Cronkhite-Canada Syndrome

Cronkhite-Canada Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Cronkhite-Canada Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • 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

  • None

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.

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

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

Resources

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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