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$Unique_ID{BRK03635}
$Pretitle{}
$Title{Cronkhite-Canada Disease}
$Subject{Cronkhite-Canada Disease Gastrointestinal Polyposis with Ectodermal
Changes Canada-Cronkhite Disease}
$Volume{}
$Log{}
Copyright (C) 1986, 1987 National Organization for Rare Disorders, Inc.
154:
Cronkhite-Canada Disease
** IMPORTANT **
It is possible that the main title of the article (Cronkhite-Canada
Disease) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
Gastrointestinal Polyposis with Ectodermal Changes
Canada-Cronkhite Disease
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section.
Cronkhite-Canada Disease is characterized by multiple, benign growths
(polyps) on the mucosal lining of the intestinal wall, especially in the
colon, rectum, and stomach, and by changes in ectodermal structures, i.e.,
hair, skin, fingernails, etc. The disease does not appear to be hereditary.
It is possible that the ectodermal changes result from malabsorption of
essential vitamins. Women are affected more often and more severely than
men, and most patients are elderly. The disorder can cause malnutrition and
sometimes life-threatening metabolic disturbances.
Symptoms
Cronkhite-Canada Disease appears in middle aged or elderly persons. The
numerous polyps are found primarily in the stomach and large intestine,
although they also occur in the small intestine. Patients experience
diarrhea, abdominal pain, loss of appetite, and sometimes nausea or vomiting.
Changes in skin and related structures accompany these symptoms. Body hair
may fall out, the skin becomes brownish on the face, neck, and hands, and the
toe and fingernails atrophy. There may be swelling in the ankles, lower back
and pelvis.
The changes in the lining of the intestinal tract can impair absorption
of nutrients and laboratory tests show reduced protein levels in the blood,
electrolyte (salt) imbalances, anemia, and the presence of undigested
proteins and fats in the stool. Thus, the patient may become gradually
weaker and more susceptible to infections, etc.
Causes
Cronkhite-Canada Disease does not seem to be a hereditary disease. Its
causes are unknown. Vitamin deficiencies due to malabsorption may cause the
changes in ectodermal structures.
Affected Population
Cronkhite-Canada Disease affects primarily elderly or middle aged women.
Related Disorders
Cronkhite-Canada Disease is related to several syndromes in which intestinal
polyposis plays a role. Familial Adenomatous Colon Polyposis is
characterized by numerous polyps in the large intestine and a high incidence
of colonic cancer by age 40 if untreated. In Gardner Syndrome, colonic
polyposis is associated with cysts and tumors in the skull and related
structures, and extra teeth. In Peutz-Jeghers Syndrome, numerous polyps in
the stomach and small and large intestines are associated with discoloration
of the skin and mucous surfaces. In Turcot Syndrome, familial polyposis
occurs with tumors in the central nervous system.
Therapies: Standard
Surgical removal of polyps may correct malabsorption in Cronkhite-Canada
Disease. However, they may recur or be too numerous to remove individually.
If necessary, severely affected parts of the stomach or colon can be removed.
Therapies: Investigational
This disease entry is based upon medical information available through
December 1988. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section for
the most current information about this disorder.
Resources
For more information on Cronkhite-Canada Disease, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
National Digestive Diseases Information Clearinghouse
Box NDIC
Bethesda, MD 20892
(301) 468-2162
References
CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.: W. B. Saunders Co., 1988. P. 768.
THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987. P. 739.