Cronkhite-Canada Syndrome:Clinical Analysis of 9 Cases
10.3969/j.issn.1008-7125.2023.07.001
- VernacularTitle:Cronkhite-Canada综合征9例临床分析
- Author:
Songfei LI
1
;
Shigang DING
Author Information
1. 北京大学第三医院消化内科(100191)
- Keywords:
Cronkhite-Canada Syndrome;
Multiple Gastrointestinal Polyps;
Glucocorticoids
- From:
Chinese Journal of Gastroenterology
2023;28(7):385-389
- CountryChina
- Language:Chinese
-
Abstract:
Background:Cronkhite Canada syndrome(CCS)is a rare non hereditary disease of unknown etiology,there are still challenges in its diagnosis and treatment.Aims:To explore the clinical characteristics and treatment responses of CCS in Chinese population,and to improve the understanding of its diagnosis and treatment.Method:Retrospective analysis and summary of clinical data of CCS patients at Peking University Third Hospital from 2012 to 2022.Results:From the clinical data of a total of 9 cases,the patients'average age was 63.89 years,and the male to female ratio was 1.25.all patients presented with multiple gastrointestinal polyps,non-specific gastrointestinal symptoms and at least one ectodermal manifestation.Laboratory tests for fecal occult blood were positive,and iron deficiency anemia and hypoalbuminemia were common.Histological manifestations were hyperplastic polyps and adenomatous polyps,with commonly found eosinophil infiltration.Patients'conditions could be complicated with severe osteoporosis and fractures,intestinal bacterial overgrowth,asthma,membranous nephropathy,and nodular goiter.Most patients obtained positive currative effect with glucocorticoids therapy,but recurrence of the disease may occur during or after hormone reduction.Azathioprine treatment was attempted in one patient,but the effect was poor.Conclusions:The etiology and pathogenesis of CCS are unclear,and glucocorticoids therapy is still the main treatment method.However,there are still challenges for patients with glucocorticoids resistance and contraindications to glucocorticoids therapy.
