid secretion is a major cause of the diarrhea symptoms.

Shigellainfection spreads through food or water contaminated by human waste. Sources of transmission are:

  • Contaminated milk, ice cream, vegetables and other foods which often cause epidemics
  • Household contacts (40% of adults and 20% of children will develop infection from such a source)
  • Poor hygiene and overcrowded living conditions
  • Day care centers
  • Sexual practices which lead to oral-anal contact, directly or indirectly.

Symptoms can be limited to only mild diarrhea or go on to full blown dysentery. Dehydration results from the large fluid losses due to diarrhea, vomiting and fever. Inability to eat or drink worsens the situation.

In developed countries, most infections are of the less severe type, and are often due to S. sonnei. The period between infection and symptoms (incubation period) varies from one to seven days. Shigellosis can last from a few days to several weeks, with an average of seven days.

Complications

Areas outside the intestine can be involved, including:

  • Nervous system (irritation of the meninges or meningitis, encephalitis, and seizures).
  • Kidneys (producing hemolytic uremic syndrome or HUS which leads to kidney failure).
  • Joints (leading to an unusual form of arthritis called Reiter's syndrome).
  • Skin (rash).

One of the most serious complications of this disease is HUS, which involves the kidney. The main findings are kidney failure and damage to red blood cells. As many as 15% of patients die from this complication, and half the survivors develop chronic kidney failure, requiring dialysis.

Another life-threatening condition is toxic megacolon. Severe inflammation causes the colon to dilate or stretch, and the thin colon wall may eventually tear. Certain medications (particularly those that diminish intestinal contractions) may increase this risk, but this interaction is unclear. Clues to this diagnosis include sudden decrease in diarrhea, swelling of the abdomen, and worsening abdominal pain.

Diagnosis

Shigellosis is one of the many causes of acute diarrhea. Culture (growing the bacteria in the laboratory) of freshly obtained diarrhea fluid is the only way to be certain of the diagnosis. But even this is not always positive, especially if the patient is already on antibiotics. Shigella are identified by a combination of their appearance under the microscope and various chemical tests. These studies take several days, so quicker means to recognize the bacteria and its toxins are being developed.

Treatment

The first aim of treatment is to keep up nutrition and avoid dehydration. Ideally, a physician should be consulted before starting any treatment. Antibiotics may not be necessary, except for the more severe infections. Many cases resolve before the diagnosis is established by culture. Medications that control diarrhea by slowing intestinal contractions can cause problems and should be avoided by patients with bloody diarrhea or fever, especially if antibiotics have not been started.

Rehydration

The World Health Organization (WHO) has developed guidelines for a standard solution taken by mouth, and prepared from ingredients readily available at home. This Oral Rehydration Solution (ORS) includes salt, baking powder, sugar, orange juice, and water. Commercial preparations, such as Pedialyte, are also available. In many patients with mild symptoms, this is the only treatment needed. Severe dehydration usually requires intravenous fluid replacement.

Antibiotics

In the early and mid 1990s, researchers began to realize that not all cases of bacterial dysentery needed antibiotic treatment. Many patients improve without such therapy, and therefore these drugs are indicated only for treatment of moderate or severe disease, as found in the tropics. Choice of antibiotic is based on the type of bacteria found in the geographical area and on laboratory results. Recommendations as of 1997 include ampicillin, sulfa derivatives such as Trimethoprim-Sulfamethoxazole (TMP-SMX) sold as Bactrim, or fluoroquinolones (such as Ciprofloxacin which is not FDA approved for use in children).

Prognosis

Many patients with mild infections need no specific treatment and recover completely. In those with severe infections, antibiotics will decrease the length of symptoms and the number of days bacteria appear in the feces. In rare cases, an individual may fail to clear the bacteria from the intestinal tract; the result is a persistent carrier state. This may be more frequent in AIDS (Acquired Immune Deficiency Syndrome) patients. Antibiotics are about 90% effective in eliminating these chronic infections.

In patients who have suffered particularly severe attacks, some degree of cramping and diarrhea can last for several weeks. This is usually due to damage to the intestinal tract, which requires some time to heal. Since antibiotics can also produce a form of colitis, this must be considered as a possible cause of persistent or recurrent symptoms.

Prevention

Shigellosis is an extremely contagious disease; good hand washing techniques and proper precautions in food handling will help in avoiding spread of infection. Children in day care centers need to be reminded about hand washing during an outbreak to minimize spread. Shigellosis in schools or day care settings almost always disappears when holiday breaks occur, which sever the chain of transmission.

Traveler's diarrhea (TD)

Shigella accounts for about 10% of diarrhea illness in travelers to Mexico, South America, and the tropics. Most cases of TD are more of a nuisance than a life-threatening disease. However, bloody diarrhea is an indication that Shigella may be responsible.

