Cronkhite-Canada综合征26例临床特点及诊疗过程分析
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摘要
目的探讨Cronkhite-Canada综合征(CCS)的临床特点和治疗方法。方法分析1999年10月至2018年1月北京协和医院确诊的CCS患者的临床特点,并以2012年为界分为两组进行诊治情况的对比。结果共26例患者,男18例,女8例;起病年龄(58.7±7.0)岁;首发症状主要是腹泻(12/26)和味觉减退(9/26),病理以增生性息肉(16/26)最多见;均采用糖皮质激素治疗,2013年之后的患者泼尼松起始用量略增加[(0.8±0.4)mg/(kg·d)对(0.7±0.2)mg/(kg·d),P=0.38],且有2例加用了免疫抑制剂治疗后才达到临床症状及内镜表现好转。治疗的临床症状好转率达92%(23/25),内镜表现好转率达88.9%(16/18),有2例分别在起病5年和1年后出现癌变。结论 CCS的诊断率近年呈上升趋势,免疫抑制剂治疗对于激素疗效不佳的患者亦可取得一定的疗效。
Objective To explore clininal characteristics the diagnosis and treatment of Cronkhite-Canada syndrome(CCS).Methods Clinical characteristics were retrospectively analyzed in all CCS patients diagnosed from October 1999 to January 2018 in Peking Union Medical College Hospital.The diagnostic and therapeutic strategies were compared between patients who were diagnosed before 2012 and patients after 2013.Results There were 26 patients enrolled in this study,in which 18 patients were male.The CCS onset age was(58.7±7.0) years old.Initial symptoms were mainly diarrhea(12/26) and hypogeusia(9/26).The pathology was mainly hyperplastic polyp(16/26).All patients were treated with glucocorticoid therapy.The starting dose of steroid was slightly increased after 2013 [(0.8±0.4)mg/(kg·d) vs(0.7±0.2) mg/(kg·d),P=0.38].Two patients were prescribed with immunosuppressors because of poor effects of steroid,and they got both clinical and endoscopic resolution.Clinical outcomes were improved in 23/25(92%),and polyps were decreased in 16/18 patients.Two patients were detected with colon carcinoma in the second and fifth year respectively.Conclusion The diagnosis rate seems to increase in these years.In treating CCS,immunosuppressors might get better effects on those steroid-resistant patients.
Objective To explore clininal characteristics the diagnosis and treatment of Cronkhite-Canada syndrome(CCS).Methods Clinical characteristics were retrospectively analyzed in all CCS patients diagnosed from October 1999 to January 2018 in Peking Union Medical College Hospital.The diagnostic and therapeutic strategies were compared between patients who were diagnosed before 2012 and patients after 2013.Results There were 26 patients enrolled in this study,in which 18 patients were male.The CCS onset age was(58.7±7.0) years old.Initial symptoms were mainly diarrhea(12/26) and hypogeusia(9/26).The pathology was mainly hyperplastic polyp(16/26).All patients were treated with glucocorticoid therapy.The starting dose of steroid was slightly increased after 2013 [(0.8±0.4)mg/(kg·d) vs(0.7±0.2) mg/(kg·d),P=0.38].Two patients were prescribed with immunosuppressors because of poor effects of steroid,and they got both clinical and endoscopic resolution.Clinical outcomes were improved in 23/25(92%),and polyps were decreased in 16/18 patients.Two patients were detected with colon carcinoma in the second and fifth year respectively.Conclusion The diagnosis rate seems to increase in these years.In treating CCS,immunosuppressors might get better effects on those steroid-resistant patients.
引文
[1]Cronkhite LW Jr,Canada WJ.Generalized gastrointestinal polyposis;an unusual syndrome of polyposis,pigmentation,alopecia and onychotrophia[J].N Engl J Med,1955,252(24):1011-1015.
[2]Goto A.Cronkhite-Canada syndrome:epidemiological study of 110cases reported in Japan[J].Nihon Geka Hokan,1995,64(1):3-14.
[3]刘颖娴,吴东,费贵军,等.12例Cronkhite Canada综合征患者临床及病理特点[J].基础医学与临床,2012,32(11):1349-1352.
[4]Sweetser S,Boardman L A.Cronkhite-Canada syndrome:an acquired condition of gastrointestinal polyposis and dermatologic abnormalities[J].Gastroenterol Hepatol(N Y),2012,8(3):201-203.
[5]Watanabe C,Komoto S,Tomita K,et al.Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome:a Japanese nationwide survey[J].J Gastroenterol,2016,51(4):327-336.
[6]Ward E M,Wol f sen HC.R ev iew ar t icle:the non-inher ited gastrointestinal polyposis syndromes[J].Aliment Pharmacol Ther,2002,16(3):333-342.
[7]Sweetser S,Ahlquist DA,Osborn NK,et al.Clinicopathologic features and treatment outcomes in Cronkhite-Canada syndrome:support for autoimmunity[J].Dig Dis Sci,2012,57(2):496-502.
[8]Ohmiya N,Nakamura M,Yamamura T,et al.Steroid-resistant Cronkhite-Canada syndrome successfully treated by cyclosporine and azathioprine[J].J Clin Gastroenterol,2014,48(5):463-464.
[9]Watanabe D,Ooi M,Hoshi N,et al.Successful treatment of CronkhiteCanada syndrome using anti-tumor necrosis factor antibody therapy[J].Endoscopy,2014,46 Suppl 1:E476-477.
[10]Matsui S,Kibi M,Anami E,et al.A case of Cronkhite-Canada syndrome with multiple colon adenomas and early colon cancers[J].Japan J Gastroenterol,2011,108(5):778-786.
[2]Goto A.Cronkhite-Canada syndrome:epidemiological study of 110cases reported in Japan[J].Nihon Geka Hokan,1995,64(1):3-14.
[3]刘颖娴,吴东,费贵军,等.12例Cronkhite Canada综合征患者临床及病理特点[J].基础医学与临床,2012,32(11):1349-1352.
[4]Sweetser S,Boardman L A.Cronkhite-Canada syndrome:an acquired condition of gastrointestinal polyposis and dermatologic abnormalities[J].Gastroenterol Hepatol(N Y),2012,8(3):201-203.
[5]Watanabe C,Komoto S,Tomita K,et al.Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome:a Japanese nationwide survey[J].J Gastroenterol,2016,51(4):327-336.
[6]Ward E M,Wol f sen HC.R ev iew ar t icle:the non-inher ited gastrointestinal polyposis syndromes[J].Aliment Pharmacol Ther,2002,16(3):333-342.
[7]Sweetser S,Ahlquist DA,Osborn NK,et al.Clinicopathologic features and treatment outcomes in Cronkhite-Canada syndrome:support for autoimmunity[J].Dig Dis Sci,2012,57(2):496-502.
[8]Ohmiya N,Nakamura M,Yamamura T,et al.Steroid-resistant Cronkhite-Canada syndrome successfully treated by cyclosporine and azathioprine[J].J Clin Gastroenterol,2014,48(5):463-464.
[9]Watanabe D,Ooi M,Hoshi N,et al.Successful treatment of CronkhiteCanada syndrome using anti-tumor necrosis factor antibody therapy[J].Endoscopy,2014,46 Suppl 1:E476-477.
[10]Matsui S,Kibi M,Anami E,et al.A case of Cronkhite-Canada syndrome with multiple colon adenomas and early colon cancers[J].Japan J Gastroenterol,2011,108(5):778-786.