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Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey.

ABSTRACT: First 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.A 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.The 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.The 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.

SUBMITTER: Watanabe C

PROVIDER: S-EPMC4805704 | biostudies-other | 2016 Apr

REPOSITORIES: biostudies-other

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Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey.

Watanabe Chikako C Komoto Shunsuke S Tomita Kengo K Hokari Ryota R Tanaka Masanori M Hirata Ichiro I Hibi Toshifumi T Kaunitz Jonathan D JD Miura Soichiro S

Journal of gastroenterology 20150728 4

<h4>Background</h4>First 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.<h4>Meth ...[more]

PMID: 26216651

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Project description:Background and study objectiveIn response to growing pressures on healthcare systems, the advanced clinical practice (ACP) role has been implemented widely in the UK and internationally. In England, ACP is a level of practice applicable across various healthcare professions, who exercise a level of autonomy across four domains, referred to as the four pillars of practice (education, leadership, research and clinical practice). A national framework for ACP was established in 2017 to ensure consistency across the ACP role, however current ACP governance, education and support is yet to be evaluated. This study aimed to analyse data from a national survey of the ACP role to inform the development and improvement of policies relating to ACP in the National Health Service (NHS) in England.DesignA cross-sectional survey with free-text comments.SettingThe survey was distributed across primary and secondary levels of care to three distinct groups in England, including individual ACPs, NHS provider organisations and Trusts and primary care settings.ParticipantsA total of 4365 surveys were returned, from ACP staff (n=4013), NHS provider organisations and Trusts (n=166) and primary care organisations (n=186).ResultsConsiderable variation was found in role titles, scope of practice, job descriptions and educational backgrounds of ACPs. Differing approaches to governance were noted, which led to inconsistent ACP frameworks in some organisations. A further challenge highlighted included committing time to work across the four pillars of advanced practice, particularly the research pillar. ACPs called for improvements in supervision and continuing professional development alongside further support in navigating career pathways.ConclusionsA standardised approach may support ACP workforce development in England and enable ACPs to work across the four pillars of practice. Due to the wide uptake of ACP roles internationally, this study has relevance across professions for global healthcare workforce transformation.

Dataset Information

Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey.

Publications

Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey.

Similar Datasets

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