Clinical diagnosis and treatment of 13 cases of Cronkhite-Canada syndrome

Wang, Hongxia; Yan, Bin; Zhou, Zhongyi; Wang, Jing; Dou, Yan

doi:10.11569/wcjd.v27.i9.570

Cited by 1 publication

(1 citation statement)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Till now, only 2 family cases have been reported and most of the cases are sporadic. However, both the mother and the child have APC gene c.3921-3925delAAAAG (p.Ile1307fsX6) mutation [11, 12]. What’s more, genetic sequencing analysis also found that PRKDC and MUC3A mutation might contribute to CCS pathogenesis in other case report [13, 14].…”

Section: Discussionmentioning

confidence: 99%

Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases

Huang

Wang

et al. 2021

Dig Dis

View full text Add to dashboard Cite

Introduction: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal (GI) polyposis and ectodermal dysplasia. So far, most of CCS related literatures are published as single case report or reviewed with limited case numbers. Our study was to update the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literatures and 1 case from our department). Their clinical and endoscopic data were collected, and statistical analyses were performed. Results: 1) In Chinese population, people aged 50-70 years (62.62%) had a high incidence of CCS, and the ratio of male-to-female was 2.68:1. 2) The diverse range of GI manifestations were observed in all the patients, and almost all the patients had at least one symptom of ectodermal dysplasias. 3) All CCS patients presented multiple polyps in the GI tract except esophagus, and the size and appearance of polyps were diverse. Congestion, edema and erosion were very common on the surface of polyps (96.83%) and the surrounding mucosa (85.71%) . 4) The common pathological features of polyps were hyperplastic polyps (49.25%) and tubular adenomatous polyps (44.78%). There is 5.97% cancer reported. Conclusions: middle-aged and elderly people are the high-risk group; various GI symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps; these GI polyps has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the Chinese CCS patients. Key words: Cronkhite-Canada syndrome, Clinical characteristics, Endoscopy

show abstract

Section: Discussionmentioning

confidence: 99%