Cronkhite–Canada syndrome

Cronkhite–Canada syndrome
Cronkhite–Canada syndrome
Classification and external resources
ICD-10 K63.8, K63.5, K31.7
ICD-9 211.3
OMIM 175500
DiseasesDB 1924
eMedicine derm/729
MeSH D044483

Cronkhite–Canada syndrome is a rare syndrome characterised 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.

It was characterized in 1955.[3][4]

Contents

Presentation

Polyps are most frequent in the stomach and large intestine, are also found in the small intestine, and are least frequent in the esophagus. A biopsy will reveal them to be hamartomas; the possibility that they progress to cancer is generally considered to be low,[5] 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 of the skin.

Cause

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.

Treatment

Treatments proposed include cromolyn sodium and prednisone.[6]

See also

Other conditions consisting of multiple hamartomatous polyps of the digestive tract include Peutz-Jeghers syndrome, juvenile polyposis, and Cowden disease.

External links

References

  1. ^ Vernia P, Marcheggiano A, Marinaro V, Morabito S, Guzzo I, Pierucci A (October 2005). "Is Cronkhite-Canada Syndrome necessarily a late-onset disease?". Eur J Gastroenterol Hepatol 17 (10): 1139–41. PMID 16148564. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0954-691X&volume=17&issue=10&spage=1139. 
  2. ^ Calva D, Howe JR (August 2008). "Hamartomatous polyposis syndromes". The Surgical clinics of North America 88 (4): 779–817, vii. doi:10.1016/j.suc.2008.05.002. PMID 18672141. http://linkinghub.elsevier.com/retrieve/pii/S0039-6109(08)00069-8. 
  3. ^ Cronkhite LW, Canada WJ (June 1955). "Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia". N. Engl. J. Med. 252 (24): 1011–5. doi:10.1056/NEJM195506162522401. PMID 14383952. 
  4. ^ Junnarkar SP, Sloan JM, Johnston BT, Laird JD, Irwin ST (May 2001). "Cronkhite-Canada syndrome". The Ulster medical journal 70 (1): 56–8. PMC 2449205. PMID 11428328. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2449205. 
  5. ^ Nagata J, Kijima H, Hasumi K, Suzuki T, Shirai T, Mine T (June 2003). "Adenocarcinoma and multiple adenomas of the large intestine, associated with Cronkhite-Canada syndrome". Dig Liver Dis 35 (6): 434–8. PMID 12868681. 
  6. ^ Ward E, Wolfsen HC, Ng C (February 2002). "Medical management of Cronkhite-Canada syndrome". South. Med. J. 95 (2): 272–4. PMID 11846261. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0038-4348&volume=95&issue=2&spage=272. 
v · d · eTumors: digestive system neoplasia (C15–C26/D12–D13, 150–159/211)
GI tract
Upper GI tract
Upper and/or lower
Accessory
exocrine pancreas: Adenocarcinoma · Pancreatic ductal carcinoma
cystic neoplasms: Serous microcystic adenoma · Intraductal papillary mucinous neoplasm · Mucinous cystic neoplasm · Solid pseudopapillary neoplasm
Pancreatoblastoma
Peritoneum
Primary peritoneal carcinoma · Peritoneal mesothelioma · Desmoplastic small round cell tumor

M: DIG

anat(t, g, p)/phys/devp/enzy

noco/cong/tumr, sysi/epon

proc, drug(A2A/2B/3/4/5/6/7/14/16), blte

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Look at other dictionaries:

  • Cronkhite-Canada syndrome — Cron·khite Can·a·da syndrome (krongґkīt kanґə də) [Leonard Wolsey Cronkhite, Jr., American internist, born 1919; Wilma Jeanne Canada, American radiologist, 20th century] see under syndrome …   Medical dictionary

  • Cronkhite-Canada syndrome — a rare syndrome of sporadic, widespread intestinal polyps and malabsorption accompanied by ectodermal defects such as alopecia and onychodystrophy; called also Canada Cronkhite s …   Medical dictionary

  • Cronkhite–Canada disease — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 1924 ICD10 = ICD9 = ICD9|211.3 ICDO = OMIM = 175500 MedlinePlus = eMedicineSubj = derm eMedicineTopic = 729 MeshID = D044483 Cronkhite–Canada disease is a rare syndrome characterised by… …   Wikipedia

  • Syndrome de cronkhite-canada — Affection non familiale, d étiologie inconnue associant polypose gastro intestinale alopécie hyperpigmentation cutanée onychodystrophie Biologiquement : déficits mutiples en protéines, fer, magnésium, calcium ... Le pronostic est sombre bien …   Wikipédia en Français

  • Syndrome de Cronkhite-Canada — Affection non familiale, d étiologie inconnue associant polypose gastro intestinale alopécie hyperpigmentation cutanée onychodystrophie Biologiquement : déficits mutiples en protéines, fer, magnésium, calcium ... Le pronostic est sombre bien …   Wikipédia en Français

  • Canada-Cronkhite syndrome — Can·a·da Cronk·hite syndrome (kanґə də krongґkīt) [Wilma Jeanne Canada, American radiologist, 20th century; Leonard W. Cronkhite, Jr., American internist, born 1919] Cronkhite Canada syndrome; see under syndrome …   Medical dictionary

  • Canada-Cronkhite syndrome — Cronkhite Canada s …   Medical dictionary

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Canada — Wilma J., U.S. radiologist. See Cronkhite C. syndrome …   Medical dictionary

  • Cronkhite — Leonard W., Jr., U.S. physician, *1919. See C. Canada syndrome …   Medical dictionary

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