title: Cronkhite-Canada syndrome: report of six cases and review of literature.
abstract: Cronkhite-Canada syndrome (CCS) is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin. The aim of this study was to investigate the clinical features and potential therapies for CCS. Six patients with CCS admitted from December 1992 to July 2008 to Peking Union Medical College Hospital were evaluated. All patients had clinical manifestation of nonhereditary gastrointestinal polyposis with diarrhea, skin hyperpigmentation, alopecia, and nail dystrophy. Fecal occult blood was positive in all six cases. Serum hemoglobin, potassium, calcium and protein were below the normal range in two cases. Anti-Saccharomyces cerevisiae and antinuclear antibodies were present in three cases. Multiple polyps were found in all patients by gastroscopy and colonoscopy, with only one in the esophagus. Histologically, there were hyperplastic polyps in five cases, tubular adenoma in three, and juvenile polyp in one with chronic inflammation and mucosal edema. Comprehensive treatment led by corticosteroids can result in partial remission of clinical symptoms, and long-term follow-up is necessary.
authors: Xiao-Heng Wen, Lan Wang, Jia-Ming Qian, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
authors: Xiao-Heng
authors: XH
authors: Wen
authors: Xiao-Heng Wen, Lan Wang, Jia-Ming Qian, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
authors: Lan
authors: L
authors: Wang
authors: Xiao-Heng Wen, Lan Wang, Jia-Ming Qian, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
authors: Yu-Xuan
authors: YX
authors: Wang
authors: Xiao-Heng Wen, Lan Wang, Jia-Ming Qian, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
authors: Jia-Ming
authors: JM
authors: Qian
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000505:Alopecia / Q000175:diagnosis / Q000209:etiology; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001284:Atrophy; D001706:Biopsy; D003131:Combined Modality Therapy; D013505:Digestive System Surgical Procedures; D016099:Endoscopy, Gastrointestinal; D005260:Female; D006801:Humans; D017495:Hyperpigmentation / Q000175:diagnosis / Q000209:etiology; D044483:Intestinal Polyposis / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009260:Nail Diseases / Q000175:diagnosis / Q000209:etiology; D009262:Nails / Q000473:pathology; D010288:Parenteral Nutrition; D012880:Skin Pigmentation; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents
keywords: Clinical feature; Cronkhite-Canada syndrome; Etiology; Therapeutics