Cronkhite–Canada syndrome
Cronkhite–Canada syndrome is a rare syndrome characterized by multiple polyps of the digestive tract. It is sporadic (i.e. it does not seem to be a hereditary disease),[1] and it is currently considered acquired[2] and idiopathic (i.e. cause remains unknown).
About two-thirds of patients are of Japanese descent and the male to female ratio is 2:1.[citation needed] It was characterized in 1955[3][4] by internal medicine physician Leonard Wolsey Cronkhite Jr. and radiologist Wilma Jeanne Canada[5].
Signs and Symptoms[edit | edit source]
Polyps are found throughout the GI tract (most frequently in the stomach and large intestine, followed by the small intestine) though typically avoid the esophagus[5]. A biopsy will reveal them to be hamartomas; the possibility that they progress to cancer is generally considered to be low,[6] although it has been reported multiple times in the past. Chronic diarrhea and protein-losing enteropathy are often observed. Possible collateral features include variable anomalies of ectodermal tissues, such as alopecia, atrophy of the nails, or skin pigmentation[citation needed]
Causes[edit | edit source]
The cause of the disease is unknown. It was originally thought that the epidermal changes were secondary to profound malnutrition as a result of protein-losing enteropathy. Recent findings have called this hypothesis into question; specifically, the hair and nail changes may not improve with improved nutrition.
Other conditions consisting of multiple hamartomatous polyps of the digestive tract include Peutz–Jeghers syndrome, juvenile polyposis, and Cowden disease. Related polyposis conditions are familial adenomatous polyposis, attenuated familial adenomatous polyposis, Birt–Hogg–Dubé syndrome and MUTYH.
Diagnosis[edit | edit source]
There is no specific test to diagnose Cronkhite–Canada syndrome. Diagnosis is based on symptoms and features of the disease.[7]
Management[edit | edit source]
Nutritional support is fundamental, and may include dietary guidance, supplements, tube feeding, or intravenous solutions.[8] Treatments proposed include cromolyn sodium and prednisone,[9] as well as histamine (H2) receptor antagonists or proton pump inhibitors.[8]
References[edit | edit source]
External links[edit | edit source]
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Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.- All articles with unsourced statements
- Articles with unsourced statements from March 2017
- Articles with unsourced statements from August 2020
- Syndromes of unknown causes
- Gastrointestinal tract disorders
- Rare syndromes
- Genodermatoses
- Genetic disorders with OMIM but no gene
- Syndromes affecting the gastrointestinal tract
Contributors: Prab R. Tumpati, MD
