Cronkhite Canada Syndrome
| Classification according to ICD-10 | |
|---|---|
| K63.8 | Other specified diseases of the intestine |
| K63.5 | Polyp of the colon |
| K31.7 | Polyp of the stomach and duodenum |
| ICD-10 online (WHO version 2019) | |
The Cronkhite-Canada syndrome is a so-called gastrointestinal Polyposissyndrom , a very rare, non- hereditary disease of the digestive tract of unknown cause . It manifests itself through numerous polyps in the digestive system together with changes in the skin and skin appendages such as hair loss , hyperpigmentation and growth disorders of the fingernails and toenails .
The disease is named after the American internist Leonhard W. Cronkhite jr. and the American radiologist Wilma J. Canada .
Epidemiology
The vast majority of diseases occur in patients of Japanese ancestry and then more frequently in men than women (2: 1).
The cause is unclear, apparently not hereditary. The disease manifests itself in later adulthood initially with watery diarrhea , then with signs of malabsorption . Stomach or intestinal carcinoma develops in 10% of those affected . Hypoproteinemia is found in 50% of cases .
diagnosis
The characteristic clinic consisting of the combination of
- ectodermal changes such as alopecia , hyperpigmentation, and nail dystrophy
- diffuse gastrointestinal polyposis
in (endoscopic) polyp detection leads to diagnosis.
Demarcate are the Peutz-Jeghers syndrome , and various other forms of polyposis (generalized juvenile, hyperplastic polyposis , Kappenpolyposis, lipomatous polyposis or nodular lymphoid hyperplasia and inflammatory or lymphomatous polyposis).
Complications are gastrointestinal bleeding , heart failure or infections . Overall mortality is high
therapy
The etiology and pathogenesis of the disease are unknown. Accordingly, causal therapy is not possible. Treatment is symptomatic with cromoglicic acid and corticosteroids . Because of the malabsorption, it is primarily necessary to compensate for the malnutrition including vitamins and trace elements. Malnutrition seems to be responsible for the alopecia as well as the skin and nail changes.
Web links
- Cronkhite Canada Syndrome. In: Online Mendelian Inheritance in Man . (English)
- F. Dughera, S. Baino, Cronkhite-Canada Syndrome
Individual evidence
- ^ B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9 .
- ↑ LW CRONKHITE, WJ CANADA: Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. In: The New England Journal of Medicine . Volume 252, Number 24, June 1955, pp. 1011-1015, ISSN 0028-4793 . doi: 10.1056 / NEJM195506162522401 . PMID 14383952 .
- ↑ a b c d Orphanet
- ↑ a b W. Pschyrembel: Clinical Dictionary. Verlag Walter de Gruyter, 265th edition 2014, ISBN 978-3-11-018534-8 .
- ↑ E. Ward, HC Wolfsen, C. Ng: Medical management of Cronkhite-Canada syndrome. In: Southern medical journal. Volume 95, Number 2, February 2002, pp. 272-274, ISSN 0038-4348 . PMID 11846261 .
- ↑ Christian Pox: Cronkhite Canada Syndrome. In: Gastroenterology up2date. 4, 2008, pp. 115-115, doi: 10.1055 / s-2008-1077314 .
