Medical management of Cronkhite-Canada syndrome.
We report the case of a patient with Cronkhite-Canada syndrome (CCS) successfully treated with combination medical therapy. This rare, noninherited gastrointestinal polyposis syndrome is associated with characteristic ectodermal abnormalities. The etiology and pathogenesis of CCS are not known. No medical therapy has been shown to be consistently effective, and the disease is usually fatal. The patient described herein was successfully treated with a novel combination regimen consisting of H1- and H2-receptor antagonists, cromolyn sodium, prednisone, and suppressive antibiotics. This regimen has resulted in sustained improvement in symptoms, and follow-up endoscopy has shown regression of the diffuse polyposis. Our patient's response to such therapy may provide insight into the optimal treatment for CCS.
Ward
Eric
E
Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL 32224, USA.
Wolfsen
Herbert C
HC
Ng
Christine
C
D000368:Aged; D018927:Anti-Asthmatic Agents / Q000627:therapeutic use*; D000890:Anti-Infective Agents / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D004205:Cromolyn Sodium / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D024841:Fluoroquinolones; D006634:Histamine H1 Antagonists / Q000627:therapeutic use*; D006635:Histamine H2 Antagonists / Q000627:therapeutic use*; D006801:Humans; D007417:Intestinal Polyps / Q000188:drug therapy* / Q000276:immunology / Q000628:therapy; D017336:Loratadine / Q000627:therapeutic use*; D008297:Male; D011241:Prednisone / Q000627:therapeutic use*; D011899:Ranitidine / Q000627:therapeutic use*
D018927:Anti-Asthmatic Agents; D000890:Anti-Infective Agents; D000893:Anti-Inflammatory Agents; D024841:Fluoroquinolones; D006634:Histamine H1 Antagonists; D006635:Histamine H2 Antagonists; D017336:Loratadine; D011899:Ranitidine; D004205:Cromolyn Sodium; D011241:Prednisone