Cronkhite-Canada syndrome (CCS) is a very rare disease with symptoms that include loss of taste, intestinal polyps, hair loss, and nail growth problems. It is difficult to treat because of malabsorption that accompanies the polyps. CCS occurs primarily in older people (the average age is 59) and it is not believed to have a genetic component. There have been fewer than 400 cases reported in the past 50 years, primarily in Japan but also in the U.S. and other countries.
The symptoms of Cronkhite-Canada syndrome include diarrhea, cramps, excessive gas, and abdominal discomfort. These symptoms occur because of multiple polyps that carpet the walls of the stomach, small intestine, and colon. People with Cronkhite-Canada syndrome may not be able to digest milk and dairy products (lactose intolerance), and have a decrease in their ability to properly absorb nutrients (malabsorption) through the small intestine. Approximately 15 percent of people with Cronkhite-Canada syndrome eventually develop cancer of the colon.
People with Cronkhite-Canada syndrome may have abnormally low levels of protein in the blood (protein-losing hypoproteinemia). This may cause a feeling of general ill health (cachexia), malnutrition, nausea, vomiting, a profound loss of appetite even to the point of malnutrition, weight-loss and/or excess fluid accumulation in the arms and legs (peripheral edema). An imbalance of certain essential minerals (electrolytes) may occur because of chronic diarrhea. Some people with Cronkhite-Canada may also have large skin bruises (ecchymotic plaques) and/or impaired lung function. Other symptoms may include loss of hair (alopecia), large areas of dark spots on the skin (hyperpigmentation) and degenerative changes and eventual loss of the fingernails (onychodystrophy).
The exact cause of Cronkhite-Canada syndrome is not known. It seems to occur for no known reason (sporadically) and is not thought to be genetic in origin.
Cronkhite-Canada syndrome is an extremely rare disorder that affects more females than males. (The ratio seems to be approximately 1.5 females to 1 male. Typically, the age of onset is during the middle years or old age. The average is about 59 years with a range of 31 to 85 years. Worldwide, fewer than 400 cases have been reported in the past 50 years, primarily in Japan but also in the United States and other countries.
Because so little is known of the cause of the disorder, treatment is symptomatic. The primary goal of treatment is to correct fluid, electrolyte and protein loss by nutritional supplementation or a nutritionally balanced liquid diet. Corticosteroid drugs (i.e., prednisone) may be given occasionally to help reduce intestinal inflammation. Bacterial overgrowth in the intestines, which can cause malabsorption, may be treated with antibiotics. In rare cases, symptoms have resolved for no apparent reason (spontaneous remission). Other treatment is symptomatic and supportive.
Surgical removal of polyps may help to relieve some of the symptoms of Cronkhite-Canada syndrome. However, they may recur or be too numerous to remove individually. If necessary, severely affected portions of the colon may be removed.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
A research group at the University of Texas is looking for a patient diagnosed with Cronkhite-Canada syndrome to test a theory about the cause of the disease. If the theory is correct, the poorly growing fingernails will contain a substance that should give an important clue to the cause. The test requires only that the researchers obtain one or two small fingernail clippings from an affected individual (provided through his or her physician) for detailed content analysis. There is no compensation for this study, but it is hoped that the research may lead to an understanding of what causes this difficult disease and, therefore, may help guide future treatment. For information or to participate, ask your physician to contact:
The University of Texas at Austin
1 University Station A8000
Austin, Texas 78712
Phone: (512) 796-9314
Fax: (512) 472-3514
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