Pediatric Cronkhite-Canada Syndrome Medication

Updated: Nov 17, 2015
  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD  more...
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Medication

Medication Summary

A generalized algorithm for treating 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 (30-49 mg prednisolone/day) have been widely used in Japan and have proven to be effective in about 80% of patients. Care should be used to document endoscopic remission of polyposis to avoid early relapse. [2] Other immunosuppressive agents (calcineurin inhibitors, azathioprine, anti-TNG-alpha agents) have been used as steroid-sparing agents or in steroid-refractory patients.

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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.

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.

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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