Pediatric Cronkhite-Canada Syndrome Medication

  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Jan 11, 2010
 

Medication Summary

A generalized algorithm for treating Cronkhite-Canada syndrome (CCS), such as one that addresses the use of any specific pharmacologic agent or nonstandard dosages, has not been established. Therapy is always supportive and includes vigorous fluid and electrolyte replacement and maintenance of macronutrient and micronutrient requirements. Antibiotics are given orally to treat small-bowel overgrowth or parenterally to treat systemic infections. Broad-spectrum coverage should be provided to patients with immunocompromise. Corticosteroids are frequently used for advanced stages. Anabolic steroids are used as a last resort, presumably for their regenerative properties. However, data supporting their use are questionable and insufficient to warrant specific recommendations.

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Corticosteroids

Class Summary

These drugs are presumably used for their anti-inflammatory effect. They elicit anti-inflammatory and immunosuppressive properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

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Hydrocortisone (Solu-Cortef)

 

Decreases inflammation by suppressing migration of polymorphonuclear (PMN) leukocytes and reversing increased capillary permeability. Data to support use sparse and anecdotal. Corticosteroids often used in combination with other agents. Documentation of spontaneous remissions further obscures picture. Standard corticosteroid dosage, as in other GI inflammatory conditions, is empiric but prudent starting point.

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Prednisone (Deltasone, Orasone)

 

Used for immunosuppression and anti-inflammatory effects. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. Data to support use sparse and anecdotal. Corticosteroids often used in combination with other agents. Documentation of spontaneous remissions further obscures picture. Standard corticosteroid dosage, as in other GI inflammatory conditions, is empiric but prudent starting point.

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Contributor Information and Disclosures
Author

Simon S Rabinowitz, MD, PhD, FAAP  Professor of Clinical Pediatrics, Vice Chairman, Clinical Practice Development, Pediatric Gastroenterology, Hepatology, and Nutrition, State University of New York Downstate College of Medicine, The Children's Hospital at Downstate

Simon S Rabinowitz, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Gastroenterology, American Gastroenterological Association, American Medical Association, New York Academy of Sciences, North American Society for Pediatric Gastroenterology and Nutrition, Phi Beta Kappa, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Manoj Sheth, MD  Chief Resident, Department of Pediatrics, Richmond University Medical Center of Long Island

Manoj Sheth, MD is a member of the following medical societies: Indian Medical Association and Indian Orthopedic Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Chris A Liacouras, MD  Director of Pediatric Endoscopy, Department of Pediatrics, Division of Gastroenterology and Nutrition, Associate Professor, Children's Hospital of Philadelphia and University of Pennsylvania

Chris A Liacouras, MD is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD  Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

References

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  2. Yashiro M, Kobayashi H, Kubo N, et al. Cronkhite-Canada syndrome containing colon cancer and serrated adenoma lesions. Digestion. 2004;69(1):57-62. [Medline].

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  5. Hizawa K, Nakamori M, Yao T, Matsumoto T, Iida, M. A Case of Cronkhite-Canada Syndrome with Colorectal Adenomas: Effect of the Nonsteroidal Anti-Inflammatory Drug Sulindac. Am J Gastroenterol. August 2007;102 (8):1831-2. [Medline].

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  13. Daniel ES, Ludwig SL, Lewin KJ, et al. The Cronkhite-Canada Syndrome. An analysis of clinical and pathologic features and therapy in 55 patients. Medicine (Baltimore). Sep 1982;61(5):293-309. [Medline].

  14. Nonomura A, Ohta G, Ibata T, et al. Cronkhite-Canada syndrome associated with sigmoid cancer case report and review of 54 cases with the syndrome. Acta Pathol Jpn. Sep 1980;30(5):825-45. [Medline].

  15. Russell DM, Bhathal PS, St John DJ. Complete remission in Cronkhite-Canada syndrome. Gastroenterology. Jul 1983;85(1):180-5. [Medline].

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  17. Ward EM, Wolfsen HC. Pharmacological management of Cronkhite-Canada syndrome. Expert Opin Pharmacother. Mar 2003;4(3):385-9. [Medline].

 
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