Cronkhite-Canada syndrome is a rare gastrointestinal disorder characterized by widespread colon polyps, unhealthy looking (dystrophic) nails, hair loss. Cronkhite-Canada syndrome (CCS) is a rare gastrointestinal (GI) polyposis syndrome characterized by the association of non-hereditary GI polyposis with the. Cronkhite-Canada Syndrome (CCS) is a rare non-inherited disease characterized by gastrointestinal polyposis and ectodermal abnormalities.
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Cronkhite-Canada syndrome CCS is an extremely rare disease characterized by various intestinal polyps, loss of taste, hair loss, and nail growth problems. It is difficult to treat because of malabsorption that accompanies the polyps. CCS occurs primarily in the older population average age 59 and predominantly syndromr in males. It is considered to be an acquired, not hereditary, disease.
The symptoms of Cronkhite-Canada syndrome occur because of multiple polyps occurring in the stomach, small intestine, colon and, less frequently, the esophagus. These include chronic or recurring watery diarrhea, cramps, and abdominal discomfort. These people may also have abnormally low levels of protein in the blood protein-losing enteropathycausing a feeling of general ill health cachexiamalnutrition, nausea and vomiting. The earliest symptoms reported are changes in taste and loss of smell.
An imbalance of certain essential minerals electrolytes may occur because of chronic diarrhea. Other symptoms may include loss of hair alopecialarge areas of dark spots on the skin hyperpigmentation and degenerative changes and, eventually, loss of the fingernails onychodystrophy. Patients with CCS can also have coexisting autoimmune disorders, where the body develops antibodies against an organ, thereby attacking itself, e. The exact cause of Cronkhite-Canada syndrome is unknown.
It seems to occur for no known reason sporadically and is not thought to be hereditary. Cronkhite-Canada syndrome is an extremely rare disorder that affects males predominantly. The ratio seems to be approximately 3 males to 2 females.
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 86 years. Worldwide, over cases have been canad in the past 50 years, primarily in Japan but also in the United States and other countries. Symptoms of the following disorders can be similar to those of Cronkhite-Canada syndrome.
Comparisons may be useful for a differential diagnosis:. Familial adenomatous polyposis FAP is a rare inherited cancer predisposition syndrome characterized by hundreds to thousands of precancerous colorectal polyps adenomatous polyps appearing on average at age 16 years. FAP is inherited in an autosomal dominant manner and caused by abnormalities mutations in the APC gene. Peutz-Jeghers syndrome PJS canadaa a rare inherited gastrointestinal disorder characterized by the development of polyps on the mucous sundrome 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. Other symptoms include bleeding from the rectum and dark skin discolorations around the lips, inside the cheeks, and on the arms.
Cronkhite-Canada syndrome — Mayo Clinic
Severe rectal bleeding can cause anemia and episodes of recurring, severe abdominal pain. The diagnostic criteria for Cronkhite Canada Syndrome is based on symptoms and particular features; however, there is no specific diagnostic test for this syndrome. The mean age of onset is 60, ranging from 31 to 86 years old.
There are usually large numbers of polyps in the digestive tract, most often sparing the esophagus.
The polyps have hamartomatous features, meaning they contain mucus and are inflamed within an intact surface. Besides eyndrome GI tract, findings in the skin are also diagnostic for this disease.
Patients experience alopecia loss of hairdark spots on the skin of the arms, legs and face hyperpigmented macules and have a loss of finger nails onychodystrophy. One test that can be positive with this syndrome is IgG4 plasma cells but a negative test does not rule out the syndrome.
Case Reports in Medicine
The most important aspects for a diagnosis of Cronkhite Canada Syndrome are the aforementioned physical presentations as there is no particular cronkuite to provide a definitive diagnosis of the syndrome. Treatment Treatment is based on controlling zyndrome and providing support. The primary goal of treatment is to correct fluid, electrolyte and protein loss by nutritional supplementation or a nutritionally balanced liquid diet.
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.
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. Case reports have suggested the use of immunosuppressive treatment, including aziathioprin and ciclosporin, if other treatments are not effective. Information on current clinical trials is posted on the Internet at www.
All studies receiving U. Some current clinical trials also are posted on the following page on the NORD website: For information about clinical trials sponsored by private sources, in the main, contact: For more information about clinical trials conducted in Europe, contact: Cronkhite-Canada syndrome diagnosis in the absence of gastrointestinal polyps: Int J Surg Pathol.
Surgically treated Cronkhite-Canada syndrome associated with gastric cancer. A case of sustained partial remission. A case of Cronkhite-Canada syndrome presenting with adenomatous and inflammatory colon polyps. Nat Rev Gastroenterol Sundrome. Pharmacological management of Cronkhite-Canada syndrome.
Medical management of Cronkhite-Canada syndrome. Accessed April 2, Parikh K and Elston DM. The content of the website and databases of the National Organization for Rare Disorders NORD is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD.
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syndeome Synonyms of Cronkhite-Canada Syndrome allergic granulomatous angiitis of Cronkhite-Canada Canada-Cronkhite disease CCD CCS gastrointestinal polyposis and ectodermal changes polyposis, skin pigmentation, alopecia, and fingernail changes. General Discussion Cronkhite-Canada syndrome CCS is an extremely rare disease characterized by various intestinal polyps, loss of taste, hair loss, and nail growth problems.
Causes The exact cause of Cronkhite-Canada syndrome is unknown. Affected Populations Cronkhite-Canada syndrome is an extremely rare disorder that affects males predominantly. Related Disorders Symptoms of the following disorders can be similar to those of Cronkhite-Canada syndrome.
Comparisons may be useful for a differential diagnosis: Diagnosis The diagnostic criteria for Cronkhite Canada Syndrome is based on symptoms and particular features; however, there is no specific diagnostic test for this syndrome.
Cronkhite-Canada Syndrome – NORD (National Organization for Rare Disorders)
Standard Therapies Treatment Treatment is based on controlling symptoms and providing support. Investigational Therapies Information on current clinical trials is posted on the Internet at www.
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