Pharmacological management of Cronkhite-Canada syndrome.
Cronkhite-Canada syndrome (CCS) is a rare, non-inherited gastrointestinal polyposis syndrome associated with characteristic ectodermal abnormalities. A number of potentially life-threatening complications including malnutrition, gastrointestinal bleeding and infection may occur in affected patients and CCS is fatal in many cases. The optimal therapy for CCS is not known but several treatment options have been described. Nutritional support, antibiotics, corticosteroids, anabolic steroids, histamine-receptor antagonists and surgical treatment have all been used with varying degrees of success. Unfortunately, controlled therapeutic trials have not been possible because of the rarity of the disease. Most recently, a combination regimen using histamine-receptor antagonists, cromolyn sodium, prednisone and suppressive antibiotics has been described. The reported treatment options and rates of success are reviewed.
Ward
Eric M
EM
Division of Gastroenterology and Hepatology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA.
Wolfsen
Herbert C
HC
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D045930:Anabolic Agents / Q000627:therapeutic use; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D013505:Digestive System Surgical Procedures; D004359:Drug Therapy, Combination; D016099:Endoscopy, Gastrointestinal; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D006633:Histamine Antagonists / Q000627:therapeutic use; D006801:Humans; D007417:Intestinal Polyps / Q000188:drug therapy* / Q000601:surgery / Q000628:therapy; D018529:Nutritional Support; D054328:Proton Pump Inhibitors
D000305:Adrenal Cortex Hormones; D045930:Anabolic Agents; D000900:Anti-Bacterial Agents; D004791:Enzyme Inhibitors; D006633:Histamine Antagonists; D054328:Proton Pump Inhibitors