In some cases though, aside from ruining a well deserved vacation, these infections can interrupt business conference schedules and, in the worst instances, lead to a life-threatening illness. Therefore, researchers have tried to find a safe, yet effective, way of preventing TD. Of course the best prevention is to follow closely the rules outlined by the WHO and other groups regarding eating fresh fruits, vegetables, and other foods.

One safe and effective method of preventing TD is the use of large doses of Pepto Bismol. Tablets are now available which are easier for travel; usage must start a few days before departure. Patients should be aware that Bismuth will turn bowel movements black.

Antibiotics have also proven to be highly effective in preventing TD. They can also produce significant side effects, and therefore a physician should be consulted before use. Like Pepto Bismol, antibiotics need to be started before beginning travel.

Key Terms

Dysentery
A disease marked by frequent watery bowel movements, often with blood and mucus, and characterized by pain, urgency to have a bowel movement, fever, and dehydration.
Traveler's diarrhea
An illness due to infection from a bacteria or parasite that occurs in persons traveling to areas where there is a high frequency of the illness. The disease is usually spread by contaminated food or water.
Oral Rehydration Solution(ORS)
A liquid preparation developed by the World Health Organization that can decrease fluid loss in persons with diarrhea. Originally developed to be prepared with materials available in the home, commercial preparations have recently come into use.
Antibiotic
A medication that is designed to kill or weaken bacteria.
Anti-motility medications
Medications such as loperamide (Imodium), dephenoxylate (Lomotil), or medications containing codeine or narcotics which decrease the ability of the intestine to contract. These may worsen the condition of a patient with dysentery or colitis.
Food-borne illness
A disease that is transmitted be eating or handling contaminated food.
Fluoroquinolones
A relatively new group of antibiotics that have had good success in treating infections with many gram-negative bacteria, such as . One drawback is that they should not be used in children under 17 years of age, because of possible effect on bone or cartilage growth.
Dialysis
A form of treatment for patients with kidneys that do not function properly. The treatment removes toxic wastes from the body that are normally removed by the kidneys.
Colitis
Inflammation of the colon or large bowel which has several causes. The lining of the colon becomes swollen, and ulcers often develop. The ability of the colon to absorb fluids is also affected, and diarrhea often results.
Carrier state
The continued presence of an organism (bacteria, virus, or parasite) in the body that does not cause symptoms, but is able to be transmitted and infect other persons.
Stool
Passage of fecal material; a bowel movement.
Meninges
Outer covering of the spinal cord and brain. Infection is called meningitis, which can lead to damage to the brain or spinal cord and lead to death.

Further Reading

For Your Information

    Books

  • Biddle, Wayne. A Field Guide to Germs. New York: Henry Holt and Company, Inc., 1995.
  • Butterton, Joan R., and Stephen B. Calderwood. "Acute Infectious Diarrheal Diseases and Bacterial Food Poisoning." In Harrison's Principles of Internal Medicine edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
  • Hamer, Davidson H., and Sherwood L. Gorbach. "Infectious Diarrhea and Bacterial Food Poisoning." In Sleisenger & Fordtran's Gastrointestinal and Liver Disease, edited by Mark Feldman, et al. Philadelphia: W.B. Saunders Company, 1997.
  • Karlen, Arno. Man and Microbes: Disease and Plagues in History and Modern Times. New York: G. P. Putnam's Sons, 1995.
  • Keusch, Gerald T. "Shigellosis." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
  • Thielman, Nathan M., and Richard L. Guerrant. "Food-Borne Illness." In Conn's Current Therapy, 1996, edited by Robert E. Rakel. Philadelphia: W.B. Saunders Company, 1996.

    Periodicals

  • Khan, Wasif A. "Treatment of Shigellosis: V. Comparison of Azithromycin and Ciprofloxacin." Annals of Internal Medicine 26 (May 1, 1997): 697-703.
  • "Traveler's Diarrhea: Don't Let It Ruin Your Trip." Mayo Clinic Health Letter (January 1997).
  • "When Microbes are on the Menu." Harvard Health Letter (December 1994): 4-5.

    Organizations

  • Centers for Disease Control and Prevention, 1600 Clifton Rd., NE, Atlanta, GA 30333, USA (404)639-3311 http://www.cdc.gov.
  • National Institute of Diabetes and Digestive and Kidney Diseases. http://www.niddk.nih.gov/NIDDK_HomePage.html.
  • The World Health Organization Headquarters, CH-1211 Geneva 27, Switzerland. +41 22 791 2111; (fax) +41 22 791 0746. http://www.who.ch.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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