ArticleJournal of gastroenterology2016
Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey.
Article in Journal of gastroenterology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 65 papers.
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Who cites it
65 citing papers in PubMed, 106 citations in OpenAlex.
- Comprehensive nursing care for advanced digestive malignancy patients during chemotherapy based on probiotic intervention: a randomized controlled study on improvement of gastrointestinal function and prevention of complications.Frontiers in cellular and infection microbiology · 2025Trial
- Cronkhite-Canada syndrome: An elusive gastrointestinal disorder with multisystem involvement-pathogenesis, diagnosis, and therapeutic strategies.World journal of gastrointestinal pathophysiology · 2026Review
- Intussusception in Cronkhite-Canada Syndrome.Diagnostics (Basel, Switzerland) · 2026Article
- Challenges and Opportunities in Cronkhite-Canada Syndrome Research: A Bibliometric Analysis Based on the Web of Science Core Collection.Medical science monitor : international medical journal of experimental and clinical research · 2026Article
- Cronkhite-Canada Syndrome: A Diagnostic Challenge and Management With Vedolizumab.The American journal of case reports · 2026Article
- Cronkhite-Canada syndrome, hypothyroidism and positive autoantibodies: a case report.Journal of medical case reports · 2026Article
- Cronkhite-Canada syndrome after corticosteroid and mesalazine treatment: A case report and 3-year follow-up.Medicine · 2026Article
- Case Report: A case of Cronkhite-Canada syndrome associated with sigmoid colon cancer and early esophageal cancer.Frontiers in medicine · 2026Article
- Cronkhite-Canada Syndrome With Multiple Mesenteric Lymphadenopathy: A Case Report.JGH open : an open access journal of gastroenterology and hepatology · 2025Article
- Arrhythmias in Cronkhite-Canada Syndrome.Internal medicine (Tokyo, Japan) · 2025Article
- A Case of Asymptomatic Cronkhite-Canada Syndrome Diagnosed by Endoscopic and Pathological Findings.Cureus · 2025Article
- Infliximab treatment for Cronkhite-Canada syndrome in pregnancy: A case report.Case reports in women's health · 2025Article
- Article
- A case of membranous nephropathy complicated by Cronkhite-Canada syndrome successfully treated with mizoribine.CEN case reports · 2025Article
- A Case of Ascending Colon Cancer Complicated by Cronkhite-Canada Syndrome Treated with Robot-Assisted Right Hemicolectomy.Surgical case reports · 2025Article
- Efficacy and safety of azathioprine in patients with Cronkhite-Canada syndrome: a case series from Peking Union Medical College Hospital.BMC pharmacology & toxicology · 2024Article
- Cronkhite-Canada syndrome tends to be accompanied by colorectal cancer: Report of seven cases.JGH open : an open access journal of gastroenterology and hepatology · 2024Article
- Cronkhite-Canada syndrome complicated with pulmonary embolism: A case report.World journal of clinical cases · 2024Article
- Spontaneous remission of Cronkhite-Canada syndrome without immunosuppressive medication in the context of decompensated cirrhosis: A case report.Clinical case reports · 2024Article
- Cronkhite-Canada Syndrome With Advanced Gastric Cancer.ACG case reports journal · 2024Article
5 more citing papers are in PubMed but not listed here.
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Authors and funding
9 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundFirst reported in 1955, Cronkhite-Canada syndrome (CCS), a rare syndrome characterized by ectodermal abnormalities and inflammatory changes of the gastrointestinal tract mucosa, has been associated with a poor prognosis and life-threatening malignant complications. In a large population survey, we endeavored to characterize the course and treatment outcome of CCS through clinical and endoscopic assessment, and to explore its optimal treatment and surveillance strategy.
methodsA retrospective analysis of 210 patients with CCS was conducted via a questionnaire-based nationwide survey of 983 teaching hospitals located throughout Japan. We assessed clinical features, endoscopic findings, treatments used, and short- and long-term outcomes.
resultsThe average age at diagnosis was 63.5 years. In all cases, upper or lower gastrointestinal tract polyposis was confirmed, accompanied by characteristic ectodermal abnormalities. Of the treatments used, oral corticosteroids (30-49 mg/day) were the most effective treatment for active disease, with adjunctive nutritional support considered beneficial. With corticosteroid treatment, abdominal symptoms were relieved within a few months, whereas polyp regression often required more than 6 months. Maintenance of endoscopic remission with or without steroids for 3 years significantly lowered the development of CCS-related cancer, compared with relapsers or nonresponders, underscoring the importance of sustained endoscopic remission for cancer prevention.
conclusionsThe prognosis of CCS has greatly improved through the use of improved medical treatment. Although CCS continues to be relentlessly progressive, carrying a high cancer risk, a sufficient dose and duration of corticosteroid therapy accompanied by nutritional support and periodic endoscopic surveillance appears to improve its natural history.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.