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Cronkhite-Canada Syndrome

Synonyms of Cronkhite-Canada Syndrome

  • Allergic Granulomatous Angiitis of Cronkhite-Canada
  • Canada-Cronkhite Disease
  • CCD
  • Cronkhite-Canada Syndrome
  • Gastrointestinal Polyposis and Ectodermal Changes
  • Polyposis, Skin Pigmentation, Alopecia, and Fingernail Changes

Disorder Subdivisions

  • No subdivisions found.

General Discussion

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.

Symptoms

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).

Causes

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.

Affected Populations

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.

Related Disorders

Symptoms of the following disorders can be similar to those of Cronkhite-Canada syndrome. Comparisons may be useful for a differential diagnosis:

Familial polyposis (FAP) is a hereditary condition characterized by multiple benign growths (polyps) which develop around puberty in the mucous lining of the gastrointestinal tract. Symptoms include bleeding, diarrhea, crampy abdominal pain, and/or weight loss. Persistent rectal bleeding may result in secondary anemia. Symptoms may not occur until colon cancer is present. (For more information on this disorder, choose "Familial Polyposis" as your search term in the Rare Disease Database.)

Peutz-Jeghers syndrome (intestinal polyposis, type II) is a rare inherited gastrointestinal disorder characterized by the development of polyps on the mucous lining of the intestine and dark discolorations on the skin and mucous membranes. Symptoms include nausea, vomiting, and abdominal pain that occur because of a form of intestinal obstruction (intussusception). Other symptoms include bleeding from the rectum and dark skin discolorations around the lips, inside the cheeks, and on the arms. Severe rectal bleeding can cause anemia and episodes of recurring, severe abdominal pain. (For more information on this disorder, choose "Peutz- Jeghers" as your search term in the Rare Disease Database.)

Gardner syndrome (intestinal polyposis, type III) is a rare inherited form of familial polyposis characterized by multiple benign growths in the colon. It is also associated with the development of extra teeth (supernumerary dentition), fibrous changes (dysplasia) of the skull and jaw, multiple fibrous tumors, and fatty cysts on the skin (epithelial). Symptoms may include bleeding, diarrhea or constipation, abdominal pain, and/or weight loss. (For more information on this disorder, choose "Gardner" as your search term in the Rare Disease Database.)

Turcot syndrome is an extremely rare inherited disorder characterized by familial polyposis and tumors of the central nervous system (i.e., medulloblastoma, glioblastoma, or ependymoma). Symptoms may include diarrhea, bleeding from the rectum, and/or abdominal discomfort. Neurological symptoms vary greatly and depend on the type and location of the brain tumor. Symptoms may include impaired ability to coordinate movement (ataxia) and difficulty speaking.

The following disorders may be associated with Cronkhite-Canada syndrome as secondary characteristics. They are not necessary for a differential diagnosis:

Lactose intolerance is an inborn error of carbohydrate metabolism characterized by the impaired ability to absorb nutrients from the small intestine (malabsorption syndrome). People with lactose intolerance cannot properly digest lactose which is a sugar found in milk and dairy products. Symptoms of lactose intolerance may include severe diarrhea, vomiting, abnormally low levels of body fluids (dehydration), and failure to thrive. (For more information on this disorder, choose "Lactose Intolerance" as your search term in the Rare Disease Database.)

Standard Therapies

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.

Investigational Therapies

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
Email: prpl@cc.nih.gov

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:

Marc Lewis
Associate Professor
The University of Texas at Austin
1 University Station A8000
Austin, Texas 78712
Email: lewis@psy.utexas.edu
Phone: (512) 796-9314
Fax: (512) 472-3514

Organizations related to Cronkhite-Canada Syndrome

References

TEXTBOOKS
Oberhuber G, Cronkhite-Canada Syndrome. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:338.

Yamada T, Alpers DH, Owyang C, et al. Eds. Textbook of Gastroenterology. 2nd ed. J. B. Lippincott Company. Philadephia, PA; 1995:1960.

REVIEW ARTICLES
Ward EM, Wolfsen HC. Review Article: the non-inherited gastrointestinal polyposis syndromes. Aliment Pharmacol Ther. 2002;16:333-42.

Oberhuber G, Stolte M. Gastric polyps: an update of their pathology and biological significance. Virchows Arch. 200;437:581-90.

JOURNAL ARTICLES
Ward EM, Wolfsen HC. Pharmacological management of Cronkhite-Canada syndrome. Expert Opin Pharmacother. 2003;4:385-89.

Egawa T, Kubota T, Otani Y, et al. Surgically treated Cronkhite-Canada syndrome associated with gastric cancer. Gastric Cancer. 2000;27:156-60.

Ward EM, Wolfsen HC, Ng C. Medical management of Cronkhite-Canada syndrome. South Med J. 2002;95:272-74.

Fossati M, Lombardi F, Cattaneo C, et al. Cronkhite-Canada syndrome. A case of sustained partial remission. Dig Liver Dis. 2001;33:785-77.

Goto A. Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan. Nippon Geka Hokan. 1995;64:3-14.

FROM THE INTERNET
McKusick VA, Ed. Online Mendelian Inheritance in Man (OMIM). The Johns Hopkins University. Entry Number; 175500: Last Edit Date; 2/25/1995.

Rabinowitz SS. Cronkhite-Canada Syndrome. eMedicine. Last Updated: January 10, 2003. 12pp.
www.emedicine.com/ped/topic508.htm

Serwin AB, Mysliwiec H. Cronkhite-Canada Syndrome. eMedicine. Last Updated: October 22, 2001. 14pp.
www.emedicine.com/derm/topic729.htm

Cronkhite-Canada syndrome. CHORUS-Collaborative Hypertext of radiology. Last Updated: 14 March 2001. 1p.
www.chorus.rad.mcw.edu/doc/00084.html

Cronkhite-Canada syndrome Information Page Diseases Database. Last major update: 1 Mar 2003. 3pp.
www.diseasesdatabase.com/sieve/item1.asp?glngUserChoice=1924

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2008/05/03 00:00:00 GMT+0

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