The Cronkhite-Canada Syndrome with Focus on Immunity and Infection Report of a Case

Lin, Hwai Jeng; Tsai, Ying-Yang; Lee, Shou‐Dong; Lai, Kwok‐Hung; Ng, Wai-Wah; Tam, Tseng-Nip; Lin, Hsin‐Hung; Liou, Lieh-Bang; Tsay, Shyh Haw

doi:10.1097/00004836-198710000-00018

Cited by 20 publications

(9 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…3 As shown in our case, the malnutrition may be generally associated with the protein-losing enteropathy with hypoalbuminemia. Although spontaneous remission of protein-losing enteropathy has been reported, 15 and a few cases have been effectively treated with enteral nutrition, total parenteral nutrition, steroids, and antiplasmin therapy, [4][5][6][7] there is no documented therapy for protein-losing enteropathy.…”

Section: Discussionsupporting

confidence: 61%

Application of Technetium-99m-Labeled Human Serum Albumin Scan to Assist Surgical Treatment of Protein-Losing Enteropathy in Cronkhite-Canada Syndrome: Report of A Case

Tseng

Sheu

Lee

et al. 2005

Diseases of the Colon &Amp; Rectum

View full text Add to dashboard Cite

Cronkhite-Canada syndrome is a rare form of nonhereditary gastrointestinal polyposis associated with ectodermal change and protein-losing enteropathy. Here we report a 63-year-old male presenting with diffuse gastrointestinal polyposis, onychodystrophy, cutaneous pigmentation, alopecia, diarrhea, hypoalbuminemia and lower leg edema. Technetium-99m-labeled human serum albumin scan confirmed the patient to have protein-losing enteropathy, which originated from the transverse and descending colon. Subtotal colectomy was performed. Albumin level and ectodermal change were gradually improved during three years of outpatient clinic follow-up. Based on our finding, Technetium-99m-labeled human serum albumin scintigraphy is helpful to localize the protein-losing origins and surgery is an effective treatment for Cronkhite-Canada syndrome with protein-losing enteropathy.

show abstract

Section: Discussionsupporting

confidence: 61%

Application of Technetium-99m-Labeled Human Serum Albumin Scan to Assist Surgical Treatment of Protein-Losing Enteropathy in Cronkhite-Canada Syndrome: Report of A Case

Tseng

Sheu

Lee

et al. 2005

Diseases of the Colon &Amp; Rectum

View full text Add to dashboard Cite

show abstract

“…Although azathioprine used with or without steroid maintenance therapy was reported to be associated with sustained clinical remission [1, 5, 32], we could not confirm this finding. Since immunological dysregulation is one of the important factors hypothesized to be present in CCS [5, 14, 33–36], the long-term use of immunoregulatory drugs or biologics may be useful for active or refractory disease.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2015

View full text Add to dashboard Cite

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.Electronic supplementary materialThe online version of this article (doi:10.1007/s00535-015-1107-7) contains supplementary material, which is available to authorized users.

show abstract

“…Various aetiologies have been proposed, but many recent studies favour an autoimmune process characterised by immune dysregulation 13 14 16 17. An autoimmune aetiopathogenesis is supported by the demonstration of antinuclear antibodies,18 an association with hypothyroidism,8 19 and other autoimmune diseases (including systemic lupus erythematosus, rheumatoid arthritis and scleroderma),14 20–22 elevated serum IgG4 levels and polyp infiltration by IgG4 plasma cells 13 16.…”

Section: Aetiopathogenesismentioning

confidence: 99%

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

Slavík¹,

Montgomery

2014

J Clin Pathol

View full text Add to dashboard Cite

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.

show abstract

The Cronkhite-Canada Syndrome with Focus on Immunity and Infection Report of a Case

Cited by 20 publications

References 0 publications

Application of Technetium-99m-Labeled Human Serum Albumin Scan to Assist Surgical Treatment of Protein-Losing Enteropathy in Cronkhite-Canada Syndrome: Report of A Case

Application of Technetium-99m-Labeled Human Serum Albumin Scan to Assist Surgical Treatment of Protein-Losing Enteropathy in Cronkhite-Canada Syndrome: Report of A Case

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

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

Contact Info

Product

Resources

About