A severe course of Cronkhite-Canada syndrome and the review of clinical features and therapy in 49 Chinese patients

She, Qiang; Jiang, Jian-Xia; Xinmin, SI; Tian, Xueying; Shi, Rui-Hua; Zhang, Guoxin

doi:10.4318/tjg.2013.0527

Cited by 20 publications

(24 citation statements)

References 11 publications

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“…Formal surveys of CCS have yet to be reported in other countries. In Asia, She et al collected and described case reports of 50 CCS patients in China, 9 and Yun et al reported on 13 CCS cases in South Korea. 8 In the United States, the Mayo Clinic reported detailed information from 14 CCS patients treated from 1955 to 2009.…”

Section: Discussionmentioning

confidence: 99%

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Estimated Prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated With <i>SLCO2A1</i> Gene, and Intestinal Behçet’s Disease in Japan in 2017: A Nationwide Survey

Oba

Murakami

Nishiwaki

et al. 2021

Journal of Epidemiology

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Background: Cronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet's disease (BD) are classified as intractable intestinal disorders in Japan. However, the national prevalence of these diseases remains unknown. We performed a nationwide survey to estimate the patient numbers and prevalence rates of these diseases throughout Japan in 2017. Methods: We conducted a mail-based survey targeting hospitals across Japan to estimate the annual numbers of patients with CCS, CEAS, and intestinal BD in 2017. Using a stratified random sampling method, we selected 2,979 hospital departments and asked them to report the number of patients who met specific diagnostic criteria. The total number of patients for each disease was estimated by multiplying the reported numbers by the reciprocal of the sampling rate and response rate. The corresponding prevalence rates per 1,000,000 population were calculated based on the mid-year population of Japan in 2017. Results: The overall survey response rate was 68.1% (2,029 departments). The estimated numbers of patients with CCS, CEAS, and intestinal BD were 473 (95% confidence interval [CI], 357-589), 388 (95% CI, 289-486), and 3,139 (95% CI, 2,749-3,529), respectively; the prevalence rates per 1,000,000 population were 3.7 (male: 4.0; female: 3.5), 3.1 (male: 3.0; female: 3.1), and 24.8 (male: 24.5; female: 25.0), respectively. The male-to-female ratios were 1.10, 0.94, and 0.93 for patients with CCS, CEAS, and intestinal BD, respectively. Conclusions: Estimates of the national prevalence of CCS, CEAS, and intestinal BD in Japan were generated and found to be higher than those previously reported.

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Section: Discussionmentioning

confidence: 99%

“…[2][3][4] Numerous case series have been reported in Asian countries. [5][6][7][8][9] In Japan, Watanabe et al reported that 213 CCS patients were diagnosed between 2000 and 2013 in 140 teaching hospitals. 10 CEAS was first reported in 1968 11 and initially described as chronic nonspecific multiple ulcers of the small intestine.…”

Section: Introductionmentioning

confidence: 99%

Estimated Prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated With <i>SLCO2A1</i> Gene, and Intestinal Behçet’s Disease in Japan in 2017: A Nationwide Survey

Oba

Murakami

Nishiwaki

et al. 2021

Journal of Epidemiology

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“…Numerous treatment approaches, including aggressive nutritional support,14 100 101 antibiotics,8 42 histamine receptor antagonists,14 cromolyn sodium,14 immune suppression (corticosteroids, azathioprine and cyclosporine),13 20 102 103 and surgery89 104 105—often in different combinations—have had variable success 4 6 100 102 106. Apart from nutritional support, corticosteroids are currently recommended as the mainstay of medical treatment,26 with azathioprine used for its steroid-sparing effect 13.…”

Section: Therapy and Outcomementioning

confidence: 99%

Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease

Slavík¹,

Montgomery

2014

J Clin Pathol

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Cronkhite–Canada syndrome is a rare gastro-entero-colopathy of uncertain aetiology first described almost 60 years ago. It is characterised by diffuse gastrointestinal polyposis sparing only the oesophagus, ectodermal abnormalities and an unpredictable but often fatal clinical course. The disease may demonstrate extremely diverse clinical and endoscopic features, which often leads to a delay in diagnosis. A high index of suspicion and recognition of the characteristic histological findings frequently facilitate a correct diagnosis, but the distribution of the gastrointestinal pathology and its microscopic features may be atypical. The pathologist thus requires a thorough knowledge of both the typical and many atypical faces of this disease, for which various documented therapies often still prove ineffective. Close correlation with clinical findings, including any pertinent ectodermal abnormalities, and careful examination of biopsies derived from polypoid and endoscopically spared mucosa will ensure a timely and correct diagnosis in patients with this enigmatic syndrome.

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“…The de ciency of zinc and copper could also lead to hypogeusia [16]. Serum electrolyte disturbances is very common in CCS patients due to malabsorption and appetite loss [8]. However, we didn't get enough data to con rm the connection between hypogeusia and electrolyte disturbances in current study.…”

Section: Discussionmentioning

confidence: 83%

“…It mainly explored the best potential of treatment and monitoring strategies by evaluating the treatment e cacy of endoscopy and other approaches. As for Chinese CCS patients, The most recent literature in international journal was published in 2013 with limited cases (49 Chinese CCS patients) [8]. It summarized the clinical characteristics of CCS patients to better understand CCS in China.…”

Section: Introductionmentioning

confidence: 99%

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

Huang

Wang

et al. 2020

Preprint

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Background: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal polyposis and ectodermal dysplasia. Its etiology still remains indefinite to us. Currently, the diagnosis is mainly based on typical clinical manifestations and endoscopic features. However, there is a lack of these information from Chinese CCS patients. Our study was to investigate the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literature 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 gastrointestinal (GI) manifestations were observed in all the patients, and the most common symptoms were weight loss (98.80%) and loss of appetite (93.94%). 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. The size of polyps varied from 0.5 cm (88.64%) to 0.6-1.0 cm (81.82%) . The polyps were various in shapes, the most of them were nodular polyps (58.14%) ,what’s more,the sessile polyps were the most common(90.24%). 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 only 5.97% cancer reported. Conclusions: middle-aged and elderly people are the high-risk group; various gastrointestinal symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps; these gastrointestinal polyps has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the CCS patients in China.

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A severe course of Cronkhite-Canada syndrome and the review of clinical features and therapy in 49 Chinese patients

Cited by 20 publications

References 11 publications

Estimated Prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated With <i>SLCO2A1</i> Gene, and Intestinal Behçet’s Disease in Japan in 2017: A Nationwide Survey

Estimated Prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated With <i>SLCO2A1</i> Gene, and Intestinal Behçet’s Disease in Japan in 2017: A Nationwide Survey

Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease

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

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